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Hematology/Oncology Flashcards

1
Q

Physiologic anemia nadir

A

6-9 weeks

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2
Q

Diamond-Blackfan anemia BM findings

A

paucity of erythroid precursors

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3
Q

Diamond-Blackfan anemia age

A

< 1 year

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4
Q

Diamond-Blackfan anemia findings

A

triphalangeal thumb, cataracts/glaucoma, cleft lip, CHD

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5
Q

TEC triggers

A

viral - parvovirus, echovirus, HHV-6

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6
Q

TEC age

A

1-4 years

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7
Q

Fanconi anemia lab findings

A

macrocytic pancytopenia

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8
Q

Fanconi anemia PE findings

A

short stature, skin pigment changes, thumb/radius abnormalities

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9
Q

Fanconi anemia genetics

A

AR

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10
Q

Dyskeratosis congenita

A

reticulated hyperpigmentation, nail dystrophy, mucosal leukoplakia, BM failure

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11
Q

Dyskeratosis congentia age range

A

5-15 years

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12
Q

Evans syndrome

A

autoimmune hemolytic anemia and ITP

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13
Q

Normal adult hemoglobin

A

alpha2, beta2

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14
Q

Fetal hemoglobin

A

alpha2, gamma2

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15
Q

NBS findings: FAS

A

HgbS trait

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16
Q

NBS findings: FS

A

SCD or HbS trait with other variant

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17
Q

NBS findings: FSC, FSD

A

sickling disorder

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18
Q

NBS findings: FAE

A

HbE trait

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19
Q

Anemia: adolescent

A

Female < 12, Male < 13.5

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20
Q

Cow milk intake associated with iron deficiency

A

> 900 cc/day

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21
Q

G6PD genetics

A

x-linked

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22
Q

Elevated MCHC

A

hereditary spherocytosis

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23
Q

Elevated RDW

A

iron-defieicency anemia (normal in thalassemia)

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24
Q

Findings in iron-deficiency anemia

A

low Fe, low ferritin, high TIBC

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25
Q

Alpha thalassemia syndromes

A

silent carrier, thal trait, HgH disease, hydrops fetalis

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26
Q

Cooley’s anemia

A

beta thalassemia major

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27
Q

Findings in beta thalassemia

A

“hair-on-end” skull x-ray, chipmunk facies

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28
Q

Peak incidence of death in SCD

A

6 mo to 3 years

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29
Q

Second most common hemoglobin variant

A

HgbE

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30
Q

Measurement of extrinsic pathway

A

PT/INR (VII, X, V, II, fibrinogen)

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31
Q

Measurement of intrinsic pathway

A

PTT (XII, XI, IX)

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32
Q

Peak incidence of ITP

A

2-5 years

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33
Q

Alloimmune thrombocytopenia pathophysiology

A

maternal IgG against paternal Pla1 Ag (may arise with 1st pregnancy but increased with subsequent pregnancies)

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34
Q

Hemophilia genetics

A

XLR (33% = new mutations)

35
Q

Hemophilia A

A

factor IIX

36
Q

Hemophilia B

37
Q

Hemophilia labs

A

prolonged PTT

38
Q

vWD genetics

A

AD (incomplete penetrance)

39
Q

Standard risk ALL

A

1-10 yo, WBC < 50K, pre-B, hyperdiploid, minimal residual disease

40
Q

Survival in AML

41
Q

Most common presentation for Hodgkin lymphoma

A

painless LAN (B symptoms = worse prognosis)

42
Q

Infratentorial tumors

A 
Most common (70%)
Cerebellar astrocytoma, medulloblastoma, ependymoma, brainstem glioma
43
Q

MRI findings in medulloblastoma

A

Contrast enhancing midline tumor (exclusively in the cerebellum)

44
Q

Medulloblastoma treatment

A

resection, XRT (not in < 3 yo), chemo

45
Q

Most common malignancy in infancy

A

neuroblastoma

46
Q

Neuroblastoma features associated with poor prognosis

A

stage III or IV (except IVS), age > 1, high N-myc amplification

47
Q

IVS neuroblastoma

A

infant (< 1 year), resectable primary and only mets to liver, skin or BM

48
Q

Most common site of rhabdomyosarcoma

A

head/neck/orbits

49
Q

Retinoblastoma features associated with good prognosis

A

< 2 years, unilateral, no extension

50
Q

Management of bilateral retinoblastoma

A

MRI to rule out PNET of pineal gland

51
Q

Hepatoblastoma associations

A

Beckwith-Wiedeman, FAP

52
Q

Tumors associated with von Hippel Lindau

A

CNS - retinal hemangioblastoma

Visceral - renal cell carcinoma, pheochromocytoma, pancreatic tumor

53
Q

Pearson syndrome findings

A

pallor, pancreatic dysfunction, lactic acidemia, FTT

54
Q

RBC indice that differentiates TEC and DBA

A

MVC - normal in TEC, increased in DBA

55
Q

SCD: common age for dactylitis

56
Q

SCD: rate of stroke

57
Q

SCD: screening for stroke

A

TCD every 6-12 months, MRA/V if velocity > 200 cm/sec

58
Q

SCD: chelation therapy

A

deferoxamine

59
Q

SCD: non-infectious etiology of ACS

A

fat-emboli

60
Q

SCD: age range for sequestration

A

6 mo - 2 years

61
Q

SCD: recurrence rate for sequestration

62
Q

SCD: chronic complications

A

gallstones, restrictive pulmonary dz, LVH, retinal detachment, delayed puberty, infertility in female patients

63
Q

Hgb Bart’s

A

tetramer of gamma-globin that forms with decrease in alpha-globin synthesis

64
Q

Diagnosis of beta thalassemia

A

increase in HgA2

65
Q

HbH

A

beta-globin tetramers that occur in alpha thalassemia

66
Q

Peak age of Hodgkin lymphoma

A

adolescence

67
Q

Primary location of Hodgkin lymphoma

A

above the diaphragm

68
Q

Most common presentation of NHL

A

lymphadenopathy without fever or weight loss

69
Q

3rd most common childhood cancer

70
Q

Wilms tumor prognosis

A

90% 4 year survival

71
Q

Desmopressin is contraindicated in which type of vWD

72
Q

Prognosis of stage IV Hodgkin disease

73
Q

Hemolysis triggers (G6PD vs. HS)

A

G6PD - drugs, HS - viral infection

74
Q

HS genetics

A

AD (high rate of new mutations)

75
Q

Malignancy most closely associated with TLS

A

Burkitt lymphoma (HL)

76
Q

Findings in TLS

A

elevated K, PO4, uric acid; decreased Ca

77
Q

WAGR

A

wilms tumor, aniridia, genital anomalies, MR

78
Q

Target cells

A

hemoglobinopathy

79
Q

Howell-Jolly bodies

A

splenic dysfunction (SCD)

80
Q

Evaluation of Wilms tumor

A

CT chest/abd/pelvis, echo (r/o extension), CXR

81
Q

Chemo SE: hearing loss

82
Q

Chemo SE: neurotoxicity

A

vincristine/vinblastine

83
Q

Shwachman-Diamond syndrome

A

pancreatic insufficiency (2nd MCC), BM dysfunction, skeletal anomalies, increased risk of leukemia

84
Q

Treatment of autoimmune hemolytic anemia (AIHA)

A

corticosteroids

Pediatrics (14 decks)

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