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Hematology/Oncology Flashcards

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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
Alpha thalassemia syndromes
silent carrier, thal trait, HgH disease, hydrops fetalis
26
Cooley's anemia
beta thalassemia major
27
Findings in beta thalassemia
"hair-on-end" skull x-ray, chipmunk facies
28
Peak incidence of death in SCD
6 mo to 3 years
29
Second most common hemoglobin variant
HgbE
30
Measurement of extrinsic pathway
PT/INR (VII, X, V, II, fibrinogen)
31
Measurement of intrinsic pathway
PTT (XII, XI, IX)
32
Peak incidence of ITP
2-5 years
33
Alloimmune thrombocytopenia pathophysiology
maternal IgG against paternal Pla1 Ag (may arise with 1st pregnancy but increased with subsequent pregnancies)
34
Hemophilia genetics
XLR (33% = new mutations)
35
Hemophilia A
factor IIX
36
Hemophilia B
factor IX
37
Hemophilia labs
prolonged PTT
38
vWD genetics
AD (incomplete penetrance)
39
Standard risk ALL
1-10 yo, WBC < 50K, pre-B, hyperdiploid, minimal residual disease
40
Survival in AML
30%
41
Most common presentation for Hodgkin lymphoma
painless LAN (B symptoms = worse prognosis)
42
Infratentorial tumors
``` Most common (70%) Cerebellar astrocytoma, medulloblastoma, ependymoma, brainstem glioma ```
43
MRI findings in medulloblastoma
Contrast enhancing midline tumor (exclusively in the cerebellum)
44
Medulloblastoma treatment
resection, XRT (not in < 3 yo), chemo
45
Most common malignancy in infancy
neuroblastoma
46
Neuroblastoma features associated with poor prognosis
stage III or IV (except IVS), age > 1, high N-myc amplification
47
IVS neuroblastoma
infant (< 1 year), resectable primary and only mets to liver, skin or BM
48
Most common site of rhabdomyosarcoma
head/neck/orbits
49
Retinoblastoma features associated with good prognosis
< 2 years, unilateral, no extension
50
Management of bilateral retinoblastoma
MRI to rule out PNET of pineal gland
51
Hepatoblastoma associations
Beckwith-Wiedeman, FAP
52
Tumors associated with von Hippel Lindau
CNS - retinal hemangioblastoma | Visceral - renal cell carcinoma, pheochromocytoma, pancreatic tumor
53
Pearson syndrome findings
pallor, pancreatic dysfunction, lactic acidemia, FTT
54
RBC indice that differentiates TEC and DBA
MVC - normal in TEC, increased in DBA
55
SCD: common age for dactylitis
< 2 years
56
SCD: rate of stroke
15%
57
SCD: screening for stroke
TCD every 6-12 months, MRA/V if velocity > 200 cm/sec
58
SCD: chelation therapy
deferoxamine
59
SCD: non-infectious etiology of ACS
fat-emboli
60
SCD: age range for sequestration
6 mo - 2 years
61
SCD: recurrence rate for sequestration
50%
62
SCD: chronic complications
gallstones, restrictive pulmonary dz, LVH, retinal detachment, delayed puberty, infertility in female patients
63
Hgb Bart's
tetramer of gamma-globin that forms with decrease in alpha-globin synthesis
64
Diagnosis of beta thalassemia
increase in HgA2
65
HbH
beta-globin tetramers that occur in alpha thalassemia
66
Peak age of Hodgkin lymphoma
adolescence
67
Primary location of Hodgkin lymphoma
above the diaphragm
68
Most common presentation of NHL
lymphadenopathy without fever or weight loss
69
3rd most common childhood cancer
NHL
70
Wilms tumor prognosis
90% 4 year survival
71
Desmopressin is contraindicated in which type of vWD
type 2 B
72
Prognosis of stage IV Hodgkin disease
> 80%
73
Hemolysis triggers (G6PD vs. HS)
G6PD - drugs, HS - viral infection
74
HS genetics
AD (high rate of new mutations)
75
Malignancy most closely associated with TLS
Burkitt lymphoma (HL)
76
Findings in TLS
elevated K, PO4, uric acid; decreased Ca
77
WAGR
wilms tumor, aniridia, genital anomalies, MR
78
Target cells
hemoglobinopathy
79
Howell-Jolly bodies
splenic dysfunction (SCD)
80
Evaluation of Wilms tumor
CT chest/abd/pelvis, echo (r/o extension), CXR
81
Chemo SE: hearing loss
cisplatin
82
Chemo SE: neurotoxicity
vincristine/vinblastine
83
Shwachman-Diamond syndrome
pancreatic insufficiency (2nd MCC), BM dysfunction, skeletal anomalies, increased risk of leukemia
84
Treatment of autoimmune hemolytic anemia (AIHA)
corticosteroids

Pediatrics (14 decks)

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