Hematology and Oncology

Question 1

MC location of medulloblastoma in a child is the cerebellar vermis, so what are the resultant symtpoms? what is the often teh presenting symptom?

Answer 1

BALANCE is affected dt
truncal and gait instability.
MC initial/presenting sx is increased ICP.

Question 2

EPO level in polycythemia vera. why

Answer 2

low because JAK2 is mutated and alwyas activated, so EPO activation of JAK2 is not necessary

Question 3

leukemoid v CML

Answer 3

leukemoid has incrased LAP

Question 4

does enoxaparin or unfractionated heparin have higher risk of HIT?

Answer 4

unfractionated heparin

Question 5

hereditary thrombophilia:
MC in whites, point mutation in ___ that makes it unable to respond to activated protein C, so slowed degradation of this factor and continue dthrombin formation and slowed degredation fo activated F8. PT and PTT nml.

Answer 5

factor 5 leiden

Question 6

hereditary thrombophilia: 2MCC in whites, increased prothrombin levels.

Answer 6

prothrombin mutation (f2)

Question 7

hereditary thrombophilia: inherited form rare. acquired form with DIC, cirrhosis, nephrotic syndrome.

Answer 7

antithrombin deficiency

Question 8

hereditary thrombophilia: decr inactivation of factors 5a and 8a. warfarin induced skin necrosis.

Answer 8

factor C and S deficiency

Question 9

congenital hypoplastic anemia (macrocytic; low retic) and TRIPHALANGEAL THUMBS.

Answer 9

diamond blackfan syndrome

Question 10

macrocytosis, pancytopenia, ABSENT THUMBS, RTA

Answer 10

fanconi anemia

Question 11

concentration of hemoglobin in a given volume of RBC.

Answer 11

MCHC

Question 12

gallstone hx + jaundice + SM + hemolytic anemia = dx?

Answer 12

hereditary spherocytosis

Question 13

when do you give IvIg in ITP?

Answer 13

when platelets are <30,000

Question 14

thalassemia treatment

Answer 14

deferoxamine dt iron overload

Question 15

LMWH v warfarin -

use in ESRD v. Liver disease

Answer 15

LMWH in LIVER disease

Warfarin in ESRD

Question 16

need warfarin bridge in a patient with kidney disease - use what?

Answer 16

use unfractionated heparin to bridge (not LMWH)

Question 17

show as suprasellar calcificantions - low grade malignancy derived form remnants of rathke pouch. Sx: bitermporal hemianopsia (optic hciasm compression). Endocripat -GH deficit, DI (pit stalk compression). in young kids.

Answer 17

cranipharyngiomas

Question 18

how are cranippharyngiomas diff than pit adenoma

Answer 18

Diff from pit adenoma in that pit adenom dont have calcifications and present woth ANTERIOR pit sx - amenorrhea and galatorrhea.

Question 19

drugs that decr folic acid absorptin v. antagonism

Answer 19

phenytoin decr absorp

MTX or TMP are antagonisms

Question 20

Diarrhea, abd pain, vomiting after cookout –1wk later–> abd pain, fatigue, scleral icterus, diff abd tenderness, +2 pitting edema =

Answer 20

hemolytic uremic syndrome

Question 21

MCC of anemia and low retics in sickle cell disease - name of crisis and cause

Answer 21

aplastic crisis, MC secnodary to invfetion like parvo

Question 22

electroyte changes in tumor lysis syndrome

Answer 22

hyperI uricemia and potassium.
hypoCa

hypER-uricema (nucelic acids released and metabolized into uric acid) and kalemia and phosphatemia (K and Phos are released from cells). hypOCa (dt hyperphos bidn and precipitating caclium = reduced intravascular levels).

Question 23

granular casts on UA =

Answer 23

renal insufficiency

Question 24

it K dependent clotting factors =what drug inhibits?

Answer 24

2, 7, 9, 10, c, s — warfarin inhibits all

Question 25

erythrocyte CD55 adn CD59 testing - [HA, cytopenia, hypercoag].

Answer 25

PNH

Question 26

cOOMBS NEGATIVE HEMOLYTIC ANEMIA, jaundice, SM; see reticulocytosis, hyperBr, spherocytosis, FHx of anemia. Dx HS with acidified glycerol lysis test for OSMOTIC FAGILITY.and eosin-5-maleimide binding. Tx with blood transf, folic acid supp, splenectomy.

dx?

Answer 26

hereditary spherocytosis

Question 27

Positive coombs + low Hb + reticulocytosis = ?

tx with CS.

Answer 27

warm agglutinin autoimmune HA

Question 28

Small child with: eczema, micro-thrombocytopenia, recurrent infectoin = ?

Answer 28

XLR WISKOTT-ALDRUCH SYNDROME. WAS gene is cytoskeleton remodeling regulator in hematopoietic cells.

Question 29

Hepatic vein thrombosis + elevated Br + LDH elevation + abd pain (i.e. dt intraabd thormbosis) dark urine + anemia + low haptoglobin = ?

Answer 29

pNH

Question 30

ncr LDH + decr haptoglobin =

Answer 30

hemolytic anemia

Question 31

thrombocytopenia + MAHA + renal insuff + neuro changes + fever =

Answer 31

thrombotic thrombocytopenic purpura

Question 32

etiology of TTP

Answer 32

autoab decreases ADAMTS13, so ADAMTS13 can’t celave vWF off endothelial surface, so vWF b/u and traps plts = THROMBI. Dx: see SCHISTOCYTES on PBS. Tx: plasma exchange.

Question 33

> 90% of Pulm Emb originate in the ____ thigh veins

Answer 33

PROXIMAL thigh veins - femoral, popliteal, iliac.

Question 34

Child <16yo with Congenital marrow failure (erthro/thrombocytopenia, Macrocytosis, anemi leukopenia), poor growth, morphologic abn (thumbs bent), hypopigmenttation. Dx?

Answer 34

A: Faconi anemia TORCH - toxo, other (syph), rubella, CMV, HSV

Question 35

dog + EGGSHELL UNILOCULAR HEP ATIC CYSTS. tx with

albendazole and surgery.

Answer 35

hydatid liver cyst=echinococcus.