Hemonc

Question 1

african american pt with low hematocrit and high reticulocyte count, recurrent episodes of abd pain that resolve with hydration. Ddx

Answer 1

sickle cell anemia

Question 2

acte chest syndrome

Answer 2

vaso-occlusive crisis localized to the pulmonary vasculature that occurs in sickle cell pt

Question 3

autosplenectomy

Answer 3

sickle cell pt. due to vaso-occlusive crises that cause repeated infarctions. Spleen is shrunken,brown discoloration and fibrotic.

Question 4

the primary mechanism of iron deficiency is

Answer 4

blood loss, especially in GI tract

Question 5

cause of hypochromic, microcytic anemia

Answer 5

iron deficiency

Question 6

total body iron content is regulated by

Answer 6

Hepcidin from hepatic parenchymal cells

Question 7

X linked coagulopathy, intramuscular hemorrhage, hemorrhage into large joints, prolonged bleeding following surgery.

Answer 7

Hemophilia A

Question 8

signs of disorder of secondary hemostasis

Answer 8

bleeding from joints and muscles, bleeding after surgery

Question 9

coagulopathy with x-linker inheritance and PTT pronlongation

Answer 9

Hemophilia A: only males can get it

Question 10

deficiency of CD55 and CD59, hemolytic anemia, hypercoagulable state and decreased blood counts

Answer 10

Paroxysmal nocturnal hemoglobinuria

Question 11

bone marrow aspiration in aplastic anemia

Answer 11

dry tap

Question 12

easy bleeding form mucosal surfaces including gingivae, nasal mucosa, GI tract and menorrhagia.

Answer 12

Von willebrand disease

Question 13

inheritance of von willebrand disease

Answer 13

Autosomal dominant with variable incomplete penetrance

Question 14

pt with severe B thalasemia receives regular blood transfusions. Lymph node biopsy revelas phagocytic cells with golden yellow cytoplasmic granules that are composed of

Answer 14

Hemosiderin

Question 15

G6PD inheritance

Answer 15

X linked recessive, males are affected and females r carriers

Question 16

Hereditary spherocytosis inheritance

Answer 16

Autosomal dominant

Question 17

pt with increased plasma volume, low EPO levels, pruritis after hot showers

Answer 17

Polycythemia vera

Question 18

mutation in polycythemia vera

Answer 18

JAK2 making bone marrow more sensitive to growth factors

Question 19

enzyme affected in acute intermittent porphyria

Answer 19

porphobilinogen deaminase

Question 20

enzyme affected in porphyria cutanea tarde

Answer 20

uroporphyrinogen decarboxylase

Question 21

enzymes affected in lead poisoning

Answer 21

ferrochalatase and ALA dehydrogenase (RLS)

Question 22

cryoprecipitate

Answer 22

fibrinogen, factor VIII, factor XIII, vWF and fibronectin

Question 23

Fresh froxen plasma

Answer 23

coagulation factors

Question 24

treatment of DIC, cirrhosis and warfarin overdose

Answer 24

fresh frozen plasma

Question 25

translocation 9:22

Answer 25

CML, bcr-abl

Question 26

Translocaiton 8:14

Answer 26

Burkitt's lymphoma c myc activation

Question 27

trans. 11:14

Answer 27

Mantle cell lymphoma, cyclin D1

Question 28

trans. 14:18

Answer 28

follicular lymphoma, bcl 2

Question 29

trans. 15:17

Answer 29

M3 type of AML, responsive to ATRA

Question 30

heparin overdose treatmenr

Answer 30

Protamine sulfate

Question 31

NSAID that does not impair platelet function

Answer 31

celcoxib: selective COX2 inhibitor

Question 32

low molecular weight heparins

Answer 32

enoxaparin, fondaparinux

Question 33

MOA of heparin

Answer 33

bind to antithrombin and activates it so it can decreased thrombin and factor Xa

Question 34

drug admin. For pt who have had a transient ischemic attack

Answer 34

low dose aspirin

Question 35

RBC membrane defect due to mutations in plama membrane scaffolding proteins spectrin and ankyrin. Cilincal manifestation of hemolytic anemia, jaundice and splenomegaly

Answer 35

hereditary spherocytosis

Question 36

high hematocrit in normal males, small testicular size, acne

Answer 36

steroid drug abuse, androgens stimulate red blood cell production

Question 37

drug of choice for prevention of venous thrombosis in pt undergoing elective surgery

Answer 37

Heparin

Question 38

pt with unusual protein in WBC that shows affinity for vit A derivative

Answer 38

Acute myeloid leukemia M3 type which responds to ATRA

Question 39

argatroban

Answer 39

direct thrombin inhibitor. Used for treatment of heparin induces thrombocytopenia

Question 40

ER/PR positive breast cancer indicates sensitivity to

Answer 40

tamoxifen

Question 41

ERB B2 positive indicated response to

Answer 41

trastuzumab, more aggressive

Question 42

reduciton of risk of non hereditary ovarian cancer

Answer 42

Oral contraceptive: by reducing the amount of times a woman ovulates.

Question 43

increased risk fo ovarian cancer

Answer 43

ovarian dysfunction, infertility, mulliparity, miscarriages, clomiphene citrate to induce ovulation.

Question 44

pt with glanzman thrombasthenia. Treatment with drug that causes the same effect

Answer 44

Abciximab, glycoprotein Iib/IIIa inhibitor that inhibits the binding to fibrinogen

Question 45

normocytic normochromic anemia, hypercalcemia, punched out lesions, increased susceptibility for infection, amyloid deposition of light chains and renal failure

Answer 45

Multiple myeloma

Question 46

Ratelgravir

Answer 46

integrase inhibitor that disrupts the ability of HIV to integrate its genome into the host cell's chromosomes, preventing transcription

Question 47

pentad of fever, neuro problems, acute renal failure, thrombocytopenia, microangiopathic hemolitic anemia

Answer 47

Thrombotic thrombocytopenic purpura and hemolytics uremic syndrome

Question 48

schistocytes on smear

Answer 48

microangiopathic hemolytic anemia or mechanical damage

Question 49

TTP and Hus pentad

Answer 49

fever, neuro, renal, thrombocytopenia, microangiopathic hemolytic anemia

Question 50

lab findings on intravascular hemolytic anemia

Answer 50

ow haptoglobin, increased hemoglobin, LDH and bilirubin

Question 51

diagnostic test for spherocytosis

Answer 51

positive osmotic fragility test

Question 52

what accumulates with methotrexate treatment

Answer 52

dihydrofolic acid polyglutamate

Question 53

part of cell cycle affected by vincristine

Answer 53

prevent separation of choromosomes during M phase

Question 54

treatment for toxicity of cisplatin

Answer 54

Amifostine

Question 55

treament for nausea and vomit in chemotherapy

Answer 55

serotonin receptor antagonist, ondansetron, granisetron and dolasetron

Question 56

cyclophosphamide toxicity treament

Answer 56

mesna

Question 57

prevention of cardiotoxicity by doxorubicin

Answer 57

dexrazoxane

Question 58

treatment of CML

Answer 58

imatinib, inhibits bcr-abl

Question 59

causes of aplastic anemia

Answer 59

radiation, environmental toxins (benzene, arsenicals), drugs such as chloramphenicol

Question 60

causes of malabsorption of B12

Answer 60

pancreatic insufficiency, intestinal bacterial overgrowth and ileal disease.

Question 61

where is folate absorbed

Answer 61

jejunum

Question 62

reed stenberg cells on a background of lymphocytic infiltrates

Answer 62

hodgin lymphoma

Question 63

DIC

Answer 63

long PT and PTT, thrombocytopenia and microangiopathic hemolytic anemia, low fibrinogen, low factor V and VIII, Elevated fibrin split products.