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hem onc Flashcards

1
Q

normal MCV

A

70-90

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2
Q

range of alpha thal

A

missing one - silent
missing two - asymptomatic with mild microcytic hypochromic anemia
missing three - hgb H disease (hgb Barts as nbn) –> tx with splenectomy
missing four –> hydrops, stillbirth (or intrauterine transfusions –> BMT)

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3
Q

small for age Mediterranean child with anemia, HSM

A

beta thal major
F only on NBS
thickened bone due to extramedullary hematopoeisis

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4
Q

complications of beta thal major

A

cholelithiasis

hemochromatosis

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5
Q

how long to tx iron def anemia

A

until 2 months after hgb normalizes (to replenish stores)

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6
Q

anemias with high RDW

A

iron deficiency and lead toxicity

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7
Q

anemia with normal/low RDW

A

thalassemias

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8
Q

ringed sideroblast

A

lead poisoning vs sideroblastic anemia

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9
Q

free erythrocyte protoporphyrin

A

elevated in lead poisoning and iron deficiency

normal in thalassemia

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10
Q

why should you correct folate and B12 deficiency together?

A

if you only correct folate, could get reversible neuro damage

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11
Q

G6PD inheritance pattern

A

X linked recessive

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12
Q

smear findings in G6PD

A

heinz bodies, helmet cells, blister cells

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13
Q

inheritance of hereditary spherocytosis

A

AD

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14
Q

dx of hereditary spherocytosis

A

osmotic fragility or

EMA flow cytometry

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15
Q

amino acid substitution in SCD

A

glu –> val

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16
Q

macrocytic anemia, renal/eye/ear anomalies, abnormal skin pigment, growth retardation, forearm anomalies

A

Fanconi anemia
AR
risk of transformation to AML/MDS

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17
Q

infant with macrocytic anemia, dysmorphic facies

A

DBA

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18
Q

how to reduce risk of febrile non hemolytic transfusion reaction

A

leukocyte reduced blood

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19
Q

mucosal ulceration, gingivitis, cellulitis, abscesses, pneumonia

A

neutropenia

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20
Q

infant with severe recurrent infections, neutropenia

A

Kostmann syndrome = severe congenital neutropenia
risk of transformation to MDS and AML
tx: gCSF, BMT

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21
Q

cytopenias, pancreatic insufficiency, skeletal anomalies

A

Schwachmann Diamond

at risk for leukemic transformation

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22
Q

mild neutropenia in healthy infant

A

isoimmune neonatal neutropenia

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23
Q

monthly recurrent oral ulcers, fever, enlarged lymph nodes

A

cyclic neutropenia

AD

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24
Q

bugs responsible for death in cyclic neutropenia patients

A

clostridium perfringens and gram negative organisms

25
Q

tx cyclic neutropenia

A

gCSF

26
Q

incidental neutropenia in child

A

chronic benign neutropenia
anti-neutrophil antibodies
usually grow out of this by age 5

27
Q

meds that cause thrombocytopenia

A

sulfas, seizure meds, vanc

28
Q

meds that cause platelet dysfunction

A

aspirin, ibuprofen

29
Q

anaphylactoid purpura

A

= HSP

30
Q

age and prognosis in ITP

A

worse in older children - more likely to be chronic

31
Q

other findings in TAR

A

renal agenesis

lymphocytosis

32
Q

vit K dependent factors

A

2, 7, 9, 10

33
Q

vit K deficiency and coag studies

A

prolonged PT and PTT

34
Q

coag studies in hemophilia

A

prolonged PTT

35
Q

dx hemophilia

A

measure factor levels

36
Q

what to do for asymptomatic hemophilia pt with head trauma

A

give factors

37
Q

coag studies in vWD

A

normal PT, normal or slightly prolonged PTT

vWF activity low

38
Q

tx vWD

A

vasopressin for minor bleeding
factor VIII for major bleeding/surgery
amicar

39
Q

bone tumor that metastasizes to lungs

A

osteosarcoma

sunbursting

40
Q

bone tumor with onionskinning

A

Ewing

41
Q

leg pain worse at night, relieved by NSAIDs, XR with radiolucent area surrounded by thick bone

A

osteoid osteoma

benign

42
Q

sites with highest risk of relapse in ALL

A

CNS and testes - sanctuary sites

43
Q

unexplained lymphadenopathy, cough, unexplained pruritus

A

lymphoma

44
Q

most common solid tumor of infancy

A

neuroblastoma

45
Q

persistent bone/joint pain, B symptoms, UTI, raccoon eyes, horner syndrome, opsoclonus-myoclonus, excess catechols

A

neuroblastoma

46
Q

dx of neuroblastoma

A

biopsy

urine VMA and HMA (NOT urinary catecholamines)

47
Q

age and neuroblastoma prognosis

A

< 1 yr: good

> 1 yr: bad

48
Q

inheritance of RB

A

bilateral - AD with incomplete penetrance
unilateral - sporadic
RB1 gene on chr13

49
Q

additional risk with RB

A

pineal gland tumors

50
Q

most common soft tissue sarcoma

A

rhabdomyosarcoma

51
Q

labs in tumor lysis

A

elevated uric acid, phosphate, potassium, LDH, renal insufficiency

52
Q

why not to give steroids if can’t rule out malignancy

A

risk of TLS

53
Q

dz that can cause anterior mediastinal masses

A

thymoma
teratoma
thyroid carcinoma
lymphoma (most likely to cause airway compromise)

54
Q

cyclophosphamide SEs

A

hemorrhagic cystitis

55
Q

cisplatin SEs

A

hearing loss and peripheral neuropathy

56
Q

bleomycin SEs

A

pulmonary fibrosis

57
Q

doxo/daunorubicin SEs

A

cardiac toxicity

58
Q

vincristine/vinblastine SEs

A

neurotoxicity and SIADH

59
Q

methotrexate SEs

A

oral ulcers, GI ulcers, bone loss

boards prep (34 decks)

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