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Peds Boards > Hem/Onc > Flashcards

Hem/Onc Flashcards

1
Q

Hemoglobin SC

A

C + S

mild hemolytic anemia

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2
Q

Hemoglobin C Heterozygote

A

Normal

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3
Q

Hemoglobin C Homozygous

A

mild hemolytic anemia, but no vasoocclusive crises

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4
Q

Diamond Blackfan Anemia

A

anemia + thumb anomalies

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5
Q

Wiskott-Aldrich Syndrome

A

x-linked immunodeficiency w/ eczema

thrombocytopenia w/ small platelets

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6
Q

TAR syndrome

A

thrombocytopenia-absent radii syndrome

autosomal recessive, presents in neonatal period

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7
Q

Schwachman-Diamond Syndrome

A

AR, SBDS gene

  • neutropenia
  • metaphyseal dysostoses
  • exocrine pancreatic insuffiency, diarrhea, recurrent infections, FTT
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8
Q

Fanconi Anemia

A 
AR, poor DNA repair
absent thumbs, absent radii
pancytopenia
microcephaly, renal anomalies
cafe-au-lait spots, dark pigmentation
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9
Q

RDW and anemia

A

increased in IDA, normal in thalassemias

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10
Q

B thal major

A
  • Cooley anemia, no B-globin production
  • only HbF present
  • transfusion dependent
  • chipmunk facies (expansion of bone marrow in facial bones)
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11
Q

Cyclic Neutropenia

A

fever, neutropenia (<200), ulcers, cervical lymphadenitis every 21 days

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12
Q

EPO production in utero

A

liver

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13
Q

Bernard-Soulier Syndrome

A

AR
mild thrombocytopenia w/ giant, abnormal platelets
severe mucocutaneous bleeding in infancy

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14
Q

Kostmann Syndrome

A

severe congenital neutropenia

AR, young age w/ severe bacterial infections, ANC <200

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15
Q

Chronic Benign Neutropenia

A

persistent neutropenia but w/o infections

autoantibodies to granulocytes

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16
Q

Complication of goat milk intake

A

folate deficiency

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17
Q

Abnormal thumbs, think….

A

trisomy 18, fanconi anemia

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18
Q

APC resistance

A

Factor V Leiden

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19
Q

Maternal Autoimmune Neonatal Thrombocytopenia due to

A

transplacental passage of maternal IgG antiplatelet antibodies

20
Q

Fanconi Anemia, main complication

A

AML

21
Q

Delayed separation of umbilical cord or omphalitis, clinical clue for

A

leukocyte adhesion deficiency

22
Q

Job Syndrome

A

Hyperimmunoglobulin E syndrome

S aureus infections, skin abscesses

23
Q

Abnormal thumbs, think….

A

trisomy 18, fanconi anemia

24
Q

Evans Syndrome

A

ITP + AIHA

25
Q

Maternal Autoimmune Neonatal Thrombocytopenia due to

A

transplacental passage of maternal IgG antiplatelet antibodies

26
Q

Type 1 vWD

A

most common, decreased in amount of vWF

27
Q

Type 2 vWD

A

2A: decreasing binding
2B: increased binding,but bleeding disorder

28
Q

Type 3 vWD

A

severe, undetectable vWF and low factor 8

29
Q

Kasabach-Merritt Syndrome

A

hemangioma thrombocytopenia syndrome

30
Q

Ewing Sarcoma translocation

A

t11:22

31
Q

Philadelphia Chromosome

A

t9:22

associated with CML

32
Q

Juvenile Monomyelocytic Leukemia

A

<2 years of age

associated with NF1, monosomy 7

33
Q

Burkitt Lymphoma, common location

A

peyer’s patches in GI tract, so ileocecal junction

34
Q

Parinaud Syndrome

A

impaired upward gaze

due to pineal tumors

35
Q

Ewing Sarcoma - features

A

diaphyseal long bones + flat bones (ribs, pelvis)

onion-skinning

36
Q

Osteoarscoma - features

A

sunburst pattern

metaphyseal involvement

37
Q

LiFraumeni Syndrome

A

p53

gliomas, ependymomas, choroid plexus carcinomas

38
Q

Langerhans Cell Histiocytosis staining

A

CD207/langerin

CD1a

39
Q

Osteochondroma - features

A

metaphyses of long bones

broad based projection from surface of the points, cartilage cap

40
Q

hemangioblastomas in CNS, think

A

VHL

41
Q

Most common malignancy in infants

A

neuroblastoma

42
Q

Orbital Chloroma, think…

A

AML

43
Q

Most common CNS tumor in children

A

glioma - nonmalignant

medulloblastoma - malignant

44
Q

Osteoid osteoma - features

A

benign tumor, lucency with surrounding sclerotic bone
often not on x-ray, but on CT
Given NSAIDs, pain due to prostaglandins

45
Q

Most common posterior fossa mass

A

astrocytoma

Peds Boards (21 decks)

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