1
Q
Iron deficient anemia s/s
A
Low MCH, high RDW, low RBC
low HgB, low relic
Low ferritin & serum Fe
TIBC & transferrin elevated
Thrombocytosis
tachycardia, pallor
fatigue
irritability, restlessnessd
PICA, pagophagia
developmental delays
behavioral disturbances
2
Q
Iron deficient anemia tx
A
Non acute: iron-ferrous sulfate
Daily max 200 mg
Reassess relic in one week
Hgb should return 4-6 weeks
Acute distress: give PRBC for Hgb <7 and symptomatic
3
Q
Blood transfusion: RBC’s
A
Anemia Hgb <7
sickle cell crisis
10-15 ml/kg
FFP to replace coats
Cryo to replace specific factors
4
Q
Blood transfusion reaction S/S
A
fever, chills, pruritus, urticaria, resp distress, HTN, AMS, flank or back pain, hypotension, hemoglobinuria, jaundice, bleeding, oliguria/anuria
anaphylactic reaction
chest pain, shock
5
Q
Blood transfusion reaction tx
A
Stop transfusion
Give NS
Give Benadryl or epinephrine
Contact transfusion services
6
Q
B12 deficiency anemia s/s
A
weakness, pallor
beefy red, smooth sore tongue
inadequate weight gain
diarrhea
elevated MCV, decreased relic
normal WBC,
hypersegmented neutrophils
thrombocytopenia
7
Q
B12 deficiency anemia Tx
A
Folic Acid 1 mg QD
Vitamin B12 IM or SQ
B12 100 mcg/day
8
Q
Normocytic Anemia s/s
A
normal MCB
from hemolysis, blood loss meds,
CKD, hypothyroidism
9
Q
Alpha Thalassemia s/s
A
hemolytic anemia
hepatosplenomegaly
mild jaundice
10
Q
Alpha Thalassemia TX
A
transfusion, folate replacement.
11
Q
Beta Thalassemia Minor s/s
A
asymptomatic
Mild pallor & splenomegaly
Hgb 9.5-11
Hit < 30%
MCV/RBC < 13
12
Q
Beta Thalassemia Major s/s
A
FTT
pallor
irritability
diarrhea
abdominal enlargement
hepatosplenomegaly
jaundice
Bone deformities in face
13
Q
Beta Thalassemia Major tx
A
RBC Q 2-4 wks
keep Hgb 9.5-11
Iron Chelation
hydroxyurea
14
Q
MCV : mean corpuscular volume
A
mean volume of RBC’s
value is 70 + age in years
determines type of anemia
15
Q
RDW: red cell distribution
A
variability of RBC sizes
Reference: 12-14%
16
Q
MCHC: mean corpuscular hemoglobin concentration
A
MCHC=HGB/HCT
grams of HgB per 100 ml/RBC
hypo or hyperchromia on smear
17
Q
WBC: elevated
A
Leukocytosis: infection or acute leukemia
18
Q
Leukopenia, neutropenia, thrombocytopenia or pancytopenia
A
infection, increased peripheral destruction of blood cells, bone marrow dysfunction, myelosuppresion, meds, leukemia, aplastic anemia
19
Q
Leukocytosis
A
with anemia could be infection or leukemia
20
Q
Reticulocyte Count
A
young red blood cells in circulation
% of RBC.s
Normal: 1.5
Absolute: ARC=%retic x RBC
Increased with anemia
21
Q
Platelet tranfusion
A
Platelet < 10,000
Prevent or stop bleeding
22
Q
FFP
A
Replace coag factors
10-15ml/kg
replaces 20% of factors
23
Q
Cryoprecipitate
A
has fibrinogen, factor VIII, Factor vWF, Factor XIII, fibroniectin
given when fibrinogen < 100ml/dl and active bleeding
1 unit per 5-10 kg
24
Q
Hemolytic transfusion reaction
A
from ABO incompatibility
sickle cell may see delayed reaction
25
Febril non hemolytic transfusion reaction
temp > 1 degree
antigen or antibody rx to leukocytes or plasma proteins
26
TRALI: Transfusion related acute lung injury.
acute onset hypoxemia
bilateral infiltrates within 6 hrs
27
TACO: transfusion associated circulatory overload
leading cause of transfusion death
common with cardiac dx or infants
give diuretics, O2 and positive vent
28
Which of the following management strategies is indicated in patients with complement deficiencies?
A. Avoid annual influenza vaccine
B. Bone marrow transplant
C. Administration of all vaccine per CDC guidelines
D. Monthly gamma globulin infusions.
C. Administration of all vaccine per CDC guidelines
29
In a pediatric patient with an MCV of 89, a Retic count of less than 3 percent and a normal WBC and Platelet count, the astute ACPNP would suspect all of the following except:
A. Acute blood loss
B. G6PD Deficiency
C. Infection
D. B12 deficiency
D. B12 deficiency
30
Lab findings that support untreated Iron Deficiency Anemia in a healthy toddler would be:
A. Low serum ferritin, low serum iron and High MCHC
B. Low retic count, elevated RDW, low serum iron, elevated TIBC
C. Low serum iron, low TIBC, low MCHC and elevated Hct
D. High TIBC, low MCHC, low serum iron and elevated ferritin
B. Low retic count, elevated RDW, low serum iron, elevated TIBC
31
The ACPNP is managing a patient with suspected Aplastic Anemia. Which of the following would be most suggestive of this diagnosis?
A. Elevated platelets and retic count
B. Neutropenia and Thrombocytosis
C. Decreased platelets, decreased reticulocyte and decreased RBC.
D. Decreased granulocytes, decreased monocytes and increase in platelets.
C. Decreased platelets, decreased reticulocyte and decreased RBC.
32
Which is true with G6PD?
A: X linked disease that affects primarily males
B. Acute exacerbations common in ingestion of fava beans
C. Typical blood smear will show an increased retic count
D. Common findings are severe hyperbilirubinemia that is difficult to manage
E. All of the above
E. All of the above
33
Which of the following is a diagnosis caused by impaired production of marrow failure?
A. Folate Deficiency
B. von Willebrand disease
C. Fanconi Anemia
D. Hemophilia A
C. Fanconi Anemia
34
Which of the following is true regarding immune thrombocytopenia?
A. Usually caused by bacteria infection
B. Platelets are destroyed by their shape and fragility
C. PT and PTT are always elevated
D. Treatment includes oral steroids or IVIG
D. Treatment includes oral steroids or IVIG
35
The term "Shift to the Left" on a CBC differential means an inflammatory process or the body's immunological response to an acute bacterial infection.
True or False
True
36
The interpretation of the MCV is the calculation of the percentage of RBC in a given volume of whole blood.
True or False
False
37
Pancytopenia is caused by all of the following except:
A. Increased peripheral destruction of mature cells
B. Sequestration
C. Production failure and intrinsic bone marrow disease
D. Polycythemia
D. Polycythemia
38
Jaundice is associated with anemia in which of the following conditions?
A. Increased red cell destruction (hemolysis)
B. Excessive blood loss (hemorrhage)
C. Deficient red cell production (ineffective hematopoiesis)
D. Infectious process of hepatitis
A. Increased red cell destruction (hemolysis)
39
In a child with a suspected transfusion reaction, the first plan is to?
A. Contact the transfusion service to report the reaction
B. Administer diphenhydramine and corticosteroids
C. Stop the transfusion and administer normal saline
D. Administer oxygen via face mask
C. Stop the transfusion and administer normal saline
40
Which of the following therapies is most effective in managing vasoocclusive crisis in sickle cell disease?
A. NSAIDS
B. Blood transfusion
C. Antibiotics
D. Cold packs
A. NSAIDS
41
A 3 year old patient with newly diagnosed ALL is being prepped for a LP. Which landmarks are used to find the L4-L5 intervertebral space?
A. Measure 3/4 of the way down from the shoulders to hips
B. The space between right and left bilateral posterior iliac crests
C. The space between bilateral femoral heads
D. The top of the sacrum
B. The space between right and left bilateral posterior iliac crests
42
A 12 year old male presents to the ED with complaints of unilateral swelling just below his knee and intermittent pain. A x-ray reveals a starburst mass in the proximal tibia. This is most concerning for?
A. Ewings sarcoma
B. AML with chloroma formation
C. Hodgkins lymphoma
D. Osteosarcoma
D. Osteosarcoma
43
Which orders are most appropriate for a 7 year old with an unremarkable physical exam and a diagnosis of Burkitt lymphoma prior to receiving chemotherapy today?
A. CBC and CMP every 6 hours
B. IV fluids at 2-3 L/m2/day
C. Urine specific gravity checks every 2 hours
D. Daily CXR:
B. IV fluids at 2-3 L/m2/day
44
A noninvasive test that can be ordered to confirm a suspicion of neuroblastoma is?
A. Bone marrow flow cytometry
B. Urine for HVA and VMA-catecholamines
C. Serum for p53 mutational testing
D. Ultrasound of the abdomen
B. Urine for HVA and VMA-catecholamines
Elevated urine catecholamines are a finding specific to the diagnosis of neuroblastoma and can be obtained by 24 hours and random urine samples.
45
Using the same child in the above question. What antibiotic would you initial start with?
A. Keflex po
B. Vancomycin IV
C. Ceftriaxone IV
D. diamox po
C. Ceftriaxone IV
46
A 14 month old is admitted into the ED with a fever of 102. He is ill appearing, lethargic, and pale. In his history you see that the child has a diagnosis of sickle cell disease. What is your course of action for this patient?
A. Discharge home on antipyretics with follow up with PCP
B. Start antibiotics and discharge home under the care of the parent
C. Admit to the hospital, obtain a CXR, CBC, and blood culture
D. Admit to the hospital and call for surgical consult
C. Admit to the hospital, obtain a CXR, CBC, and blood culture
47
A 14 year old with known sickle cell disease has a history of avascular necrosis of his shoulder a year ago. His current hemoglobin is 11.8. Which type of SCD does the patient most likely have?
A. HbSS
B. HbSC
C. HbSB
D. sickle cell trait
B. HbSC
48
The most common late effects found in children who have undergone hematopoietic stem cell transplant involve the endocrine system.
True or False
True
49
Skin is the most common organ affected by acute Graft vs Host Disease, with symptoms varying from a mild erythematous maculopapular rash to generalized erythroderma and epidermal necrolysis
True or False
True
50
Which of the following tests is least likely to give you information about a patient's humoral immune system?
A. T and B lymphocyte subpopulations
B. Quantitative immunoglobulins (IgG, IgA, IgM)
C. Delayed hypersensitivity skin testing
D. Ability to produce antibodies following immunization with Streptococcus
pneumonia
C. Delayed hypersensitivity skin testing
51
Aplastic Anemia
bone marrow failure with decreased production of hematopoietic stem cells resulting in pancytopenia & bone marrow aplasia.
52
Aplastic Anemia S/S
mucosal/gingival bleeding, HA, fatigue, easy bruising, rash, fever, mucosal ulcerations, recurrent viral infections, pallor, tachycardia, petechial rash, purpura, ecchymosis, jaundice
53
Aplastic Anemia Evaluation
CBC with low HgB, WBC & plt's. low/absent retics.
Peripheral smear: no abnormal cells
Bone marrow biopsy: no decreased or absent hematopoietic components.
54
Aplastic Anemia Management
RBC's & platelets,
Abx
BMT
Immunosuppressive Tx
55
Hemolytic Uremic Syndrome
thrombotic microangiopathy from an endothelial injury
* Classic triad: thrombocytopenia, hemolytic anemia, organ damage (often
kidneys and brain)
* Forms:
* Infection-induced
* *E.coli 0157 is the most common etiology, shiga toxin
* Co-existing condition
* Cobalamin C defect
* Atypical
* Most common in children <4 yrs
56
Hemolytic Uremic Syndrome S/S:
abd pain, watery-nonbloody diarrhea, fever, weakness, lethargy, irritability.
5-7 Days: hemorrhagic colitis: pallor, petechiae, ecchymoses, hematuria, oliguria, azotemia, htn, then anuria, hepatomegaly, splenomegaly, hematemesis, edema, tremor, sz's.
57
Hemolytic Uremic Syndrome Evaluation
CBC: reticulocytosis, abnormal RBC. anemia. thrombocytopenia, leukocytosis.
Peripheral smear: schistocytes, burr, helmet cells, fragmented erythrocytes.
Coag levels: normal
Stool culture: + for E.coli
Chem 10: elevated BUN, creatinine, bilirubin & K.
UA: hematuria, proteinuria, & casts.
58
Hemolytic Uremic Syndrome Management
No abx-make bacteria release more toxins.
Supportive care. Correct electrolytes. Adequate nutrition with renal protective diet.
PRBC transfusion.
dialysis for severe cases.
Monitor BP and correct with Ca channel blockers
BP, creatinine & UA yearly.
59
Hemophagocytic Lymphohistiocytosis (HLH)
Immune over activation causing systemic inflammation & organ damage. T-cells & macrophages not regulated.
Caused by gene mutations, immune trigger, cancer, immune deficiency, CMV, or Epstein Barr.
60
HLH S/S
Prolonged high fever, hepatosplenomegaly, hepatitis, cytopenias (at least 2).acutely ill
61
HLH Evaluation
need 5 criteria meet:
Fever > 38.5, splenomegaly, cytopenia, hypertriglyceridemia, hemophagocytosis, low NK activity, Ferritin > 500, elevated CD 25. HLA typing
62
HLH Management
Intensive care management
stem cell transplantation
dexamethasone, etoposide +/- cyclosporine,
63
Hemophilia
X linked coagulation disorder, mostly in males, that causes deficiency in clotting factors
64
Immune thrombocytopenia Purpura (ITP)
precipitated by viral illness
IgG, IgA or IgM antibodies coat platelets. platelets destroyed in spleen. immature platelets production by bone marrow. new platelets destroyed.
65
ITP S/S
bruising, petechiae, epistaxis, GI bleeding hematuria, intracranial & splenic bleeding.
66
ITP Evaluation
Platelets < 100,000. large platelets on smear.
Normal Coags.
Bone marrow Bx to confirm.
67
ITP management
oral corticosteroids for months. IVIG. monitor platelets. avoid NSAIDS.
splenectomy
*greatest concern is bleeding in brain.
68
Henoch-Schonlein Purpura
Immune mediated small-vessel vasculitis
* Often presents following a respiratory tract infection
* Presentation: Rash, pain and swelling, Hematuria, proteinuria, hypertension
* Kidney is the main origin of chronic disease, can progress to renal failure
69
Hemophilia A
Deficiency in Factor VIII.
Classic hemophilia
Most common & most severe
70
Hemophilia B
Christmas Disease
Deficiency in Factor IX
71
Hemophilia S/S
Slow persistent bleeding after minor injury, hematemesis,
epistasis, hematuria, joint pain, swelling, dec ROM, hematthroses, ecchymosis, SQ hematoma.
Neonates: cephalohematoma, subdural hematoma & IVH
72
Hemophilia Evaluation
Coags: prolonged PTT,decreased factors & normal PT.
decreased HCT
73
Hemophilia Management
PRBC, FFP, Cryo
Replace factors: 100% for major bleeding. 50-60% for minor bleeding.
Give DDAVP for mild-mod Hem A.
No contact sports. No NSAIDS
74
Henoch-Schonlein Purpura
S/S
URI sx's, fever, fatigue
Rash: nonpruritic, erythematous papule/wheals.
Rash changes to petechiae, nonblanching, palpable purpuric lesions
polyarthralgias, pain, swelling, dec ROM
diffuse colicky abd pain, vomiting. hematuria, proteinuria, HTN.
75
Henoch-Schonlein Purpura Evaluation
CBC-see elevated platelets
Chem 10 & RFT's-see elevated BUN & creatinine.
Coags: usually normal
Immune antibody panel-see IgA present.
UA for blood & protein
76
Henoch-Schonlein Purpura Management
Rest & activity limitations, NSAIDS, oral prednisone.
high dose IVIG for severe cases with renal impairment.
HTN management
77
Glucose-6-phosphate dehydrogenase deficiency
deficiency in enzyme required to reduce nicotinamide adenine dinucleotide phosphate (NADPH), which is critical in preventing oxidative damage. deficiency causes O2 radicals to damage RBC causing hemolysis.
exacerbations occur after eating fava beans; an infection, exposure to ASA, quinolones, antimalarials & sulfa meds.
78
Glucose-6-phosphate dehydrogenase deficiency S/S
fever, N/V, abd pain, diarrhea, dark or black urine, jaundice, pallor, tachycardia, hypovolemic shock, hepatosplenomegaly
79
Glucose-6-phosphate dehydrogenase deficiency Evaluation
CBC: severe anemia, highly variable RDW, polychromatic cells, spherocytic
morphology, poikilocytes, increased reticulocyte count, Heinz bodies, decreased
serum haptoglobin, hemoglobinuria, increased WBC
80
Glucose-6-phosphate dehydrogenase deficiency Management
transfusion for hemodynamic instability or HgB < 7 or <9 if persistent hemolysis.
Phototherapy for neonates with jaundice.
81
Sickle Cell Disease (SCD)
abnormal sickle hemoglobin gene inherited that causes sickling of RBC.
82
SCD S/S
anemia, vasoocclusive crisis, infections, acute chest, CVA, avascular crisis, splenic sequestration, cholelithiasis, delayed growth
83
SCD Management
only cure is BMT
PCN from 2 months to 5 yrs old.
Vaccines,
Blood transfusion.
Pain control.
84
Leukemia
ALL: acute lymphoblastic
AML: acute myeloid
CML: chronic myelogenous
distruption to hematopoietic develop. Cells (blasts) do not differentiate or mature properly.
85
ALL S/S
fevers, fatigue, anorexia, wt loss, nonspecific bone pain, lymphadenopathy, inc WBC, leukostasis
86
AML S/S
cytopenias, ecchymoses,petchiae, epistaxis, chloromas
87
CML S/S
fever, fatigue, wt loss, LUQ pain. Blasts. abnormal CBC
88
Sickle Cell Disease: Acute Infection
Concerning findings and symptoms:
* Anemia, splenomegaly, abdominal distention, shock, respiratory distress, hypoxia
* Management: blood cultures, antibiotics, supportive care
* Most common cause of death: Sepsis or meningitis
* Pneumococcus is most likely organism, requiring penicillin prophylaxis
89
Sickle Cell Disease: Acute Chest
Respiratory illness with new infiltrates
* Ischemia/infarction of lung segments due to sickling
* May have associated pleural effusions
* Lower lobes most likely affected
* Pulmonary hypertension
* Treatment
* Broad spectrum antibiotics if infiltrates present
* Exchange or straight PRBC transfusion
* Respiratory support
90
Sickle Cell Disease: Stroke (CVA)
Result of occlusions to cerebrovasculature
* Occurs in approximately 7-10% of all patients
* Annual screening between 2-16 years of age
* Associated with significant morbidity/mortality
* FAST MRI for evaluation.
* Therapy: exchange transfusions to keep hemoglobin S less than 30%
* No anticoagulation required
* Indication for hematopoietic stem cell transplant
91
Sickle Cell Disease: Splenic Sequestration
Acute pooling of blood in splenic sinuses
* Life-threatening complication can lead to hypovolemic shock and death
* First episode often occurs between age 3 months and 5 years
* Acute weakness, pallor, abdominal distention, pain, splenomegaly
* Restore circulating volume and oxygen-carrying capacity
* PRBC transfusion (not to baseline), monitor for recurrence
92
Disseminated Intravascular Coagulation (DIC)
Description & Evaluation
Disturbance in normal coagulation cascade, intravascular coagulation,
consumption of coagulation factors and platelets, which trigger
thrombosis and hemorrhage
* Results from underlying condition
* Infection, trauma, ARDS, ECMO, hematologic malignancies
* High mortality
* Laboratory Findings:
* Thrombocytopenia, prolonged PT, PTT, *prolonged D-dimer
93
Disseminated Intravascular Coagulation (DIC) Management
Management is to treat underlying cause, supportive care
* Manage shock
* Address coagulation:
* Vitamin K
* Cryoprecipitate
* Fresh Frozen Plasma
* Platelets
94
VonWillebrand Disease
Deficiency or defect of the vonWillebrand protein
* Often diagnosed later in life
* Present with easy bruising, frequent epistaxis, heavy menstrual bleeding,
surgical/dental procedure bleeding.
Have abnormal bleeding times with normal clotting factors
* Treatment and prevention with DDAVP, Amicar
95
Stroke (CVA): Ischemic
occur with cardiac disease, hematologic disorders (SCD), primary vasculitis, lipid abnormalities, metabolic abnormalities,
dehydration/shock.
96
Stroke (CVA): Hemorrhagic
Hemorrhagic: occur with vascular malformation,
cavernous malformation, aneurysm, brain tumor,
thrombocytopenia or other hematologic problems,
coagulopathies, spontaneous dissection
97
Stroke (CVA) S/S, Eval & Tx
Presentation:
* Acute neurologic symptoms: headache, confusion, lethargy and possible seizures
* Dx: history, neurologic exam, head CT?, MRI
* Labs: CBC, coags, LFT’s, electrolytes, ESR
* Physical Exam: neurological evaluation, skin assessment, abdominal
exam, cardiac evaluation
* Management:
* monitoring, treatment is focused on the underlying cause, anticoagulation therapy? (controversial)
98
The BEST evidence-based management of a child with sickle
cell disease who is diagnosed with ischemic stroke is:
A. Administration of a thrombolytic drug such as retaplase
B. Anticoagulant therapy with aspirin
C. Exchange transfusion
D. Heparin bolus and continuous heparin infusion
C. Exchange transfusion
Decrease circulating HgS and improve oxygenation.
99
The most appropriate test to confirm the diagnosis of sickle cell anemia in a newborn is:
A. Sickledex
B. Hemoglobin electrophoresis
C. Newborn screen
D. Mean corpuscular volume (MCV)
B. Hemoglobin electrophoresis
This is best confirmatory test for sickle cell anemia. The sickledex is a screening tool.
100
You suspect a patient with sepsis is developing DIC. The
diagnostic test of choice to confirm this diagnosis is:
A. D-dimer
B. Prothrombin time
C. Erythrocyte sedimentation rate
D. Fibrinogen
A. D-dimer
101
A 16-year old female presents with obesity and acute shortness of breath. She has no significant past medical history and her only medication is oral contraceptives. What is the most sensitive test for the suspected diagnosis?
A. CT angiography
B. Venous ultrasound
C. Chest x-ray
D. MRI of the chest
A. CT angiography
Case describes a pt with sx's of a PE. The best imaging for this differential is CTA
102
A cardiac patient underwent surgical intervention requiring cardiopulmonary bypass last night and is currently bleeding. You
suspect that there is factor deficiency and appropriately order the following blood component to correct the underlying deficiency:
A. Packed red blood cells
B. Platelets
C. Fresh frozen plasma
D. Cryoprecipitate
C. Fresh frozen plasma
FFP is derived from whole blood & will replace about 20% of the pt's factors.
103
A school-age child presents to the emergency department with bruising
on his legs and abdomen. His caregiver states that he had a cold approximately two weeks ago, as did his two older sisters. The CBC
reveals a hemoglobin of 12.2gm/dL, WBC 13,500, and platelet count of 48,000. You know the most likely diagnosis in this child is:
A. Severe aplastic anemia
B. Viral illness
C. Leukemia
D. Idiopathic thrombocytopenic purpura
D. Idiopathic thrombocytopenic purpura
This pt has thrombocytopenia with a normal WBC & Hgb. This is less likely to be severe aplastic anemia because only cell line is impacted. Additionally, there is a relevant history of viral illness, which often precedes the diagnosis of ITP.
104
The priority intervention for a patient with hemophilia B who has experienced an acute musculoskeletal bleed is:
A. Factor replacement therapy
B. Platelet transfusion
C. Cryoprecipitate transfusion
D. Transfusion of fresh frozen plasma
A. Factor replacement therapy
105
Acute Leukemia Treatment
Chemotherapy
* Immunotherapy
* Radiation
* Hematopoietic stem cell transplantation
* *Supportive care
106
Non-Hodgkin Lymphoma
Non-Hodgkin Lymphoma (NHL): Malignant proliferation of lymphocytes (T/B/indeterminant cells) that Multiply rapidly, unpredictable, aggressive.
107
Non-Hodgkin Lymphoma S/S
Dependent on disease site involved: Abdomen: mass, pain, nausea, vomiting, change in bowel habits,
hematochezia
Mediastinum: Dysphagia, SVC syndrome, chest pain
Head/Neck: facial swelling, snoring, rhinorrhea, cervical
lymphadenopathy
108
Non-Hodgkin Lymphoma Evaluation & Management
Diagnosis: Imaging: CXR, CT scan, PET
Biopsy & pathology
Staged according to St. Jude Staging System:
Often present emergently, require stabilization
Associated with ATLS and SVC syndrome
Treatment: Based on type and extent of involvement
Multiagent, combination chemotherapy + IT chemotherapy if CNS involved
Limited role of radiation
109
Hodgkin Lymphoma
Usually spreads to adjacent lymph nodes
Slower, more orderly spread
110
Hodgkin Lymphoma S/S
Lymphadenopathy, Fatigue, anorexia, pruritis, SVC Syndrome, Cough, shortness of breath, orthopnea, Fevers, wt loss, drenching night sweats.
111
Hodgkin Lymphoma Eval & Tx
Diagnosis:
* Imaging: CXR, CT scan, PET,
Reed Sternberg cells: large, abnormal, multinucleate WBC (have distinct owl's eye with 2 nuclei).
* Treatment: Chemo & re-staging
* Involved field radiation when indicated, usually following chemo completion
Autologous/allogeneic HSCT
* Immunotherapy
112
Wilm’s Tumor
Unilateral or bilateral solid tumor of the kidney, median age 2 to 3 years
* Abdominal distention, may have pain, microscopic or gross hematuria, malaise, fever, hypertension
*Tx: Nephrectomy or partial resection, chemotherapy or radiation
113
Retinoblastoma
Retinal tumor, unilateral or bilateral, noted with leukocoria & strabismus, often genetic
Individualized therapy with surgery, chemotherapy, cryotherapy and radiation
114
Neuroblastoma
Neoplasm of the sympathetic nervous system. Most common extracranial solid tumor in childhood cancer
* Primary tumors in the chest, abdomen, pelvis
* Often present with metastasis
* CT, MRI, MIBG
* Multi-modal tx, requires HSCT and immunotherapy + retinoids
115
Bone Tumors
Various types
* Ewings, osteosarcoma
* Pain over affected area, fever, weight loss, increased ESR
* Imaging – first plain film, then CT/MRI/PET
* Treatment usually involves resection,chemotherapy.
May have limb salvage or amputation
116
CNS Tumor
Most common solid tumor, 2nd to leukemia in childhood cancers.Many different types and placements
* Headache with morning vomiting, increased ICP,
progressive neurologic changes, lethargy, irritability
* Obtain CT or MRI
* Tx: Depends on location and severity. Usually surgical resection, radiation and
chemotherapy
117
Mediastinal Mass
Hemodynamic instability, superior vena cava syndrome, hypoxemia, hypoventilation, dysphagia.
Cough, dyspnea, hoarseness, stridor, orthopnea, syncope,
tachycardia, jugular vein distension, swollen face, cyanosis
Tx: Prone position, caution with anesthesia, positive pressure ventilation
if necessary, chest x-ray, CT.
* Emergent radiation or chemotherapy may be required
118
Tumor Lysis Syndrome
Metabolic derangements caused by rapid release of
intracellular components of lysed malignant cells
Risks: high turnover rates, large tumor burden, sensitivity to therapy, dehydration, renal dysfunction
* Hyperuricemia, hyperphosphatemia, hyperkalemia, hypocalcemia,
elevated LDH, Elevated creatinine, arrhythmia, sz,
Aggressive hydration, diuretics, correct electrolytes, rasburicase or allopurinol for hyperuricemia, dialysis may be needed.
119
Hyperleukocytosis
WBC > 100,000/mm3 with Increased blood viscosity/leukostasis leads to sludging in the microcirculation of the lungs and CNS
120
Hyperleukocytosis S/S
CNS: headache, tinnitus, ataxia, behavioral changes, seizure, hemorrhage, infarct
Lungs: tachypnea, respiratory distress, hypoxia, hemorrhage, ARDS,
respiratory failure
Other organs: renal artery/vein distention, renal failure, papilledema, dactylitis, priapism, cardiac failure
121
Hyperleukocytosis Management
Prompt chemotherapy, dexamethasone for pulmonary leukostasis,
avoid RBC transfusion until WBC reduced
* Leukapheresis and exchange transfusion controversial
122
Typhlitis / Intra-abdominal Emergencies
Inflammation of the cecum
* Right lower quadrant abdominal pain, +/- fever, mucositis.
* Broad spectrum antibiotics (include gram negative coverage), IV fluids,
pain management
123
Mass Syndromes
Abdominal, spinal compression via tumor mass
* Altered neurologic condition/status, pain, compartment syndrome
* Decrease mass effect via radiation, surgical debulking or chemotherapy
124
Febrile Neutropenia
Criteria: Single temperature > 38.3°C or > 38.0° for > 1 hour with an ANC< 500 cells/μL or an ANC expected to decrease to < 500 cells/μL within
the next 48 hours
125
Febrile Neutropenia causes
Bacteremia is the most common form of infection
* Other common sites include the GI tract (oral or intestinal mucositis, diarrhea), upper and lower respiratory tract infections, urinary tract
infection
* Diarrhea most commonly caused by Clostridium Difficile and Salmonella
* Consider both gram positive and gram-negative organisms
126
Febrile Neutropenia Evaluation
Blood cultures (all central venous access devices/lumens, consider peripheral)
* Urinalysis/urine culture if clean-catch, mid-stream specimen available
* Chest x-ray in symptomatic patients
* CT/Ultrasound for fluid collections, effusions
* Respiratory viral screening in symptomatic patients
* Stool for infectious studies in symptomatic patients
* Lumbar puncture for altered mental status
127
Febrile Neutropenia Management
Low-risk: consider oral, outpatient therapy
* Fluoroquinolone monotherapy or fluoroquinolone and amoxicillin-clavulanate
* High risk: cover gram negative organisms, strep viridans, and pseudomonas
aeruginosa
* Hospital admission and antibiotic monotherapy (antipseudomonal beta lactam, fourth-generation cephalosporin, or carbapenem) if stable
* Add gram-negative agent or glycopeptide if unstable, resistant pathogen
suspected
* Also consider adding gram-positive and anaerobic coverage for resistant
organisms
* If minimal response or signs of decline, therapy should be adjusted for clinical,
radiographic, and/or culture data
128
A 2-year-old who is receiving treatment for a neuroblastoma develops
fever to 38.9C, lethargy and has oxygen saturations to 95%. The most important, first-line management should include:
A. Obtain peripheral blood culture and CBC
B. Administration of antibiotics to include 3rd generation cephalosporin along with an antifungal
C. Obtain blood cultures from CVL and administer antibiotics to include 4th generation cephalosporin
D. Obtain blood culture and lactate level from central line
C. Obtain blood cultures from CVL and administer antibiotics to include 4th generation cephalosporin
Most important to give 4th generation cephalosporin in the setting of fever in immunocompromised to cover pseudomonas.
129
A two-year old presents with opsocolonus-myoclonus. You
know that you should include which malignancy in your
differential?
A. Wilm’s Tumor
B. Ewing’s Sarcoma
C. Neuroblastoma
D. Non-Hodgkin Lymphoma
C. Neuroblastoma
130
A child referred to the emergency room by the PCP for concerns of acute myelogenous leukemia develops dyspnea, hypoxia, and tachypnea. You are called by the nurse and find a school-age child in
impending respiratory failure. You suspect this child has developed:
A. Pulmonary complications of hyperleukocytosis
B. Typhlitis
C. Intracranial hemorrhage
D. Tumor lysis syndrome
A. Pulmonary complications of hyperleukocytosis
131
A teen with a history of relapsed osteosarcoma presents with difficulty
voiding, lacking feeling with bladder fullness. What is the most likely cause?
A. Metastatic obstruction of the bladder
B. Neurogenic bladder secondary to chemotherapy
C. Urinary retention as a side effect of oxycodone
D. Metastatic compression of the spinal cord
D. Metastatic compression of the spinal cord
132
Electrolyte disorders noted with a child with non-Hodgkin lymphoma
and tumor lysis syndrome include:
A. Hypernatremia, hyperkalemia, and hypoglycemia
B. Hypernatremia, hypokalemia, and hyperphosphatemia
C. Hyperphosphatemia, hyperkalemia, hyperuricemia
D. Hypophosphatemia, hyperuricemia, and hyperglycemia
C. Hyperphosphatemia, hyperkalemia, hyperuricemia
133
A 2-week-old infant is noted by his parents to have an asymmetrical red
reflex, with one eye producing a normal red reflex while the other eye
demonstrates no reflection at all. A probable cause of this asymmetry
is:
A. Congenital caratact
B. Retinoblastoma
C. Neuroblastoma
D. This is a normal finding depending on the position of the eyes
B. Retinoblastoma
Asymmetry is called leukocoria.
134
A 4-year-old girl has had blurry vision, ataxia and has become increasingly confused. She has vomited the past three mornings on
arising. What study could be done quickly and is effective in diagnosing her illness?
A. CT of the brain
B. MRI of the brain and spinal cord
C. Head ultrasound
D. PET scan of the brain and spinal cord
A. CT of the brain
135
Hematopoietic Stem Cell
Transplantation (HSCT)
Autologous
* Indications: solid tumor malignancies
* Sources: peripheral blood cells, bone marrow
* Complications: collection-related complications, toxicities of preparative regimen,
pancytopenia, mucositis, infection, infusion-related complications (product is
cryopreserved), veno-occlusive disease, end organ dysfunction/damage as late-
effects of chemotherapy
136
Hematopoietic Stem Cell
Transplantation (HSCT)
Allogeneic
* Indications: Malignant, Non-malignant (bone marrow failures,
hemoglobinopathies, immunodeficiencies, inborn errors of metabolism)
* Sources: Related vs unrelated donor, peripheral blood stem cells, bone marrow,
umbilical cord blood
* Complications: toxicities of preparative regimen, pancytopenia, mucositis,
infection, infusion-related complications, veno-occlusive disease, end organ
dysfunction/damage as late-effects of chemotherapy
137
HSCT Complications
Regimen Related Toxicities
* Sinusoidal Obstructive Syndrome / Veno-occlusive Disease
* Mucositis
* Pancytopenia
* Recurrence of Underlying Disease
* Graft Versus Host Disease
* Acute
* Chronic
* Infection
138
Graft Versus Host Disease
Usually presents later in the disease course, however, diagnosed based
on clinical presentation
* Autoimmune features
Skin: Hypo hyperpigmentation,
anhidrosis, plaques,
sclerodermatous-like texture
Liver: Cholestasis, Musculoskeletal: Contractures
GI: Nausea, anorexia,
malabsorption
Ocular: Xerophthalmia
Oral: Leukoplakia, xerostomia
139
Diamond-Blackfan Anemia
Hypoplastic anemia from a mutation caused on chromosome 19.
140
Diamond-Blackfan Anemia S/S
pallor, fatigue, irritability, syncope, dyspnea during feeding, irregular heartbeat, hypotonia, short stature, FTT
141
Diamond-Blackfan Anemia Evaluation
macrocytic anemia: WBC & platelet normal, reticulocytopenia, increased hgB F, elevated erythrocyte adenosine deaminase activity. DB anemia mutation screening
142
Diamond-Blackfan Anemia Management
Corticosteroids, blood transfusions, BMT
143
Osteosarcoma
tumor from primitive mesenchyme and osteoblasts. peak around growth spurts
144
Osteosarcoma S/S
pain, swelling of joint or bone. metastatic dx may present with fever, night sweats, wt loss.
145
Osteosarcoma Evaluation
Xray: starburst or bone in bone formation.
CBC, CMP, LFT's, bone marrow biopsy.
146
Osteosarcoma Management
surgical resection, chemotherapy, amputations.
Radiation not helpful.
PNP-AC Boards
flashcards
Decks in class (23)
# Cards
-
Psychiatric Emergency28
-
Toxicologic Emergencies61
-
Environmental Exposures4
-
Trauma62
-
Neuro107
-
Medications38
-
Hem/Onc146
-
Pulmonary/Respiratory129
-
Infectious Disease125
-
Rheumotalogy29
-
Musculoskeletal35
-
GI/hepatic72
-
HEENT11
-
Cardiac91
-
Integumentary1
-
Diabetes/Endocrine56
-
Fluid & Electrolytes54
-
Metabolic Disorders23
-
Pain/Sedation/Palliative Care5
-
Renal/GU28
-
Practice questions296
-
Genetics14
-
Growth & development33
