USMLE Step 1 > Hematology > Flashcards

Hematology Flashcards

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1
Q

Erythropoietin

A

synthesized in peritubular capillaries

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2
Q

Reticulocyte count

A

measure of effective erythropoiesis; correct for degree of anemia

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3
Q

Extramedullary hematopoiesis

A

Hematopoiessi outside bone marrow (e.g. spleen)

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4
Q

Newborn physiologic anemia

A

drop in Hb due to replacement of HbF RBCs with HbA

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5
Q

Pregnancy

A

Hb and Hct decreased

greater increase in plasma volume than RBC mass

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6
Q

Anemia

A

normal oxygen saturation and arterial PO2

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7
Q

MCV

A

average VOLUME of RBCs; useful for anemia classification

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8
Q

MCHC

A

average Hb concentration in RBCs

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9
Q

When is MCHC decreased? When is MCHC increased?

A

MCHC

  • decreased in microcytic anemias
  • increased in spherocytosis
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10
Q

Thalassemias

A
  • decreased in MCV

- increased in RBC count

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11
Q

RDW

A

RBC size variation

  • increased in iron deficiency
  • decreased in anemia of chronic disease, sideroblastic anemia
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12
Q

Mature RBC

A

anaerobic glycolysis; no mitochondria or HLA antigens

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13
Q

Total iron binding capacity is increased

A
  • in iron deficiency
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14
Q

Total binding capacity is DECREASED

A

in anemia of chronic disease OR sideroblastic anemia

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15
Q

% Saturation

A
  • decreased in iron deficiency or anemia of chronic disease
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16
Q

% Saturation is increased

A

sideroblastic anemia

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17
Q

Serum ferritin is decreased

A
  • iron deficiency
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18
Q

Serum ferritin is INCREASED

A

anemia of chronic disease, sideroblastic anemia, normal thalassemia

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19
Q

Microcytic anemia

A
  • seen in iron deficiency (most common cause)
  • anemic chronic disease
  • thalassemia
  • sideroblastic anemia
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20
Q

Most common cause of iron defieicny in a child

A

Meckel’s diverticulum

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21
Q

Iron deficiency in women < 50

A

Menorrhagia (heavy bleeding during menses)

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22
Q

Iron deficiency man < 50

A

Most common cause is peptic ulcer disease

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23
Q

Iron deficiency in men/women > 50

A

Colon cancer

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24
Q

Stages of iron deficiency

A
  • decreased ferritin, Fe, and % saturation

- increased TIBC, normocytic then microcytic anemia

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25
Anemia of chronic disease
Most common anemia in malignancy and alcoholics
26
Alpha Thalassemia trait
Autosomal recessive two alpha globin gene deletions - normal Hb electrophoresis
27
HbH disease
- three alpha-globin gene deletions; - hemolytic anemia - four Beta-globin genes
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Hb Bart's disease
four alpha globin gene deetions | four gamma globin genes
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B- thalassemia minor
Autosomal Recessive - DNA splicing defect - increased HbA2 and F - idecreased HbA
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B-thalassemia major
nonsense mutation with stop codon - hemolytic anemia - very elevated HbF - increased HbA2
31
Sideroblastic anemia
- defect in mitochondrial heme synthesis producing ringed sideroblasts
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Causes of sideroblastic anemia
- Alcohol - Pyroxidine deficiency (may be due to isoniazid tx of TB) - Lead poisoning
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Lead poisoning
- Inhibits ferrochetolase - ALA Dehydrase (can't convert ALA to porphobillogen??) - Ribonuclease
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Sx of lead poisoning in children
- Growth retardation - Pb in epiphyses - Abdominal colic - Encephalopathy
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Sx of lead poisoning in adult
Peripheral neuropathy | Proximal renal tubule damage (Fanconi's syndrome)
36
Lab of Pb poisoning
- Course basophillic stippling RBCs - decreased MCV - increase in Pb levels - increase in ALA
37
Vitamin B12 sources
Animal products (requires intrinsic factor reabsorption in terminal ileum)
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Vitamin B12
transfers methyl group to homocysteine - involved in proprionate metabolism - end product of succinyl CoA
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R factor
binds with B12 in mouth, removed by pancreatic enymes in small intestine
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Pernicious anemia
autoimmune destruction of parietal cells sx: chronic gastritis in body/fundus, acholrydia - increase in gastrin
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Causes of folate deficiency
- Alcohol (most common cause) - Poor diet - Drugs - Malabsorption - Pregnancy - Goat milk
42
Drugs and Folate deficiency
- Alcohol - Oral contraceptives - Phenytoin - Methrotrexate - Trimethroprim - 5-fluorouracil
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Intestinal conjugase in folate metabolism
Inhibited by phenytoin
44
Jejunal uptake of monoglutamate of folate
Inhibited by alcohol and oral contraceptives
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Dihydrofolate reductase
inhibited by methotrexate, trimethoprim
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Thimydilate synthase
inhibited by 5-fluorouracil
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Most common cause of increased serum homocysteine
Folate deficiency
48
Lab in B12/folate deficiency
- Pancytopenia - Hypersegmented neutrophils - Increased in homocysteine
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Lab finds unique to B12 deficiency
- increased gastrin (pernicious anemia) | - increased methylmalonic acid
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Likely diagnosis: B12 reabsorbed after administration of intrinsic factor.
Pernicious anemia
51
Likely diagnosis: B12 reabsorbed after administration of antiobiotics
Bacterial overgrowth
52
Likely diagnosis? | B12 reabsorbed after administration of pancreatic enzyme
Chronic pancreatitis
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Hb and Hct after acute blood loss
- Initially normal Hb and Hct | .9% saline uncovers RBC deficit
54
Aplastic anemia
- Drugs (e.g. phenylbutazone) - Infection (e.g. parvovirus) - Benzene
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Lab findings for aplastic anemia
- Pancytopenia - Hypocellular bone marrow -
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Anemia in renal disease
Normocytic | - decreased EPO
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Extravascular hemolysis
- Macrophage phagocytosis of RBCs | - Increase in unconjugated bilirubin and urine UBG
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Intravascular hemolysis
- Decrease in serum haptoglobin - Hemoglobinuria - Hemosideriuria
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Congenital spherocytosis
- Autosomal dominant - defectin spectrin - Extravascular hemolysis - Splenomegaly
60
Blood findings in spherocytosis
Normocytic anemia - Dense RBCs - Increase in MCHC - INcrease in osmotic fragility
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Blood findings in spherocytosis
Normocytic anemia dense RBCs Increase in MCHC Increase in osmotic fragility
62
PNH
missing decay acceerating factor - complement destruction RBCs - neutrophils - platelets
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Sx of PNH
- Pancytopenia - Hemoglobiuria - Positive sugar water test and acidified serum test
64
HbSS
- Autosomal Recessive | - Missense mutation (valine for glutamic acid; 6th positive B-globin chain)
65
Causes of sickling
- Increase in deoxyhemoglobin (hypoxemia and acidosis) | - HbS > 60%
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HbF
inhibits sickling | hydroxyurea increases HbF
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Pathophysiology of HbSS
``` Vasoclusive crisises Hemolytic anemia ( Extravascular) ```
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HbSS in children
Dactylitis (6 - 9 months) | Streptococcus pneumoniae sepsis (dysfunctional spleen)
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HbSS osteomyelitis caused by what microorganism?
Salmonella paratyphi
70
HbSS complications
``` Aplastic crisis (parvovirus) Acute chest syndrome Autosplenectomy Calciym bilirubinate gallstones Priapism Asceptic necrosis ```
71
HbAS
- microhematuria from sickling in renal medulla | - renal papillary necrosis
72
Hb electrophoresis
HbAS - HbA (55 - 60%) HbS (40 - 45%) HbSS - HbS 90- 95% HbF - 5-10%
73
Blood findings in HbSS
Sickle cells Target cells Howell-Jolly bodies (nuclear remnants)
74
G6PD deficiency
``` X-linked recessive Oxidant damage (due to peroxide) to Hb (e.g. primaquine, dapsone, fava beans) ```
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Blood findings G6PD deficiency
``` Heinz bodies (denatured Hb; special stain) Bite cells ```
76
Pyruvate kinase deficiency
- Decreased ATP - RBCs dehydrated - increase in 2,3 BPG (right shift of oxygen-blood curve)
77
Warm Autoimmune-hemolytic anemia
``` IgG Extravascular hemolysis (e.g. SLE, drugs) ```
78
Cold auto-immune hemolytic anemia
``` IgM Intravascular hemolysis (e.g. CLL, mycoplasma) ```
79
Pencillin HS rxn
IgG antibody against penicilin attached to RBC (type II hypersenstivity)
80
Methyldopa
drug alters Rh antigens; | IgG antibody against Rh antigens (type II antibody)
81
Quinidine
drug IgM immune complexes intravascular hemolysis type III hypersensitivity
82
Lab findings for autoimmune hemolytic anemia
Positive direct Coombs | spherocytes
83
Microangiopathic hemolysis
Mechanical damage causing intravascular hemolysis
84
Causes of macro/micro hemolysis
Aortic stenosis (most common cause0 DIC TTP HUS
85
Peripheral blood findings micro/macro hemolysis
Schistocytes | Iron deficiency from hemoglobinuria
86
Malaria
Intravascular hemolysis correlates with fever | Falciparum - ring forms and gametocytes
87
Leukemoid reaction
exaggerated WBC response to infection | Usually due to infection
88
Leukoerythroblastic reaction
Marrow infiltrative disease peripheralizes myeloblasts/nucleated RBCs
89
Leukemoid reaction
exaggerated WBC response to infection; usually due to infection
90
Causes of leukoerythroblastic reaction
Bone metastais MCC | myelofibrosis
91
Eosinophilia
Type I hypersensitivity (e.g. penicillin reaction) | Invasive helminthic infection
92
Helminths not producing eosinophili
Pinworms | Adult worms in ascariasis
93
Atypical lymphocytes
``` Mononucleosis CMV Toxoplasmosis Viral hepatitis Phenytoin ```
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Mononucleosis
due to EBV | EBV attaches to CD21 receptors in B cells
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Clinical findings for mononucleosis
Exudative tonsillitis Generalized lymphadenopathy Hepatosplenomegaly
96
Lab findings for mononucleosis
Atypical lymphocytosis | IgM heterophile antibodies against horse RBCs (MONOSPOT)
97
Lymphopenia
T-cell deficiences (HIV) | combined B/T deficiency (adenine deficiency)
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Lymphocytosis
Viral infections; whooping cough
99
Chronic myeloproliferative disease
Neoplastic stem cell disorder Splenomegaly Marrow fibrosis Risk of leukemia
100
Examples of myeloproliferative disease
Polycythemia vera Myelofibrosis Myeloid metaplasia
101
Relative polycythemia
Decrease in plasma volume, increase in RBC count, normal RBC mass
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Absolute polycythemia
Increase RBC count and RBC mass
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Appropriate polycythemia
Hypoxic stimulus for EPO to generate RBCs
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Causes of appropriate absolute polycythemia
Lung disease Cyanotic heart disease High altitude
105
Appropriate absolute polycythemia
Normal plasma volume Increase in RBC mass Decrease SaO2 Increase EPO
106
Inappropriate absolute polycythemia
No hypoxic stimulus for EPO
107
Causes of inappropriate polycythemia
Ectopic secretion EPO | Polycythemia vera
108
Polycythemia vera
Increase plasma volume and RBC mass normal SaO2 Decreased EPO
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Ectopic EPO (renal cell carcinoma)
Normal plasma volume Increase in RBC mass Normal SaO2 Increase in EPO
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Myelofibrosis myeloid metaplasia
Marrow fibrosis Extramedullar hematopoiesis Splenomegaly
111
Lab findings in myelofibrosis
Tear drop RBCs | Dry bone marrow aspirate (marrow fibrosis)
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Essential thrombocythemia
MPD with increase in abnormal appearing platelets
113
Myelodysplastic syndrome
severe anemia in elderly; 30% develop leukemia | ringed sideroblasts
114
Benzene
associated w/ aplastic anemia | acute leukemia
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Leukemia by age
ALL: newborn AML: 15- 60 CML: 40 - 60 CLL: > 60
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Acute vs chronic leukemia
Acute: blasts > 30% in bone marrow Chronic: blasts < 10% in bone marrow
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Acute Myeloproliferative Leukemia
Auer rods in myeloblasts
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Acute Promyelocytic leukemia
t(15; 17) defect in retinoic acid Rx: retinoic acid (increased maturation) DIC
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Acute monocytic leukemia (AML)
Gum infiltration
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Chronic Myelogenous Leukemia
t(9; 22) of ABL proto-oncogene Philadephia chromosome 22 Decrease leukocyte alkaline phosphatase score
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ALL
early pre-B (80%) CALLA (CD10) TdT positive CNS testicle involvement
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Indicators of good prognosis of ALL
t(12; 21)`
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Chronic Lymphoblatic Leukemia (CLL)
pts > 60 years B cell neoplasm - decrease in gamma-globulins - Generalized lymphadenopathy
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Adult T-cell Leukemia
HTLV-1 CD4 T-cells skin infiltration lytic bone lesions with hypercalcemia
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Hairy cell leukemia
positive TRAP stain ("trap the hairy spider") splenomegaly Rx with purine nucleosides
126
Nodal sites
``` germinal follicles B cells paracortex T cells sinuses histiocytes ```
127
Testicular cancer metastasis
to para-aortic lymph nodes
128
Stomach cancer metastasis
to supraclavicular lymph nodes
129
Phenytoin side effect
- ginigival hyperplasia - atypical lymphocytosis - nystagmus - ataxia
130
Cat scratch disease
Bartonella henselaw | granulomatous microabscesses
131
Follicular B-cell lymphoma
t(14, 18) | overexpression of BCL-2 (anti-apoptosis gene)
132
Burkitt lymphoma
``` t(8, 14) overexpression of c-myc EBV association common childhood Non-Hodgkin's Lymphoma "starry sky appearance ```
133
Extranodal lymphomas
risk factors: H-pylori (stomach) Sjorgen's syndrome
134
Mycosis fungiodes
CD4 T neoplasm skin lesions with Pautrier's microabcesses - when spread to blood becomes Sezary syndrome
135
Sezary syndrome
leukemic phase of Mycosis fungiodes
136
Polyclonal gammopathy
sign of chronic inflammation
137
Monoclonal gammopathy
``` M component (spike) sign of plasma cell disorder ```
138
Bence Jones proteins
light chains in urine | - predicive of a malignant plasma cell disorder (e.g multiple myeloma)
139
Multiple myeloma
patients > 45 - M spike - lytic bone lesions - pathological fractures - hypercalcemia - renal failure
140
MGUS
monoclonal gammopathy, may progress to myeloma
141
Findings in MGUS
elderly patient no Bence Jones proteins no malignant plasma cells
142
Waldenstrom's macroglobulinemia
lymphoplasmacytic lymphoma IgM M spike (vs. IgG and IgA in multiple myeloma) Hyperviscosity (b/c IgM are large molecules)
143
Hodgkin's lymphoma
neoplastic compnent Reed Stenberg cell ("owl eyes") CD15 + and CD30+ - bimodal distribution: young adulthood or > 55 years - EBV association - Constitutional symptoms (e.g. weight loss, night sweats)
144
Reed Steinberg cell
- seen in Hodgkin's lymphoma - binucleate or bilobed halves as mirror images - more RS cells present the worse the prognosis
145
Non-sclerosing Hodgkins
- female dominant | - supraclavicular nodes + anterior mediastinal nodes
146
Mixed cellularity Hodgkins
male dominant numerous RS cells EBV association
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