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Med Study Peds Review > Heme/Onc Boards > Flashcards

Heme/Onc Boards Flashcards

Med study review for heme and onc sections

1
Q

What is Wisckott-Aldrich?

A

X-linked; thrombocytopenia with small plts, eczema, immunodeficiency

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2
Q

What is TAR syndrome?

A

thrombocytopenia with absent radii (nml thumbs); high risk for bleeding in neonatal period due to plts < 30k

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3
Q

common medical history ass’d with ITP

A

recent viral illness or immunization (within past 1-6 wks)

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4
Q

blood smear findings in ITP

A

megathrombocytes

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5
Q

giant platelets on blood smear

A

Bernard-Soulier

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6
Q

blood counts in ITP

A

low plt, nml Hgb, nml WBC

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7
Q

bone marrow findings in ITP

A

normal to increased megakaryocytes

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8
Q

1st line tx of ITP

A

IVIG

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9
Q

TTP cause

A

ADAMTS13 antibodies

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10
Q

Kasabach-Merritt syndrome

A

destruction of plts in vacsular tumors (hemangioma) of the skin, liver, or spleen; aka hemangioma- thrombocytopenia syndrome

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11
Q

clinical features of vWD

A

AD; mild to moderated bleeding in mucosal surfaces or with procedures, heavy menses.

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12
Q

lab eval for vWD

A

decreased vWF antigen with proportional decreased in Factor 8 activity, decreased activity measured by ristocentin cofactor assay

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13
Q

treatment for vWD

A

DDAVP, aminocaproic acid, transexamic acid

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14
Q

thombocytopenia, abnormal platelets than do not aggregate in response to ristocetin but do aggregated in response to ADP, epi, or collagen

A

Bernar-Soulier syndrome

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15
Q

nml plt count, poor plt aggregation in response to ADP, epi, and collagen

A

Glanzamann Thrombasthenia

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16
Q

differentiate primary hemostatic problem from coag problem

A

1 = petechiae, coag = deep bleeds (hemearthrosis)

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17
Q

prolonged PT, nml PTT

A

aqu or ihn F 7 problem, milk vit K def, mild liver dz, therapeutic warfarin

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18
Q

prolonged PTT, nml PT, corrects with mixing study

A

F 8, 9 , 11, 12 def

19
Q

prolonged PTT, nml PT, does not correct with mixing study

A

inhibitor present, like SLE antiCoag

20
Q

What is hemophilia A, what does PT and PTT look like with this dz

A

F8 def, prolonged PTT with nml PT

21
Q

X-linked factor clotting defs

A

8 & 9

22
Q

AR factor clotting defs

A

11, 12, 13, 5,7, 10

23
Q

what med can you give a kid with mild hemophilia A prior to tooth ext & what test should be done prior

A

DDAVP and DDAVP challenge

24
Q

What is hemophilia B, how do you tx it?

A

F 9 def, tx with F9 concentrate

25
Q

which F def doesn’t result in bleeding

A

F 12 def

26
Q

vit K dep coag factors

A

10, 9, 7, 2, prot C, prot S

27
Q

Skin necrosis with warfarin and why?

A

prot C def - no prot C to serve as anti-thrombotic agent during transient hypercoaguable state during initiation of warfarin, bridge with heparin

28
Q

Cause of neonatal purpura fulminans in infancy

A

homozygous protein C or S def

29
Q

single most common childhood malignancy

A

ALL

30
Q

Synds at increased risk for ALL

A

downs, Ataxia telang, bloom, fanconi anemia, NF

31
Q

pallor and limp in a child

A

ALL

32
Q

CBC findings in ALL presentation

A

anemia, reticulocytopenia, +/- neutrophilia

33
Q

BM bx with leukemic lymphoblasts

A

ALL

34
Q

most impt predicitive factor for 2nd remission in ALL

A

length of time of 1st remission

35
Q

Synds at increased risk for AML

A

downs, fanconi anemia, kostmann, PNH, bloom, NF

36
Q

orbital or epidural chloroma

A

AML - collection of leukemia cells

37
Q

auer rod

A

AML

38
Q

t(9;22)

A

Philadelphia chromosome, CML

39
Q

<2yo, splenomegaly, elevated fetal Hgb, thrombocytopenia, rash

A

juvenile myelomonocytic leukemia

40
Q

tx for aplastic anemia (2)

A

immunosuppression with ATG, cyclosporine, + steroids; or BMT

41
Q

blood products given to aplastic anemia pts should be _________ to prevent ________

A

leukoreduced, alloimunization

42
Q

lab and cong anomalies ass’d with fanconi anemia

A

PANcytopenia, (AR, DNA repair imparied), absent or abnl thumbs, short staturs, abnl radii, micocephaly, cafe-au-lait, renal anomalies

43
Q

fanconi anemia at risk for other malignancies

A

AML>ALL, hepatic, squamous cell carcinoma

44
Q

3 MC types of red cell aplasias and differentiation

A

Parvo - school aged, rash, fever, arthropathy
TEC (trans erythroblastopenia of childhood) - 1-4 yo, normocytic RBCs
DBA (diamond blackfan) - <1 yo, macrocytic RBCs, congenital anomalies (SS, glaucoma, webbed neck, CHD, DD, renal abn, hypogonadism)

Med Study Peds Review (1 decks)

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