Heme/Onc Boards

Question 1

What is Wisckott-Aldrich?

Answer 1

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

Question 2

What is TAR syndrome?

Answer 2

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

Question 3

common medical history ass’d with ITP

Answer 3

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

Question 4

blood smear findings in ITP

Answer 4

megathrombocytes

Question 5

giant platelets on blood smear

Answer 5

Bernard-Soulier

Question 6

blood counts in ITP

Answer 6

low plt, nml Hgb, nml WBC

Question 7

bone marrow findings in ITP

Answer 7

normal to increased megakaryocytes

Question 8

1st line tx of ITP

Answer 8

IVIG

Question 9

TTP cause

Answer 9

ADAMTS13 antibodies

Question 10

Kasabach-Merritt syndrome

Answer 10

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

Question 11

clinical features of vWD

Answer 11

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

Question 12

lab eval for vWD

Answer 12

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

Question 13

treatment for vWD

Answer 13

DDAVP, aminocaproic acid, transexamic acid

Question 14

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

Answer 14

Bernar-Soulier syndrome

Question 15

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

Answer 15

Glanzamann Thrombasthenia

Question 16

differentiate primary hemostatic problem from coag problem

Answer 16

1 = petechiae, coag = deep bleeds (hemearthrosis)

Question 17

prolonged PT, nml PTT

Answer 17

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

Question 18

prolonged PTT, nml PT, corrects with mixing study

Answer 18

F 8, 9 , 11, 12 def

Question 19

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

Answer 19

inhibitor present, like SLE antiCoag

Question 20

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

Answer 20

F8 def, prolonged PTT with nml PT

Question 21

X-linked factor clotting defs

Answer 21

8 & 9

Question 22

AR factor clotting defs

Answer 22

11, 12, 13, 5,7, 10

Question 23

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

Answer 23

DDAVP and DDAVP challenge

Question 24

What is hemophilia B, how do you tx it?

Answer 24

F 9 def, tx with F9 concentrate

Question 25

which F def doesn't result in bleeding

Answer 25

F 12 def

Question 26

vit K dep coag factors

Answer 26

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

Question 27

Skin necrosis with warfarin and why?

Answer 27

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

Question 28

Cause of neonatal purpura fulminans in infancy

Answer 28

homozygous protein C or S def

Question 29

single most common childhood malignancy

Answer 29

ALL

Question 30

Synds at increased risk for ALL

Answer 30

downs, Ataxia telang, bloom, fanconi anemia, NF

Question 31

pallor and limp in a child

Answer 31

ALL

Question 32

CBC findings in ALL presentation

Answer 32

anemia, reticulocytopenia, +/- neutrophilia

Question 33

BM bx with leukemic lymphoblasts

Answer 33

ALL

Question 34

most impt predicitive factor for 2nd remission in ALL

Answer 34

length of time of 1st remission

Question 35

Synds at increased risk for AML

Answer 35

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

Question 36

orbital or epidural chloroma

Answer 36

AML - collection of leukemia cells

Question 37

auer rod

Answer 37

AML

Question 38

t(9;22)

Answer 38

Philadelphia chromosome, CML

Question 39

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

Answer 39

juvenile myelomonocytic leukemia

Question 40

tx for aplastic anemia (2)

Answer 40

immunosuppression with ATG, cyclosporine, + steroids; or BMT

Question 41

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

Answer 41

leukoreduced, alloimunization

Question 42

lab and cong anomalies ass'd with fanconi anemia

Answer 42

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

Question 43

fanconi anemia at risk for other malignancies

Answer 43

AML>ALL, hepatic, squamous cell carcinoma

Question 44

3 MC types of red cell aplasias and differentiation

Answer 44

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)