hematology

Question 1

what might a serum ferritin level lower than 100-120 ng/ml in a pt with inflammatory disease suggest?

Answer 1

iron deficiency concomitant with anemia of chronic disease

Question 2

changes in iron studies seen in anemia of chronic disease

Answer 2

decreased transferrin and TIBC level

increased serum ferritin

Question 3

changes in iron studies seen in iron deficiency anemia

Answer 3

increased transferrin and TIBC levels

decreased ferritin levels

Question 4

characteristic finding on BM biopsy in aplastic anemia

Answer 4

hypoplastic BM (<20% cellularity) with normal maturation of all cell lines

Question 5

initial management in pts with aplastic anemia (after BM biopsy)…

Answer 5

withdrawl of any causative agents

CT scan of chest to R/O thymoma

Question 6

in a question stem, what should you look for to dx. aplastic anemia?

Answer 6

look at all 3 cell lines - all will be decreased
reticulocytes will be decreased
should have hypocellular BM biopsy

Question 7

how do you supplement iron in iron deficiency anemia?

Answer 7

oral ferrous sulfate, 325 mg three times daily

Question 8

who should receive parenteral iron therapy?

Answer 8

those pts with iron deficiency anemia who have problems with malabsorption ex. celiac dz, Crohn’s dz, small bowel dz

Question 9

what are “bite” cells in G6PD deficiency caused by?

Answer 9

produced when accumulated oxidized Hb remains adherent to the RBC mb with an adjacent mb bound clear zone; spleen takes a “bite” out of these RBCs

Question 10

how can you tell a pt apart with hereditary spherocytosis and warm-AIHA?

Answer 10

direct Coomb’s test is positive in warm AIHA and negative in spherocytosis

Question 11

characteristic findings in a pt with thalassemia

Answer 11

1 low MCV

2. target cells on PBS
3. normal results on iron studies

Question 12

common lab findings in hemolytic anemia

Answer 12

elevated LDH
decreased haptoglobin
reticulocytosis

Question 13

schistocytes are associated with ? (4)

Answer 13

mechanical/prosthetic heart valves
TTP
HUS
DIC

Question 14

peripheral blood smear findings in iron deficiency anemia

Answer 14

microcytosis
hypochromia
anisocytosis - diff. sizes
poikilocytosis - diff shapes

Question 15

what types of cell characterize warm-AIHA on peripheral blood smear?

Answer 15

spherocytes

Question 16

what does it mean when a “mixing corrects to normal”

Answer 16

pt likely has a factor deficiency

- it will remain abnormal if a factor inhibitor is present

Question 17

how can you make the dx. of DIC?

Answer 17

prolonged PT, aPTT and thrombin time
positive D-dimers
decreased fibrinogen
decreased platelet count
microangiopathic hemolytic anemia

Question 18

what disease do you suspect in a pt who has a personal/family history of bleeding tendency, prolonged bleeding time, elevated aPTT and a low factor VIII level?

Answer 18

von willebrand disease

Question 19

in someone with a low factor VIII level, how can you distinguish vWD from hemophilia A?

Answer 19

vWD causes prolonged bleeding time, while hemophilia A does not

Question 20

best screening test for bleeding disorder

Answer 20

clinical history

Question 21

what risk factors of bleeding require further screening (PT, aPTT, plt count) pre-operatively?

Answer 21

personal/family history of bleeding
liver disease
significant alcohol use
malabsorption
anticoagulation therapy

Question 22

a pt with sickle cell dz presents with transient aplastic crises – what should you consider?

Answer 22

acute infection with parvovirus B19

• dx. with serum IgM abs
• recovery is usually spontaneous

Question 23

management of acute chest syndrome

Answer 23

exchange transfusion

Question 24

best test to diagnose osteonecrosis (avascular necrosis) in pt with sickle cell dz?

Answer 24

MRI of the hip

Question 25

where is pain typically located in avascular necrosis?

Answer 25

groin

Question 26

reflex sympathetic dystrophy

Answer 26

complex regional pain syndrome - localized osteoporosis - pain in extremities with swelling, limited ROM, vasomotor instability and skin changes

Question 27

therapy of pt with sickle cell dz who developed stroke

Answer 27

chronic blood transfusion therapy to maintain peripheral blood HbS level < 50%

Question 28

what should you consider in any pt with an otherwise unexplained decrease in platelet count and/or new thrombotic event 5-10 days after initiation of heparin therapy? how should you tx. them?

Answer 28

1. heparin induced thrombocytopenia | 2. stop heparin and administer argatroban

Question 29

gold standard dx. test for HIT

Answer 29

C14-serotonin release assay | - but negative assay does not exclude HIT

Question 30

TTP is due to deficiency of what?

Answer 30

ADAMST13

Question 31

pentad of TTP

Answer 31

``` neurological symptoms fever renal failure thrombocytopenia micropathic hemolytic anemia ```

Question 32

Tx. of TTP

Answer 32

plasma exchange

Question 33

Evans syndrome

Answer 33

combination of warm AIHA and ITP

Question 34

condition in which platelets clump together and cannot be counted properly

Answer 34

pseudothrombocytopenia

Question 35

how can you dx. pseudothrombocytopenia

Answer 35

finding clumps of platelets (can occasionally adhere to neutrophils) on stained blood film

Question 36

what do you do if you find pseudothrombocytopenia?

Answer 36

redraw pts blood using alternative anticoagulant to EDTA, such as heparin or sodium citrate

Question 37

gestational thrombocytopenia

Answer 37

usually have platelet levels around 70 000

Question 38

first line tx. for pts with ITP

Answer 38

steroids

Question 39

indications for steroid tx. in ITP

Answer 39

symptomatic bleeding and plt count < 50 000 OR plt counts < 15 000

Question 40

Tx of pt diagnosed with antiphospholipid syndrome

Answer 40

indefinite anticoagulation

Question 41

how do you confirm the diagnosis of antiphospholipid syndrome?

Answer 41

2 positive lab tests (anticardiolipid ab or lupus inhibitor assay) ATLEAST 12 weeks apart - this is to ensure the findings were not transient

Question 42

what are the antiphospholipid ab?

Answer 42

IgG anti-cardiolipin | B2-glycoprotein I abs

Question 43

how can you diagnose antiphospholipid syndrome?

Answer 43

history of a thrombotic event (ex. recurrent pregnancy loss) in association w/ persistent lupus anticoagulant or persistently elevated ab

Question 44

who is antiphospholipid syndrome common in?

Answer 44

SLE pts | - can also be been in cancer, infections (HIV) or drugs

Question 45

decreased AG in the presence of anemia, proteinuria, hypercalcemia and renal failure suggests...

Answer 45

multiple myeloma

Question 46

what is MGUS characterized by?

Answer 46

monoclonal spike < 3 g/dL < 10% plasma cells in BM absence of lytic bone lesions, anemia, hypercalcemia or renal failure