1
Q
- ) Iron Deficiency Anemia produces this type of anemia (also on 2012, 2014)
a. hypochromic, macrocytic
b. normochromic, macrocytic
c. hypochromic, microcytic
d. normochromic, microcytic
A
C. Trans on “Acquired Hemolytic Anemia in Adults” p.5 col.1
- Iron Deficiency Anemia
• PBS: microcytic, hypochromic cells
• ↓ Hgb, Hct, ↑ TIBC
• Transferrin saturation: 10-15%
2
Q
- ) Pregnant women should routinely be given iron because (also on 2012, 2013, 2014)
a. the fetus needs iron
b. the mother has lost iron from her previous monthly menses
c. she will lose blood when she delivers
d. all of the above
A
A. Although A & C both sound correct, this was the answer given during our feedback. The closest supporting explanation I found is this:
Anemia in Pregnancy: “Acute blood loss and chronic anemia in pregnancy are major causes of maternal morbidity and mortality worldwide. Anemia increases the likelihood of intrauterine growth retardation, premature birth, and fetal loss.” – 2007 WHO theme: Safe Blood for Safe Motherhood
Trans on “Acquired Hemolytic Anemia in Adults” p.5 col.2
3
Q
- ) Why does a patient develop iron deficiency anemia after gastroduodenal bypass surgery? (2014)
a. Because of poor iron absorption
b. Because of poor iron utilization
c. Because of poor iron intake
d. Because of decrease in the reticulo-endothelial system
A
A. Trans on “Acquired Hemolytic Anemia in Adults” p.5 col.2
One of the causes of IDA is decreased iron intake or absorption. Malabsorption is a particular problem in post-gastrectomy as iron absorption occurs in the proximal small intestines.
4
Q
- ) During the first week of treatment with oral iron, which laboratory parameter should be taken? (also on 2012, 2013 and 2014)
a. hemoglobin
b. hematocrit
c. reticulocyte count
d. red cell indices
A
C. HPIM 16th ed, p. 590
The response to iron therapy varies depending on the erythropoietin stimulus and the rate of absorption. Typically, the reticulocyte count should begin to increase within 4-7 days after initiation of therapy and peak at 1 ½ weeks.
5
Q
- ) The duration of treatment with iron is usually six (6) months because (2012, 2013, 2014)
a. the body’s iron stores have to be replenished
b. this will cover for the future occurrence of bleeding
c. this will facilitate more absorption of iron
d. all of the above
A
A. The goal of therapy is not only to repair the anemia, but also to provide stores of at least 0.5 to 1.0 g of iron. Sustained treatment for period of 6-12 months afte correction of anemia is necessary to achieve this.
6
Q
- ) Which food is rich in iron? (2013, 2014)
a. Fruits
b. Vegetables
c. Red meat
d. Fish
A
C. Although iron is also contained in fish, vegetables (poorly absorbed), and dried fruit, meat is its most important source.
7
Q
- ) The gold standard in the diagnosis of iron deficiency anemia is (also on 2012, 2013, 2014)
a. serum iron
b. serum ferritin
c. total iron binding capacity
d. hemosiderin in the bone marrow
A
D. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 5 col. 1
Evaluation of bone marrow iron stores
• GOLD STANDARD: Bone marrow hemosiderin
8
Q
- ) Parenteral iron is given if (2012, 2014)
a. rapid increase in hemoglobin (HB) is desired
b. malabsorption syndrome exists
c. the patient requests for it
d. rapid utilization of iron by the body
A
B. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 6 col. 1
Parenteral iron therapy is indicated for those who: • Cannot tolerate oral iron • Has an acute iron need • Needs iron on an on going basis • GIT disorders (see #7)
9
Q
- ) Infants should be given iron supplements as early as 2 months of age because
a. they are easily prone to colic
b. human and cow’s milk are poor sources of iron
c. they bleed easily
d. they have poor iron absorption
A
B. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 4 col. 1
Iron Deficiency in Children
• Iron supplements are indicated because human and cow’s milk are poor sources of iron
10
Q
- ) The most common single cause of iron deficiency in women is
a. poor intake of iron
b. obesity
c. poor release of iron by the reticulo-endothelial system
d. menstrual blood loss
A
D. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 3 col. 2
Blood loss due to menstruation is the most common cause of iron deficiency in women.
11
Q
- ) Chronic ingestion of non-steroidal anti-inflammatory medication can cause iron deficiency anemia by (2014)
a. interfering with iron transport
b. reducing amount of total iron binding capacity
c. inducing occult GI bleeding
d. preventing iron incorporation in the red cells
A
C. I presently can’t find where this was said explicitly during hema but given what we learned in pharma and GI, we know that excessive doses of NSAIDs can cause gastric upset and bleeding gastric and duodenal ulcers.
12
Q
- ) A transfusion reaction that usually appears rapidly that may result in fever, shock, or death is which of the following? (2014)
a. Immediate Hemolytic Transfusion Reaction
b. Transfusion Associated Circulatory Overload
c. Allergic Transfusion Reaction
d. Febrile Non-Hemolytic Transfusion Reaction
A
A. Trans on “Blood Components, Transfusion Reactions, Autologous Transfusion”, p. 4 col. 2, p. 5 col. 1
IHTR is a life-threatening transfusion reaction event which occurs soon after transfusion (1-2 h) of incompatible RBCs. Signs and symptoms, which include fever, chills, anemia, jaundice, and decreased haptoglobulins, occur within minutes of transfusion. Prompt diagnosis and treatment is essential.
13
Q
- ) A donor who has ingested aspirin on the day of donation is temporarily deferred because (2012)
a. he has fever
b. he is infected
c. aspirin alters the quality of platelets
d. aspirin causes a hypercoagulable state
A
C. Robbins 7th ed, p. 428
A bleeding tendency may appear concurrently with chronic toxicity, because aspirin acetylates platelet cyclooxygenase and block the ability to make thromboxane A, an activator of platelet aggregation.
14
Q
- ) This is a cause for permanent deferral of a blood donor (2012, 2013)
a. upper respiratory infection
b. hepatitis B
c. fever
d. ingestion of contraceptive pill
A
B. The effects of A, C, and D are transient so you can pretty much cross all of them out. That leaves us with B. Har har.
15
Q
- ) Why is type O considered a universal donor?(2012, 2014)
a. it does not contain agglutinogens A and B
b. it does not contain anti A and B antibodies
c. it is the most common blood type
d. it is easy to procure
A
A. HPIM 16th ed, pp. 662-663
Type O individuals produce both anti-A and anti-B isoagglutinins, and are thus not recognized by any ABO isoagglutinins.
16
Q
- ) What is the purpose of doing a crossmatch before transfusion? (2012, 2013)
a. to detect autoantibodies present in the recipient
b. to prevent alloimmunization
c. to detect alloantibodies in the recipient
d. to avoid sensitization of the recipient
A
C. see explanation for #17
B. 2012’s answer
17
Q
- ) Which of the following blood components should have a crossmatch donor done before transfusion? (2014)
a. PRBC
b. platelets
c. WBC
d. fresh frozen plasma
A
A. HPIM 16th ed, p. 663
Cross-matching is ordered when there is a high probability that the patient will require a packed RBC (PRBC) transfusion. Blood selected for cross-matching must be ABO compatible and lack antigens for which the patient has alloantibodies. Non-reactive cross-matching confirms the absence of any major incompatibility and reserves that unit for the patient.
18
Q
- ) What is the reason why an Rh negative recipient should not receive an Rh positive blood? (2012, 2013, 2014)
a. Presence of incompatibility
b. Prevention of alloimmunization to D antigen
c. Prevention of immediate post transfusion reaction
d. Prevention of infection
A
B. Trans on “Blood Components, Transfusion Reactions, Autologous Transfusion”, p. 2 col. 2
An Rh negative patient who lacks anti-D may receive transfusions of Rh-positive blood in urgent situations where Rh-negative blood is unavailable. No immediate danger results from such a practice, but the patient may become alloimmunized to the D antigen and risk problems with pregnancy or transfusion in the future.
19
Q
- ) The most frequent cause of a febrile non-hemolytic transfusion reaction is (2014)
a. IgG protein in the transfused blood
b. ABO incompatibility
c. Presence of WBC and cytokines in the transfused blood
d. Presence of malarial parasite in the transfused blood
A
A. Trans on “Blood Components, Transfusion Reactions, Autologous Transfusion”, p. 6 col. 1
FNHTR is caused by the leukocyte antibodies present in the patient’s plasma, which are commonly directed against the antigens present on monocytes, granulocytes, or lymphocytes.
20
Q
- ) The “window period” in the testing for HIV in donor represents (2014)
a. the time from the infection of the donor up to the time that the antibody is detected
b. the duration of the HIV laboratory test
c. the incubation period of the reagents
d. the time when HIV symptoms became manifest
A
A. The window period is the time from infection until a test can detect any change. The average window period with antibody tests is 22 days. Antigen testing cuts the window period to approximately 16 days and NAT (Nucleic Acid Testing) further reduces this period to 12 days.
Taken from wikipedia
21
Q
- ) What is the optimum temperature for storing packed RBC? (2014)
a. 0 oC
b. room temperature
c. 4-6 oC
d. -20 oC
A
C. I can’t find this sa transes or HPIM for some reason but I distinctly remember na this was mentioned during the Blood Bank tour.
22
Q
- ) Which of the following is a ground for permanent donor deferment?
a. ingestion of antibiotics
b. ingestion of alcohol
c. fever
d. diabetes
A
D. Again, turn on your well-honed testmanship skills. A, B, C have transient effects whereas diabetes is a lifelong condition.
23
Q
- ) Thawed fresh frozen plasma (FFP) cannot be refrozen because (2012, 2014)
a. it is potentially infected
b. it has lost the activity of most of the coagulation factors
c. the plastic bag is already brittle
d. cytokines are released in the process of thawing
A
B. Mentioned during the Blood Bank tour.
24
Q
- ) The following screening tests are done in blood donors except (2015)
a. hemoglobin determination
b. Hepatitis A
c. Hepatitis B
d. Hepatitis C
A
B. Trans on “Blood Components, Transfusion Reactions, Autologous Transfusion”, p. 1 col. 2
Donor Evaluation includes: • Focused medical history • Limited physical examination • Lab testing for hematocrit and hemoglobin • Infectious disease testing for: o Malaria o Syphilis o Hepa B surface antigen (HBsAg) o Hepa B core antibody (anti-HBc) o Hepa C virus antibody (anti-HCV) o HIV-1 and HIV-2 antibody o HIV p24 antigen o HTLV-I and HTLV-II antibody o HIV and HCV genome (NAT)
25
25. ) In severe iron deficiency anemia with symptoms of high output failure, which is the best blood product for transfusion? (2014)
a. packed RBC
b. fresh whole blood
c. whole blood
d. heparinized whole blood
A. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 5 col. 2
Red cell transfusion is indicated for symptoms of anemia, cardiovascular instability, continued and excessive blood loss, and for immediate intervention.
26
26. ) Elderly patients who develop deep vein thrombosis should be worked up for (2012, 2014)
a. Vasculitis
b. Malignancy
c. Atherosclerosis
d. liver cirrhosis
B.
2010 smiley says atherosclerosis. 2011 beta feedback says malignancy. Journals say malignancy. Stick with malignancy. DVT may signal undiagnosed malignancy. (NEJM, 1992)
27
27. ) The most serious complication of deep vein thrombosis is (2012, 2013, 2014)
a. myocardial infarction
b. cerebrovascular thrombosis
c. peripheral vascular disease
d. pulmonary embolism
D.
HPIM 16th ed. P.1491: The most important consequence of this DVT is pulmonary embolism and the syndrome of chronic venous insufficiency.
28
28. ) Arterial thrombosis occurs when (2012)
a. there is an increase in the circulatory coagulation factors
b. prolonged immobilization
c. damage to the endothelium of blood vessels
d. increased synthesis of prothrombin
C.
Robbins, p.132: Arterial or cardiac thrombi usually begins at the site of endothelial injury (e.g. atherosclerotic plaque) or turbulence (vessel bifurcation).
29
29. ) The initiating event in the formation of arterial thrombus is (2012, 2013)
a. activation of Factor X
b. adherence of platelets to damaged blood vessels promoting aggregation
c. activation of anti-thrombin III
d. increased synthesis of prothrombin
B.
Robbins, p.132: Arterial or cardiac thrombi usually begins at the site of endothelial injury (e.g. atherosclerotic plaque) or turbulence (vessel bifurcation)…p.133: The thrombus is usually superimposed on an atherosclerotic plaque, although forms of vascular injury (vasculitis, trauma) may be involved.
30
30. ) Hyperviscosity of the blood can lead to thrombosis because
a. there is a slowing of blood flow in the circulation
b. there are more circulating coagulation factors
c. plasminogen activator is deficient
d. anti-thrombin III is deficient
A.
Hyperviscosity of the blood may be caused by increased plasma viscosity, increased RBC or WBC or due to decreased deformability of cells promoting stasis. Viscosity is the resistance to flow. (Hypercoagulable states trans: abnormalities in blood flow)
31
31. ) Why is diabetes a risk factor for developing thrombosis?
a. high blood sugar causes slowing of blood flow(2014)
b. there is an acquired Protein C deficiency
c. homocysteine is proportionately increased if blood sugar is elevated
d. diabetes is associated with endothelial damage
D.
Hypercoagulable states trans: acquired disorders causing thrombotic disorders
1. vascular disorders that promote damage to vascular wall: atherosclerosis, DM, vasculitis, prosthetis materials
2. abnormal rheology
3. platelet dysfunction
4. others
32
32. ) The most common leukemia in children is (2012, 2013, 2014)
a. Pre T ALL
b. Pre B ALL
c. AML
d. JMML
B.
Acute lymphoblastic leukemia is the most common malignancy in children
Early B acute lymphoblastic leukemia: 80%
T acute lymphoblastic leukemia: 20%
Mature B lymphoblast acute lymphoblastic leukemia: 1%
33
33. ) Finding on cytogenetics examination of the bone marrow
a. enables further characterization of leukemias into those with good vs poor prognosis
b. the finding of t(9:22) is seen exclusively in patients with CML
c. the finding of t(9:22) in patients with LL is associated with good prognosis
d. does not impact on chemotherapy and survival
A.
Pediatric hematologic malignancy trans
Diagnostic evaluation: Cytogenetics/FISH
Detects translocations for prognosis
34
34. ) The single most significant prognostic factor in children with ALL is
a. the presence of CNS involvement
b. extent of hepatomegaly
c. age at diagnosis
d. initial WBC at diagnosis
D.
```
Pediatric hematologic malignancy trans
Poor prognostic factors:
a. Age <100000/uL
c. Slow response to therapy
d. Hypoploidy of blasts
e. T(4;11)
f. Philadelphia gene
g. (-) TEL AML 1 gene rearrangement
h. Induction failure
```
35
35. ) Myelodysplastic syndrome is characterized by
a. hyperleukocytosis at diagnosis
b. signs and symptoms associated with pancytopenia
c. testicular involvement (for ALL, not MDS)
d. bone marrow aplasia
B.
Adult Hematologic Malignancies: MDS, a stem cell disorder, leads to pancytopenia in many cases and undergoes progression to acute leukemia. Also called oligoleukemia or preleukemia.
36
36. ) The Auer Rod
a. is pathognomonic for acute lymphoblastic leukemia
b. is seen in stacks with acute myelogenous leukemia without maturation
c. may be found in all forms of childhood leukemia
d. represents granules forming elongated needles seen in acute myelogenous leukemia
D.
Auer rods can be seen in the leukemic blasts of Acute Myeloid Leukemia. Auer rods are clumps of azurophilic granular material that form elongated needles seen in the cytoplasm of leukemic blasts.
37
37. ) Important “sanctuary” sites for acute lymphoblastic leukemia are (2012, 2014)
a. liver and spleen
b. lymph nodes and CNS
c. testes and CNS
d. Lymph nodes and liver
C.
Acute Leukemia manifestations of extramedullary invasion
a. CNS
b. testicular mass
c. enlarged kidneys
d. GI mass
e. bone and joint
38
38. ) Hematopoietic stem cell transplantation (2014)
a. is the only curative form of treatment for ALL
b. allows intensification of therapy and replacement of diseased marrow with normal precursors
c. utilizes hematopoeitic stem cells derived from fetal liver, bone marrow, and cord blood
d. is associated with less risk than conventional chemotherapy
B
39
39. ) The use of immunosuppressive agents in hematopoietic transplantation aims to
a. prevent graft rejection and graft versus host disease
b. prevent graft versus host disease only
c. directly destroy abnormal hematopoietic cells
d. prevent infections
A.
Allogeneic HSCT conditioned with triple agent immunosuppression, and specifically with high-dose stem cell return is probably an effective treatment for successful engraftment (Prevent rejection).
40
40. ) The use of high dose chemotherapy and / or radiotherapy as a preparative regimen for hematopoietic stem cell transplantation aims to.
a. eradicate primary disease
b. prevent graft rejection
c. directly destroy abnormal hematopoietic cells
d. prevent infections
A.
The potential role of intensive immunosuppression and hematopoietic stem cell transplantation in the treatment of severe autoimmune diseases has been evaluated for several years.
41
41. ) Immunophenotyping
a. involves characterization of blast cells by identifying cell surface antigens
b. is less sensitive and specific compared to immunochemical staining
c. need not be performed with blast morphology is definite
d. can be performed on bone marrow samples only
A.
A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system.
42
42. ) Rouleaux formation is seen in (2012, 2014 )
a. Afibrinogenemia
b. Paraproteinemia
c. sickle cell anemia
d. autoimmune hemolytic anemia
B.
Hemopathology Microscopy trans: Rouleaux formation, the linear alignment of at least 4 RBCs in a thin area of a blood smear resembling a stack of coins, is caused by changes in the surface charge of the erythrocyte membrane when this membrane is coated with excessive amounts of protein such as globulins and fibrinogen. The most common cause of Rouleaux formation, however, is paraproteinemia due to a monoclonal gammopathy.
43
43. ) Microcytosis is best defined as (2014)
a. high RDW
b. low MCV
c. high MCH
d. low MCHC
B.
Microcytosis - also known as or related to microcytic anemia, mcv, mean cell volume reduced
44
44. ) On a peripheral blood smear, red cells will normally have a central pallor which occupies (2012, 2014)
a. 1/3 of its diameter
b. ½ of its diameter
c. 2/3 of its diameter
d. ¾ of its diameter
A.
Hemopathology Microscopy trans: The peripheral blood smear of a normal individual contains normochromic, normocytic, RBCs with central pallor occupying only 1/3 of the cell diameter.
45
45. ) A neoplasm of immunosecreting terminally differentiated B lymphocytes is
a. B-cell chronic lymphocytic leukemia
b. B-cell acute lymphocytic leukemia
c. multiple myeloma
d. Chronic myelogenous leukemia
A.
Hemopathology Microscopy trans: neoplasm of terminally differentiated or mature B lymphoid cells is chronic lymphocytic leukemia showing lymphoid aggregates or infiltrates
46
46. ) The definitive diagnosis of chronic myelogenous leukemia is based on the demonstration of
a. marrow hyperplasia
b. leukocytosis with immature cells
c. megakaryocytic proliferation
d. Philadelphia chromosome
D.
Hemopathology Microscopy trans: Cytogenetic evaluation of CML showing Philadelphia chromosome, a defining characteristic to differentiate from other chronic myeloproliferative disorders.
47
47. ) An example of a neoplasm involving more than one cell line is
a. aplastic anemia
b. multiple myeloma
c. acute lymphoblastic leukemia
d. chronic myelogenous leukemia
D. Hemopathology Microscopy trans: CML shows granulocytic and megakaryocytic hyperplasia
48
48. ) According to the WHO classification, the diagnosis of AML requires the presence of ___ blasts in the marrows (2012, 2013, 2014)
a. > 10%
b. > 20%
c. > 30%
d. > 40%
B. Hemopathology Microscopy trans: bone marrow smear shows greater than or equal to 20% blasts
49
49. ) The clinical manifestation that distinguishes aplastic anemia from leukemia (2014)
a. severity of pallor
b. severity of bleeding
c. CNS involvement
d. presence of hepatosplenomegaly
D.
The symptoms of aplastic anemia are much like leukemia; increased incidence of infections, bleeding and bruising (because of low platelets), and fatigue and shortness of breath due to anemia. This disease can occur at any age. Physical exam will be nonspecific. The patient may look quite ill, secondary to an infection, or may just have some bruises on the skin. The liver and spleen are not usually enlarged as they may be in acute leukemia.
Aplastic anemia trans: lymphadenopathy and splenomegaly are highly atypical of AA.
50
50. ) Acquired aplastic anemia in contrast to inherited form of bone marrow failure appears to be caused largely by (2012, 2014)
a. immune mediated destruction of marrow cells
b. loss of hematopoietic stem cells due to DNA damage
c. low threshold for apoptosis
d. NOTA
A. Aplastic anemia trans: acquired idiopathic AA is due to immune mediated destruction of marrow cells or via primary stem cell defects.
51
51. ) This disease condition can co-exist with aplastic anemia
a. myelodysplastic syndrome
b. paroxysmal nocturnal hemoglobinuria
c. acute leukemia
d. myelofibrosis
Answer: B
AA may be primary or secondary to PNH. Ham’s test (checks whether the RBC becomes more fragile when placed in mild acid) and Sugar Lysis test (more sensitive) need to be done in patients with AA, as positive results in these tests would point towards AA secondary to PNH
(Topic Conference: Aplastic Anemia trans, p. 1, column 2).
52
52. ) An infectious state that is occasionally associated with bone marrow failure is
a. varicella zoster infection
b. hepatitis
c. strep throat
d. typhoid fever
Answers: B, C
Viral infections that can result in acquired aplastic anemia include Hepatitis B and infectious mononucleosis , which is caused by the Epstein –Barr Virus (Bone Marrow Failure Syndromes Trans, page 1, column 2). Pure red cell aplasia may result from parvovirus infection, while acquired pure amegakaryocytic thrombocytopenic purpura is associated with hepatitis, parvo, HIV and measles. Acute and chronic bacterial infections can lead to anemia of chronic disease
(Bone Marrow Failure Syndromes Trans, page 3, column 2).
53
53. ) The following are the most important variables in disease outcome in myelodysplastic syndrome except (2014)
a. number of blasts in peripheral blood
b. number of blasts in the bone marrow
c. cytogenetic abnormality
d. severity of cytopenia
Answer: A
Bone Marrow Blast Count, Karyotype and Cytopenias (presence/number) are all part of the International Prognostic Scoring System for MDS. Percentage of blasts in peripheral blood is used to classify MDS according to the French-American-British system
(Bone Marrow Failure Syndromes Trans, page 5, column 1)
54
54. ) Anemia of chronic renal failure (2012, 2013, 2014)
a. usually microcytic normochromic
b. erythropoietin deficiency is usually seen when creatinine is > 2 mg/dL
c. supplemental iron should be avoided as much as possible
d. erythropoietin alpha dose of 50-75 U/kgbw per week is usually required.
Answer: B
A is wrong because the anemia of chronic renal disease is normocytic and normochromic. Microcytic (initially normocytic) anemia is seen in acute and chronic inflammatory states (Bone Marrow Failure Syndromes trans, P. 4, column 2). C is wrong because iron supplementation is necessary to ensure adequate response to erythropoietin administration in chronic renal disease
(HPIM p. 1658).
55
55. ) Anemia of chronic disease (2012)
a. reticulocyte count is usually normal or increased
b. iron level is normal
c. iron binding capacity is increased
d. ferritin value is elevated
Answer: D
In anemia of chronic disease, reticulocyte counts are decreased, iron levels are decreased (30 mcg/mL, versus a normal value of 70-90 mcg/mL) and total iron binding capacity is decreased (200, versus a normal value of 250-400). The normal value for ferritin is 20-220. The mean value for serum ferritin in ACD is 150. In inflammatory states, serum ferritin is increased
(Bone Marrow Failure Syndromes Trans,P.4, column 2).
56
56. ) The proposed mechanisms for anemia of chronic inflammatory condition include (2012, 2014)
a. increased red cell survival
b. decreased responsiveness to erythropoietin
c. decreased levels of erythropoietin
d. all of the above
Answer: B
Anemia of chronic disease is associated with reduced erythropoietin response
(Bone Marrow Failure Syndromes Trans,P.3, column 2).
57
57. ) The life span of platelets is (2014)
a. 12 to 24 hours
b. 7-10 days
c. 45-60 days
d. 100-120 days
Answer: B
The life span of a platelets is 8 days
(HPIM, p. 2380).
58
58. ) The key organ in the pathophysiology of chronic immune thrombocytopenic purpura is (2012, 2013)
a. bone marrow
b. liver
c. spleen
d. kidneys
Answer: C
In Chronic ITP, there is production of antibodies to platelets (specifically directed to Gp IIb/IIIa). The antibody-coated platelets attach to Fc receptors in macrophages in the spleen , and are subsequently phagocytosed. A splenectomy is performed isf te patient is unresponsive to steroids, in order to remove the site of antiplatelet antibody production and platelet destruction
(Acquired Bleeding Disorders trans, pp.2-3).
59
59. ) A chronic ITP patient failed to respond to splenectomy. The presence of an accessory spleen is suspected by the presence of this abnormal red cell in the peripheral blood smear (2013, 2014)
a. Howell-Jolly bodies
b. target cells
c. tear-drop cells
d. histiocytes
Answer: A
Howell-Jolly bodies, seen through Wright’s stain, are nuclear inclusions. Target Cells are seen in obstructive jaundice, post-splenectomy state, thalassemia and hemoglobin c disease. Tear drop cells are seen in myelofibrosis. Histiocytosis is seen in certain inflammatory disorders and malignancies
(Hemopathology Microscopy- additional notes- trans).
60
60. ) This screening test reflects the function of the intrinsic pathway of coagulation
a. bleeding time
b. prothrombin time
c. partial thromboplastin time
d. thrombin time
Answer: C
The intrinsic pathway involves factors XII, XI, IX, VIII, while the extrinsic pathway involves factor VII (Acquired Bleeding Disorders trans, p1 column 2). Partial thromboplastin time measures the initiation of clotting at the level of factor XII through sequential steps to the final plot end point. It does not, however, measure factor VII, factor XIII or anticoagulants. Bleeding time assesses the function of platelets and their interaction with the vascular wall. Prothrombin time measures the extrinsic clotting system after activation of clotting by tissue factor in the presence of calcium. Thrombin Time measures the final step of the clotting cascade, in which fibrinogen is converted to fibrin. A (hopefully) useful mnemonic: pEt (prothrombin time- extrinsic) and pItt (partial thromboplastin time- intrinsic)
(Congenital Bleeding Disorders trans, p.1, column 2)
61
61. ) Which factor is not a vitamin K dependent coagulation factor (2013)
a. factor I
b. factor II
c. factor VII
d. factor IX
e. factor X
Answer: A
Factors II, VII, IX and x are vitamin K-dependent factors produced in the liver
(Congenital Bleeding Disorders Trans, p. 4, column 1)
62
62. ) Vitamin K deficiency causes abnormality of this screening test (2013, 2014)
a. bleeding time
b. platelet count
c. prothrombin time
d. partial thromboplastin time
Answer: C
Prothrombin time is used to test factor VII, which is a vitamin K dependent coagulation factor
(Congenital Bleeding Disorders Trans, p. 4, column 1)
63
63. ) One of the following statements regarding treatment of DIC is not correct (2013)
a. correct any reversible cause
b. fresh frozen plasma and platelet concentrates are given for actively bleeding patients
c. heparin is used for patients with acrocyanosis and incipient gangrene
d. warfarin is effective for chronic DIC
Answer D
Management of DIC includes treating the underlying cause (abruptio placenta → prompt delivery of fetus and placenta, sepsis → antibiotics)and transfusion of platelets (for thrombocytopenia) or plasma (replacement of clotting factors). Anticoagulants may be used if the predominant presentation is thrombosis. No mention of warfarin
(Acquired bleeding disorders trans, pp.4-5)
64
64. ) Abnormal red cell morphology seen in DIC due to a microangiopathic hemolytic anemia (2014)
a. spherocytes
b. target cells
c. dacrocytes
d. schistocytes
Answer: D
Spherocytes are (obviously)nearly spherical, smaller in diameter and lack the central pallor. They are seen in hereditary spherocytosis, autoimmune hemolytic anemia, and direct physical or chemical injury (eg. Burns). Target cells are thinner than normal, with a dark-staining central hemoglobin containing area. They are seen in obstructive jaundice, post-splenectomy state, thalassemia and hemoglobin C disease. Dacrocytes are also known as tear drop cells and are seen in myelofibrosis. Schistocytes are fragments of red blood cells, and they signify hemolysis
(Hemopathology Microscopy- additional notes- trans pp.1-2).
65
65. ) This is true regarding bleeding disorder secondary to aspirin (2013)
a. aspirin impairs platelet release reaction
b. a severe bleeding disorder
c. aspirin causes reversible inhibition of cyclooxygenase enzyme
d. impaired hemostasis reverses in 24 hours
Answer: A
This is weird. Platelets release proaggregatory materials in order to recruit additional platelets to the growing plug (emedicine, Platelet Disorders). Aspirin inhibits prostacyclin formation. Prostacyclin is an antiaggregating and vasodilating prostaglandin, which means that aspirin promotes aggregation. C and D are wrong because aspirin causes irreversible inhibition of COX, an affect that lasts for the 8-day life span of the platelets
(HPIM, p. 2380)
66
66. ) A 20 year old male had laboratory tests done because of prolonged bleeding following dental extraction. Results were as follows: normal platelet count, bleeding time and prothrombin time, but prolonged PTT which was corrected by mixing test. The most likely diagnosis is:
a. von willebrand’s disease
b. hemophilia
c. chronic ITP
d. Glanzmann’s thrombasthenia
Answer: B.
Normal platelet count, bleeding time and prothrombin time with prolonged PTT is characteristic of hemophilia. PTT tests for almost all factors (except factors VII and XIII), including factors VIII and IX, either of which may be lacking in hemophilia (Congenital Bleeding Disorders Trans, pp1-2).
A is wrong because bleeding time and PTT are prolonged in Von willebrand’s disease, a hereditary disorder of the vWF molecule (responsible for the adherence of platelets to damaged endothelium), although PTT can be normal in type 1 vWD (Congenital Bleeding Disorders Trans, p. 5, column 1).
C is wrong because platelet counts would be low in chronic ITP (Acquired Bleeding Disorders trans, p. 3, column 1).
Glanzmann’s thrombasthenia results from a deficiency in the Gp IIb/IIIa complex. Platelets do not aggregate, resulting in lifelong bleeding (emedicine, Platelet Disorders), making D wrong as well
67
67. ) A 30 year old male is suspected of having aplastic anemia. The expected bone marrow finding is
a. hypocellular marrow with 20% myeloblasts
b. hypocellular marrow with increased lymphocytes and plasma cells
c. granulocytic hyperplasia with all stages of myeloid maturation seen
d. megakaryocytic hyperplasia
Answer: B
In aplastic anemia, the marrow is hypocellular and fat-laden. (Hemopathology Microscopy trans, p.1, column 2). Although the marrow is largely devoid of hematopoietic cells, there may be scattered lymphocytes and plasma cells (Hemopathology Microscopy trans, p.4, column 2- see picture). The answer is not A because the presence of 20% myeloblasts in the bone marrow indicates acute myeloid leukemia (Hemopathology Microscopy trans, p. 2, column 2). C is suggestive of chronic myelogenous leukemia. This condition can also be accompanied by megakaryocytic hyperplasia
(Hemopathology Microscopy trans, p.3- may pictures ulit).
68
68. ) A 30 year old male diagnosed to have aplastic anemia 5 years ago gradually developed jaundice, pallor, reddish urine especially in the morning. Urinalysis was negative for red blood cells. The most helpful diagnostic test would be.
a. bone marrow aspiration and biopsy
b. leukemia immunophenotying
c. urine hemosiderin determination
d. flow cytometry for CD55, CD59
Answer: D
CD55, also known as Decay Accelerating Factor, and CD59, also known as membrane inhibitor of reactive lysis, are examples of complement regulating surface proteins. These proteins interact with complement in order to dissociate the convertase complexes of the classic and alternative pathways, as well as halt the amplification of the activation process. The absence of these proteins would result in the destruction of blood cells, as in PNH, a condition associated with Aplastic Anemia
(emedicine, PNH).
69
69. ) The underlying pathophysiology for paroxysmal nocturnal hemoglobinuria is (2014)
a. hereditary
b. lack of glycosyl phosphatidyl inositol-linked cell surface membrane proteins
c. tumor necrosis factor and gamma interferon elaborated by cytotoxic T cells
d. presence of red cell antibodies
Answer: B
PNH results from a genetic mutation that leads to the inability to synthesize the glycosyl phosphatidyl inositol anchor that links surface proteins to cell membranes
(emedicine, PNH).
70
70. ) Apheresis can be used to collect all of the following except (2014)
a. leukocytes
b. macrophages
c. hematopoietic progenitor cells
d. platelets
Answer: B
The types of apheresis are (from the Block A Blood Transfusion trans, p. 3, column 2)
• Plasmapheresis – removal of plasma from blood
• Plateletpheresis – equivalent to 6-10 random units
• Erythrocytapheresis – removal of RBCs from blood
• Leukapheresis – contain a minimum of 1.0 x 1010/L
• Hematopoietic progenitor cell removal and collection of hematopoietic progenitor cells from the peripheral blood
• Therapeutic pheresis
o Therapeutic cytapheresis i.e. Thrombocytopheresis
o Therapeutic plasmapheresis (Plasma Exchange)
71
71. ) Patients at greatest risk of developing transfusion associated circulatory overload may include:
a. children
b. elderly people
c. patients with chronic normo-volemic anemia
d. all of the above
Answer: D
From the Block A Blood Transfusion trans:
Population at Risk for TA-GVHD
• Lymphopenic patients
• Those on bone marrow suppression
• Fetus receiving intrauterine transfusions
• Newborn receiving exchange transfusion
• Congenital immunodeficiency syndromes
• Those with hematologic and oncologic disorders
• Patients receiving blood components from relatives
• HLA types with haplotypes identical to donors
72
72. ) Fatal transfusion reactions are most frequently caused by (2012, 2013, 2014)
a. clerical errors
b. improper refrigeration
c. overheated blood
d. mechanical trauma
Answer: A
This was mentioned verbatim in one of our lectures, although the Block A Blood Transfusion trans mentions the following:
Some Typical Causes of Transfusion-Associated Deaths
• Acute hemolysis (ABO-incompatible blood components)
• Acute pulmonary edema
• Bacterial contamination of product
• Delayed hemolytic reactions
• Anaphylaxis
• External hemolysis (temp >40oC)
• Acute hemolysis; damaged blood components (non-deglycerolized, improper solution)
• Transfusion-Associated Graft Versus Host Disease (TA-GVHD)
```
Location of Preventable Transfusion-Associated Deaths (In Order of Occurrence)
Laboratory
Nursing Service
Anesthesia Service
Clinical Staff
```
73
73. ) When a suspected hemolytic reaction occurs, the first thing to do is (2012, 2013, 2014)
a. slow the transfusion rate and call the physician
b. administer medication to stop the reaction
c. stop the transfusion but keep the intravenous line open with saline
d. first inform the laboratory to begin an investigation
Answer: C
This was stated in the management of the various types of hemolytic transfusion reactions
(Block A Blood Transfusion trans).
74
74. ) Pre transfusion irradiation of all blood products in certain patients is done to prevent which of the following
a. cytomegalovirus
b. transfusion associated graft vs. host disease
c. febrile non hemolytic transfusion reaction
d. hemolytic transfusion reaction
Answer: B (feedback answer: C)
Irradiation would prevent both CMV and GVHD (Block A Neonatal transfusion trans, p. 1, column 2), but the answer given during the feedback was C.
75
75. ) What describes the clonal cell in multiple myeloma? (2012, 2014)
a. bilobed nucleus with prominent eosinophilic nucleoli
b. large mononuclear cell, fine chromatin material with minimal cytoplasm
c. round cell with round indented nucleus and perinuclear clearing
d. small round cell with clumped chromatin material and minimal to moderate cytoplasm.
Answer: C
The cell in multiple myeloma is a large, mononuclear cell with an indented nucleus and perinuclear clearing (Adult Hematologic Malignancies trans, p. 4, column 2) . B is characteristic of Acute Lymphoblastic Leukemi, while D is characteristic of Chronic Lymphocytic Leukemia
(Hemopathology Microscopy trans, p. 4, column 1).
76
76. ) What is included in the “B symptoms” of lymphoma? (2012, 2013 )
a. Pruritus
b. Jaundice
c. Weight Loss
d. Anorexia
Answer: C
B-symptoms are a group of symptoms that may be present in individuals with lymphoma. There may be a variety of symptoms in lymphoma patients. The most common is the enlargement of lymph nodes. Apart from this, a set of 3 symptoms are considered very important in identifying how the cancer is likely to behave. These are:
• Fever (i.e., temperature >38°C [>100.4°F]) for 3 consecutive days
• Weight loss exceeding 10% of body weight in 6 months
• Drenching night sweats
These are called the B-symptoms. The presence of one or more of these symptoms increases the likelihood that the disease may be present in many parts of the body even though it may not be identifiable using usual methods of testing.
77
77. ) Which anemic patient with high creatinine likely has multiple myeloma? (2012, 2014)
a. Normal serum calcium and high uric acid
b. High serum calcium and lytic lesions on bone scan
c. > 30% plasma cell on bone marrow aspirate and lytic lesions on skeletal survey
d. 10% plasma cell on bone marrow aspirate and elevated serum Ig
Answer: C (HPIM 16th Ed. p. 734)
The classic triad of myeloma is marrow plasmacytosis (>10%), lytic bone lesions, and a serum and/or urine M component. There are two important variants of myeloma, solitary bone plasmacytoma and extramedullary plasmacytoma. These lesions are associated with an M component in fewer than 30% of the cases, they may affect younger individuals, and both are associated with median survivals of 10 or more years. Solitary bone plasmacytoma is a single lytic bone lesion without marrow plasmacytosis. Extramedullary plasmacytomas usually involve the submucosal lymphoid tissue of the nasopharynx or paranasal sinuses without marrow plasmacytosis. Both tumors are highly responsive to local radiation therapy.
78
78. ) Prognosis is best correlated with that in Multiple Myeloma? (2012, 2014)
a. CRP and B microglobulin
b. LDH and serum creatinine
c. ESR and LDH
d. serum calcium and LDH
Answer: A
Serum ß2-microglobulin is the single most powerful predictor of survival and can substitute for staging. Patients with ß2-microglobulin levels 0.004 g/L only 12 months. It is also felt that once the diagnosis of myeloma is firm, histologic features of atypia may also exert an influence on prognosis. Other factors that may influence prognosis are the number of cytogenetic abnormalities, chromosome 13q deletion, % plasma cells in the marrow, circulating plasma cells, performance status, and serum levels of IL1-6, soluble IL-6 receptors, C-reactive protein, hepatocyte growth factor, C-terminal cross-linked telopeptide of collagen I, TGF-ß, and syndecan-1.
(HPIM 16th Ed. p. 734)
79
79. ) Lymphoma and Multiple Myeloma are disorder of what lineage? (2012, 2014)
a. Myeloid
b. Lymphoid
c. Unknown
d. Stromal Cells
Answer: B
Lymphoma is a type of cancer that originates in lymphocytes (a type of white blood cell in the vertebrate immune system). There are many types of lymphoma. Lymphomas are part of the broad group of diseases called hematological neoplasms.
(Wikipedia)
80
80. ) The diagnostic test for the thalassemias is (2012, 2014)
a. Autohemolyis test
b. Coombs’ Test
c. Hemoglobin Electrophoresis
d. Osmotic Fragility Test
Answer: C
Of the many methods available for hemoglobin analysis, electrophoretic techniques are used for routine clinical purposes. Electrophoresis at pH 8.6 on cellulose acetate membranes is especially simple, inexpensive, and reliable for initial screening. Agar gel electrophoresis at pH 6.1 in citrate buffer is often used as a complementary method because each method detects different variants. Comparison of results obtained in each system usually allows unambiguous diagnosis, but some important variants are electrophoretically silent. These mutant hemoglobins can usually be characterized by more specialized techniques such as isoelectric focusing and/or high pressure liquid chromatography (HPLC).
(HPIM 16th Ed. p. 652)
81
81. ) The definitive diagnostic procedure for Hereditary Spherocytosis is (2012, 2014)
a. Hgb electrophoresis
b. Ham’s Test
c. Osmotic Fragility Test
d. Coomb’s Test
Answer: C
Trans on “Congenital Hemolytic Anemia” p. 1 col. 1
Kindly check the nice algorithm for diagnosis of Hemolytic Anemia.
82
82. ) Which treatment option offers a clinical cure for hereditary spherocytosis? (2012, 2014)
a. PRBC transfusion
b. Splenectomy
c. Folic Acid
d. Iron chelatation
Answer: B
Splenectomy is recommended in patients with moderate or severe hemolysis. Although the RBC1 defect and its consequent morphology persist, anemia is ameliorated. The operative risk is low, particularly if performed by laparoscopy. RBC survival after splenectomy is normal or nearly so; if it is not, an accessory spleen or another diagnosis should be sought. Because of the potential for gallstones and for episodes of bone marrow hypoplasia or hemolytic crises, splenectomy should be performed in symptomatic individuals; cholecystectomy should not be performed without splenectomy, as intrahepatic gallstones may result. Splenectomy in children should be postponed until age 4, if possible, to minimize the risk of severe infections with gram-positive encapsulated organisms. Pneumococcal, meningococcal, and Haemophilus influenzae vaccines should be administered at least 2 weeks before splenectomy. In patients with severe hemolysis, folic acid (1 mg/d) should be administered prophylactically.
(HPIM 16th Ed. p. 669)
83
83. ) In Beta thalassemia major, this Hgb is markedly elevated (2012, 2014)
a. Hb A1
b. Hb A2
c. Hb H
d. Hb F
Answer: D
Severity is highly variable. Known modulating factors are those that ameliorate the burden of unpaired a-globin inclusions. Alleles associated with milder synthetic defects and coinheritance of a-thalassemia trait reduce clinical severity by reducing accumulation of excess a globin. HbF persists to various degrees in ß thalassemias. ?-Globin gene chains can substitute for ß chains, simultaneously generating more hemoglobin and reducing the burden of a-globin inclusions. The diagnosis of ß-thalassemia major is readily made during childhood on the basis of severe anemia accompanied by the characteristic signs of massive ineffective erythropoiesis: hepatosplenomegaly, profound microcytosis, a characteristic blood smear (Fig. 91-5), and elevated levels of HbF, HbA2, or both.
(HPIM 16th Ed. p. 652)
84
84. ) The clinical triad of hemolytic anemia does not include: (2014)
a. Splenomegaly
b. Hepatomegaly
c. Jaundice
d. Pallor
B
85
85. ) The objective of hypertransfusion and supertransfusion in thalassemia major is (2012, 2014)
a. to increase the patient’s Hgb level
b. to increase patient’s RBC in the blood
c. to increase the patients iron stores
d. to suppress erythropoiesis
Answer: D
The diagnosis of ß-thalassemia major is readily made during childhood on the basis of severe anemia accompanied by the characteristic signs of massive ineffective erythropoiesis: hepatosplenomegaly, profound microcytosis, a characteristic blood smear (Fig. 91-5), and elevated levels of HbF, HbA2, or both. Many patients require chronic hypertransfusion therapy designed to maintain a hematocrit of at least 27 to 30% so that erythropoiesis is suppressed. Splenectomy is required if the annual transfusion requirement (volume of RBCs per kilogram of body weight per year) increases by >50%. Folic acid supplements may be useful. Vaccination with Pneumovax in anticipation of eventual splenectomy is advised, as is close monitoring for infection, leg ulcers, and biliary tract disease.
(HPIM 16th Ed. p. 652)
86
86. ) In tumor lysis syndrome, what is the metabolic imbalance encountered? (2012,2014)
a. inc K, low PO4, low Ca, High uric acid
b. inc. K, High Po4, High Ca, High uric acid
c. inc. K, High Po4, low Ca, high Uric acid
d. dec. K, High Po4, low Ca, High Uric acid
Answer: C
Tumor lysis syndrome (TLS) is a very serious and sometimes life-threatening complication of cancer therapy. It can be defined as a constellation of metabolic abnormalities resulting from spontaneous or treatment-related tumor necrosis or fulminant apoptosis. The metabolic abnormalities observed in patients with tumor lysis syndrome include hyperkalemia, hyperuricemia, and hyperphosphatemia with secondary hypocalcemia. These can lead to acute renal failure (ARF). The main principles of TLS are the identification of high-risk patients, initiation of preventive therapy, and early recognition and intervention of its complications.
(Emedicine)
87
87. ) In chronic lymphocytic leukemia, the proliferating cells in the blood are: (2014)
a. large with round nuclei, prominent nucleoli and scant cytoplasm
b. large indented nuclei, prominent nucleoli and abundant cytoplasm
c. small irregular nuclei, inconspicuous nucleoli and vacuolated cytoplasm
d. small with round nuclei, inconspicuous nucleoli and scant cytoplasm
D
88
88. ) Red cell hypochromasia is seen only in: (2014)
a. myelodysplastic syndrome
b. sickle cell disease
c. spherocytosis
d. polychythemia
A
89
89. ) At birth fetal red cells contain 53 to 95% (2012, )
a. Hb A1
b. Hb A2
c. Hb F
d. Hb H
Answer: C
After the first 10 to 12 weeks of development, the fetus' primary form of hemoglobin switches from embryonic hemoglobin to fetal hemoglobin. At birth, fetal hemoglobin comprises 50-95% of the child's hemoglobin. These levels decline after six months as adult hemoglobin synthesis is activated while fetal hemoglobin synthesis is deactivated. Soon after, adult hemoglobin (hemoglobin A in particular) takes over as the predominant form of hemoglobin in normal children.
(Wikipedia)
90
90. ) The only indication in fresh whole blood in neonates is: (2014)
a. hemodialysis
b. exchange transfusion
c. hypovolemic shock
d. uremia
B
91
91. ) Choice of blood for exchange transfusion in ABO incompatibility: (2014)
a. Type O Rh positive PRBC
b. Type-specific Rh positive FWB
c. Type O Rh positive FWB
d. Type-specific Rh positive PRBC
C
92
92. ) A hemophilic baby should be delivered by: (2013, 2014)
a. normal vaginal delivery
b. caesarian section
c. outlet forceps extraction
d. vacuum extraction
B
93
93. ) Prevention of hemorrhagic disease of the newborn is achieved by: (2013)
a. cryosupernate transfusion at birth
b. fresh frozen plasma transfusion at birth
c. cow’s milk formula
d. routine Vit K prophlaxis at birth
Answer: D
The more appropriate term for hemorrhagic disease of newborn is vitamin K deficiency bleeding (VKDB). Historically, all bleeding disorders in the newborn were grouped together under the diagnosis of hemorrhagic disease of the newborn (HDN). With methods available today for the accurate diagnosis of other factor deficiency states and immune thrombocytopenias, VKDB can be distinguished from other disorders by exclusion and appropriate analysis of these other factors involved in coagulation. Prevention of VKDB with intramuscular vitamin K is of primary importance in medical care. A single dose of intramuscular vitamin K after birth effectively prevents classic VKDB. While oral vitamin K prophylaxis improves coagulation tests at 1-7 days, it has not been tested in randomized trials for its effect on either classic or late VKDB.
(Emedicine).
94
94. ) Treatment of bleeds in Von Willebrand’s Disease: (2014)
a. platelet concentrates
b. stored plasma
c. Cryoprecipitates
d. Cryosupernates
Answer: C
FRESH-FROZEN PLASMA
FFP16 contains stable coagulation factors and plasma proteins: fibrinogen, antithrombin, albumin, as well as proteins C and S. Indications for FFP17 include correction of coagulopathies, including the rapid reversal of warfarin; supplying deficient plasma proteins; and treatment of thrombotic thrombocytopenic purpura. FFP18 should not be routinely used to expand blood volume. FFP19 is an acellular component and does not transmit intracellular infections, e.g., CMV20. Patients who are IgA-deficient and require plasma support should receive FFP21 from IgA-deficient donors to prevent anaphylaxis (see below).
95
The blood component of choice for Hemophilia B is:
a. Fresh Frozen plasma
b. Cryoprecipitates
c. Cryosupernates
d. Platelet concentrates
C
96
[Blood Type]
Anti-A Testing: +
Anti-B Testing: -
Type A
97
[Blood Type]
Anti-A Testing: -
Anti-B Testing: +
Type B
98
[Blood Type]
Anti-A Testing: -
Anti-B Testing: -
Type O
99
[Blood Type]
Anti-A Testing: +
Anti-B Testing: +
Type AB
100
101. In the presence of prolonged neonatal jaundice, with exposure to naphthalene balls, one should consider:
a. G6PD Deficiency
b. Autoimmune Hemolytic Anemia
c. Thalassemia Major
d. Neonatal Hepatitis Syndrome
A
101
101. In the presence of prolonged neonatal jaundice, with exposure to naphthalene balls, one should consider:
a. G6PD Deficiency
b. Autoimmune Hemolytic Anemia
c. Thalassemia Major
d. Neonatal Hepatitis Syndrome
D.
Thalassemia is a Congenital Hemolytic Anemia presenting with a hemoglobin defect. A silent carrier is hematologically normal. Patient with thalassemia trait has mild anemia (microcytosis and hypochromia). Thalassemia Intermedia presents with a benign clinical course. Thalassemia Major exhibits the clinical triad of CHA, hepatomegaly, craniofacial changes, stunting growth and is diagnosed at 6mos-2yrs of age via electrophoresis. (CHA Trans p3)
102
103. The 4 allele deletion in Alpha Thalassemia that is incompatible with life is:
a. Silent Carrier
b. Thalassemia Trait
c. Hemoglobin H Disease
d. Hydrops Fetalis
D.
Silent carrier -1 allele lost; Thalassemia Trait -2 alleles lost; Hb H disease -3 alleles lost; Hydrops fetalis -4 alleles lost (CHA Trans p3)
103
104. What risk do neonates have when they receive directed donations from first degree relatives:
a. Acute hemolytic reaction
b. Transfusion-associated GVHD
c. Febrile reaction
d. Allergic reaction
B.
There is a higher probability of graft vs. host from a direct relative because they share the same Hla A. This may react with the baby’s immune system. (Transfusion in Neonates Trans)
104
105. Which one is not an indication for exchange transfusion in neonates?
a. Rh incompatibility
b. Iron Deficiency Anemia
c. ABO incompatibility
d. Sepsis
B. Exchange Transfusions indications: Rh incompatibility, ABO incompatibility, DIC, Sepsis, Progressive Hyperbillirubinemia (Transfusion in Neonates Trans)
105
Which statement is not true regarding transfusion in neonates?
a. The antibody-producing mechanisms and cellular immune system of neonates are mature.
b. Hypothermia in the newborn causes adverse metabolic effects.
c. Transfusion in neonates is different and unique.
d. The newborn does not compensate for hypovolemia as well as an adult.
A. The antibody-producing mechanisms and cellular immune system of neonates are immature. The only antibody present in the neonate is the IgG. (Transfusion in Neonates Trans)
106
106. Indication to irradiate blood:
a. Third degree relative blood donor
b. History of frequent allergic reactions to blood transfusion
c. History of febrile non-hemolytic transfusion reactions
d. Blood donor with history of asthma
A
107
107. These tests may be most informative in diagnosing Paroxysmal Nocturnal Hemoglobinuria:
a. Ham’s Test and Sucrose Lysis Test
b. Immunophenotyping and FISH
c. Erythropoietin Assay and Coomb’s Test
d. Chromosomal Analysis
A.
An acquired mutation in the PIG-A gene in a hematopoietic stem cell is required for the development of PNH, but PIG-A mutations probably occur commonly in normal individuals. If the PIG-A mutant stem cell proliferates, the result is a clone of progeny deficient in glycosylphosphatidylinositol-linked cell surface membrane proteins. Such PNH cells are now accurately enumerated using fluorescence-activated flow cytometry of CD55 or CD59 expression on granulocytes rather than Ham or sucrose lysis tests on red cells. (HPIM Ch102)
108
108. Site of maximum iron absorption:
a. Stomach
b. Duodenum
c. Distal ileum
d. Colon
B
109
109. A 6 year old child with 4 year history of easy bruisability is brought to PGH with multiple purpuras on both shins and forearms. The rest of the PE is unremarkable. You think it is normal bruising of childhood, but the parents are concerned. If bleeding is spontaneous, what would you expect to see?
a. Bruise out of proportion to degree of trauma
b. Bleeding from a cut lasting 30 seconds
c. Hematomas after immunizations
d. A and C
D
110
110. You decide to order screening tests. Which one will you order?
a. CBC, Platelets, Clotting Time, Bleeding Time, Clot Retraction Time
b. CBC, Platelets, Prothrombin Time, Partial Thromboplastin Time
c. CBC, Platelets, Bleeding Time
d. CBC, Platelets
B
111
111. Hemophilia A is:
a. X-Linked
b. Autosomal Dominant
c. Autosomal Recessive
d. Not a familial disease
A
112
112. 15 year old boy with LLQ pain; HR 120 bpm; BP 80/60. Family history includes 2 uncles who died of a bleeding disorder. Your differential diagnosis would include the following except:
a. Acute appendicitis
b. Urinary Tract Infection
c. Iliopsoas Bleed
d. Acute Peptic Ulcer Disease
D
UTI and iliopsoas bleed may unquestionably radiate pain to the LLQ. Appendicitis, though very rarely, may present with LLQ pain, too. (J Chin Med Assoc • December 2005 • Vol 68 • No 12) On the other hand, Acute Peptic Ulcer Disease presents with pain on the upper abdomen or the back. (http://www.bupa.co.uk/)
113
113. Your primary working impression is:
a. Von Willebrand Disease
b. Platelet Function Defect
c. Acute Immune Thrombobytopenic Purpura
d. Hemophilia A
D
Hemophilia and VWD are the most common congenital bleeding disorders. Hemophilia A affects only males whereas VWD statistically affect more females (though theoretically it affects males & females equally). (CBD Tras p2-3)
114
114. Management would include the following except:
a. FWB transfusion
b. Cryoprecipitate
c. Platelet concentrate transfusion
d. Pain medication
D. Hemophiliacs need to normal lvels of Factor VIII. What can the pain medication do? ☺
115
115. Which of the following is true about hemophilia?
a. Clinical features of Hemophilia A and B are indistinguishable
b. Mild hemophilia would present with spontaneous bleeding
c. Gum bleeds and epistaxis occur in hemophilia
d. There is no such thing as a female hemophilia
A. Mild/Moderate hemophiliacs present with trauma-related bruising or bleeding and excessive bleeding after sugery/dental extraction for boys; while mild bruising/bleeding and heavy menses for girls. Females are carriers of Hemophilia A& B but manifest in Hemophilia C. Hemophiliacs present with soft tissue bleeding but VWD patients present commonly present with epistaxis.
116
116. Which of the following will present with a decreased Platelet, prolonged Prothrombin Time, prolonged Partial Thromboplastin Time?
a. Acute Immune Thrombocytopenic Purpura
b. Disseminated Intravascular Coagulation
c. Hemophilia
d. Vitamin K Deficiency
B.
Hemophilia –inc. PTT, normal PT & platelet
Vit K def –inc PT, inc PTT, normal platelet
ITP –dec platelet
DIC –dec platelet, inc PT, inc PTT
(CBD trans p2; Acquired BD p5)
117
117. Which among the following is consistent with a platelet type of bleeding?
a. Local measures are effective to stop the bleeding
b. Bleeding may last for days
c. Bleeding within the muscles is often
d. Hemarthrosis is common
A. Platelet Defects –immediate onset, superficial, petechiae, ecchymoses, autosomal dominant, immediate response to therapy, local measures are effective (CBD Trans p1)
118
118. Liver disease can impair hemostasis and cause bleeding by which of the following mechanism?
a. Increase levels of intrinsic pathway inhibitor
b. Dysfibrinogenemia
c. Increase levels of anti-thrombin III
d. Marked thrombocytosis
B
119
119. Which characterizes bone marrow with AITP?
a. Presence of marked reticulin formation
b. Megakaryoctes increased
c. Cellularity of bone marrow decreases
d. Presence of atypical non-hematologic cell
A
120
120. The following are complications of hyperleukocytosis except:
a. Pulmonary edema
b. Stroke
c. Metabolic acidosis
d. Hypercalcemia
D
121
121. The peak age for ALL is:
a. 2 to 5 years of age
b. 0 to 1 year
c. 10 to 14 years of age
d. 7 to 10 years of age
A
122
122. These patients have “standard risk” ALL
a. Infants <50,000/mm3
c. Positive for CNS leukemia
d. Positive for Philadelphia chromosome
No Answer
123
123. The following is an adverse prognosis factors in ALL
a. DNA index > 1.16 or hyperdiploidy
b. Age 1-9 years at diagnosis
c. Rapid early response
d. Age > 10 years at diagnosis
A.
Among the Good Prognostic Factors in ALL are DNA 10(NOT >10) is a poor prognostic factor. (PHM Trans p3)
124
124. Which of the following is true of AML?
a. All patients should have HLA matched sibling BMT once in remission for cure
b. Patients with good prognosis have favorable cytogenetics such as inv (16) and t(15;17)
c. The cure rate in the US is 80% similar to ALL
d. The total duration of chemotherapy is 2 to 3 years
e. Age is a strong prognostic factor
B
125
125. Disseminated Intravascular Coagulation is a common presentation of:
a. ALL
b. JMML
c. AML M3
d. AML M6
e. AML M5
C
126
126. Acquired Aplastic Anemia appears to be caused largely by:
a. Stem cell defect
b. Stromal defect
c. Immune-mediated
d. Decreased in hematopoietic growth factors
B
127
127. Hematopoiesis in patients with Aplastic Anemia is reflected by:
a. Increased number of CD34+ cells
b. Decreased levels of IFN gamma
c. Decreased hematopoietic colony formation
d. Decreased levels of TNF
C
128
128. The diagnosis of Aplastic Anemia should be questioned in the presence of:
a. Splenomegaly
b. Increased mast cells in the marrow
c. Normal marrow reticulin
d. Macrocytosis
A
129
129. Infection that has been implicated I the causation of Aplastic Anemia:***
a. Tuberculosis
b. Malaria
c. Ebstein-Barr virus
d. Coxsackie virus
C
130
130. What is the currently recommended immunosuppressive regimen for severe Aplastic Anemia?
a. Cyclosporine
b. Antithymocyte Globulin
c. Cyclosporine and Antithymocyte Globulin
d. Cyclophosphamide
C
131
131. The finding of this cytogenetic abnormality may herald the clonal evolution in Aplastic Anemia:
a. Trisomy 7
b. Monosomy 7
c. Monosomy 8
d. Inversion 16
B
132
132. The best way to distinguish between Aplastic Anemia and MDS is:
a. Cellularity of the bone marrow
b. Severity of pancytopenia
c. Abnormal karyotype
d. All of the above
A
133
133. The following are the most important variables in the disease outcome of MDS except
a. Number of blast in peripheral blood
b. Number of blast in bone marrow
c. Cytogenetic abnormality
d. Severity of cytopenia
A
134
134. Which drug is useful in Multiple Myeloma but not in Lymphomas?
a. Prednisone
b. Rituximab
c. Thalidomide
d. Doxorubicin
A
135
135. Which cell is clonal in Hodgkin’s disease?
a. B cell
b. T cell
c. NK cell
d. Reed Sternberg cell
D
[OS 216] Hematology Samplex
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