Hem/Onc

Question 1

precipitants of hemolytic crisis in pts w/ G6PD def

Answer 1

sulfonamides, antimalarial drugs, fava beans

Question 2

most common inherited cause of hyper-coagulability

Answer 2

factor V leiden mutation

Question 3

most common inherited bleeding disorder

Answer 3

von willebrand’s dz

Question 4

most common inherited hemolytic anemia

Answer 4

hereditary spherocytosis

Question 5

diagnostic test for hereditary spherocytosis

Answer 5

osmotic fragility test

Question 6

pure RBC aplasia

Answer 6

diamond-blackfan anemia

Question 7

meds and viruses that lead to aplastic anemia

Answer 7

chloramphenicol, sulfonamides, radiation, HIV, chemotherapeutics, hepatitis, parvovirus B19, and EBV

Question 8

distinguish polycythemia vera from secondary polycythemia

Answer 8

both have increased hematocrit and RBC mass, but polycythemia vera should have normal O2 sat and low EPO levels

Question 9

TTP pentad

Answer 9

FAT RN - fever, anemia, thrombocytopenia, renal dysfunction, neurologic abnormalities

Question 10

HUS triad

Answer 10

anemia, thrombocytopenia, and acute renal failure

Question 11

tx for TTP

Answer 11

emergent large-volume plasmapheresis, corticosteroids, anti-platelet drugs. platelet transfusion is contraindicated!

Question 12

auer rods on blood smear

Answer 12

AMl

Question 13

AML subtype associated w/ DIC. tx?

Answer 13

M3. tx w/ retinoic acid

Question 14

electrolyte changes in tumor lysis syndrome

Answer 14

decreased Ca, increased K, increased phosphate, increased creatinine, increased LDH, and increased uric acid* everything is high except Ca

Question 15

intracellular inclusions seen in thalassemia, G6PD def, and post-splenectomy

Answer 15

heniz bodies

Question 16

virus associated w/ aplastic anemia in pts w/ sickle cells anemia

Answer 16

parvovirus B19

Question 17

four causes of microcytic anemia

Answer 17

TAILS - thalassemia, anemia of chronic dz, iron def, lead poisoning, and sideroblastic anemia

Question 18

autoimmune hemolytic anemia (AIHA) and hereditary spherocytosis (HS) can cause extravascular hemolytic anemia. peripheral blood smear may show spherocytes w/o central pallor in both. how do you differentiate them?

Answer 18

AIHA = - family hx and + coombs testHS = + family hx and - coombs tests

Question 19

recent chemo + fever

Answer 19

febrile neutropenia (hem/onc emergency)

Question 20

IgM spike on electrophoresis + hyperviscosity + s/s of increased size of spleen, liver, and some lymph nodes, tiredness due to anemia, tendency to bleed/bruise easily, night sweats, HA/dizziness, visual problems, pain/numbness in extremities = dx?

Answer 20

waldenstrom’s macroglobulinemia

Question 21

transplant rejection
hyperacute - time, path, tx?
acute - time, path, tx?
chronic - time, path, tx?

Answer 21

hyperacute - w/in mins; due to preformed antibodies (prevent w/ ABO compatibility); tx w/ cytotoxic agents

acute - w/in days to months; due to T-cell mediated destruction; tx w/ corticosteroids, antilymphocyte antibodies (OKT3), tacrolimus, or MMF

chronic - w/in months to years; due to chronic immune reaction causing fibrosis causing gradual loss of organ function; no tx available

Question 22

transfusion reactions…

non-hemolytic febrile rxn - mech, s/s, tx?

minor allergic rxn - mech, s/s, tx?

hemolytic transfusion rxn - mech, s/s, tx?

Answer 22

transfusion reactions…
non-hemolytic febrile rxn:
mech = due to cytokine formation during storage of blood
s/s = fever, chills, rigors, and malaise 1-6 hrs after transfusion
tx = stop transfusion and give tylenol

minor allergic rxn:
mech = due to antibody formation against donor proteins
s/s = prominent urticaria
tx = give anti-histamines and if severe, possibly stop transfusion and give epi

hemolytic transfusion rxn:
mech = due to antibody formation against donor erythrocytes, resulting either from ABO incompatbility or from minor antigen mismatch
s/s = fevers, chills, nausea, flushing, tachycardia, hypotension during or shortly after transfusion
tx = stop transfusion and give increased IVF

Question 23

iron studies in iron def anemia
MCV = ?
iron = ?
TIBC = ?
Ferritin = ? 
Transferritin Sat = ?

Answer 23

iron studies in iron def anemia
MCV = decreased
iron = decreased
TIBC = increased
Ferritin = decreased
Transferritin Sat = decreased

Question 24

iron studies in anemia of chronic disease
MCV = ?
iron = ?
TIBC = ?
Ferritin = ?
Transferritin Sat = ?

Answer 24

iron studies in anemia of chronic disease
MCV = normal/decreased
iron = decreased
TIBC = increased
Ferritin = increased
Transferritin Sat = normal/decreased

Question 25

iron studies in thalessemia
MCV = ?
iron = ?
TIBC = ?
Ferritin = ?
Transferritin Sat = ?

Answer 25

iron studies in thalessemia
MCV = decreased
iron = normal/increased
TIBC = decreased
Ferritin = increased
Transferritin Sat = increased

Question 26

3 causes of microangiopathic hemolytic anemia

Answer 26

HUS, TTP, and DIC

Question 27

pt presents w/ the 5 P’s - painful abdomen, port wine-colored urine, polyneuropathy, psychological disturbances, precipitated by drugs, alcohol, and starvation

Answer 27

acute intermittent porphyria (due to defect in prophobilinogen deaminse leading to accumulation of prophobilinogen, ALA, corphorphobilinogen)

Question 28

pt presents w/ blistering cutaneous photosensitivty

Answer 28

porphyria cutanea tarda (due to defect in uroporphyrinogen decarboxylase leading to accumulation of uroporphyrin (tea-colored urine))

Question 29

multiple myeloma - CRABBI

Answer 29

hyperCalcemia, Renal insufficiency, Anemia, Bone lytic lesions/Back pain, Bence Jones proteinuria, IgM (monoclonal M protein spike)

Question 30

major cause of anemia in pts w/ ESRD? type of anemia? tx? s/e of tx?

Answer 30

major cause is due to def of erythropoeitin. anemia is normocytic and normochromic. tx of choice is recombinant erythropoietin (started at Hb