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1

What confirms dx of leukemia?

bone marrow biopsy with greater than 25%

2

Most common pediatric renal malignancy?

Nephroblastoma

3

Wilms tumor is aka

Nephroblastoma

4

Burr cells are seen in?

Spiculated RBCs of similar size with regularly spaced projections. Seen in uremia or artifact of preparation

5

Pappenheimer bodies.

Seen in sideroblastic anemia. Results from phagosomes that engulf excessive amounts of iron.

6

Heinz bodies seen in

G6PD deficiency

7

Heinz bodies are

when Hb oxidizes in GPD deficiency, they precipitate into these heinz bodies.

8

Universal screening for iron deficiency anemia

complete CBC 9-12 months

9

Tx for iron deficiency anemia

empiric oral iron therapy. Recheck Hg in 4 weeks, if it has risen by 1g/dL, continue for 2-3 months.

10

Tx for ITP

If only skin manifestations: observe, regardless of platelet count. If bleeding, then regardless of platelet count give IVIG or glucocorticoids

11

S pneumo, H influenze and N. meningitidis have what in common?

Encapsulated bacteria

12

Common vaccinations of childhood that use live attentuated viruses?

Measles, mumps, rubella and chicken pox

13

What can prevent pneumococcal sepsis in sickle cell pateints

Pneumococcal vaccine and pencillin propylaxis (kids should get it till age 5)

14

Common vaccinations of childhood with bacterial toxoids

Tetanus and diptheria

15

Epistaxis, visible nasal mass, nasal obstruction

Juvenile angiofibroma

16

Side effects of hydroxyurea treatment

Suppresses the bone marrow. Can cause thrombocytopenia, leukopenia and anemia. but these effects are generally temporary and reversible but can predispose patient to infection.

17

MOA of hydroxyurea

Increases proportion of fetal hemoglobin so that the proportion of HgS is reduced and chances of sickling reduce

18

What bones are frequently affected in osteonecrosis due to sickle cell anemia?

Humeral and femural heads

19

What is anistocytosis

Blood cells of unequal size

20

Lab findings for iron deficiency anemia

Decreased MCV, decreased transferrin, anistocytosis => increased RDW, decreased MCH, decreased trasnferrin saturation, increased TIBC. Smear shows small hypochromic rbcs.

21

MCHC is elevated or depressed in hereditary spherocytosis? Why?

It's elevated. Due to cellular dehydration and membrane loss, the MCHC is high.

22

What are the diagnostic tests for hereditary spherocytosis?

Osmotic fragility test with acidified glycerol lysis test and abnormal eosin-5-maleimide binding test (flow cytometry)

23

What is the typical manifestation of hereditary spherocytosis

Coombs negative hemolytic anemia, jaundice and splenomegaly

24

Genetic pattern of hereditary spherocytosis

75% A/D, 25% spontaneous

25

What are lab findings/diagnostic tests for hereditary spherocytosis

Imp finding is elevated MCHC. Due to cell dehydration and lower cell volume, this is elevated. Spherocytes on peripheral blood smear, coombs negative. Diagnostically do the acidfied glycerol lysis test and the eosin 5 maleimide binding test.

26

How is vitamin K related to factors 2,7,9,10 and factors C and S.

Vitamin K is a cofactor for the enzyme gamma glutamyl carboxylase that adds carboxyl groups to the glutamate residues of several coagulation factors such as 2, 7, 9, 10 and C and S. These extra carboxyl groups add the factors affinities for platelets

27

Most common primary bone tumor in children

Osteosarcoma

28

Osteosarcomas are found most commonly in

Metaphyses of long bones: proximal humerus, distal femur, proximal tibia.

29

PE findings and X -ray findings for osteosarcoma

Large tender mass. X-ray finding: sun-burst periosteal pattern and "codman" triangle.

30

Lymphoblast histology/staining

Lack peroxidase positive material, often contain +PAS staining material, 95% immunostain for TdT. (TdT is only present in pre B and T lymphocytes)