MKSAP: Hematology

Question 0

What happens to iron levels during an infection? Why?

Answer 0

• the iron levels decrease during an infection bc there is an increase in hepcidin and bacterial lactoferrin binds to the iron (bacteria use iron to support their growth)
• cytokine levels also increase, which affects transferrin and ferritin expression = low total iron binding capacity & low transferrin levels & high ferritin levels

Question 1

3 Major criteria for dx of polycythemia vera?

Answer 1

1. Elevated RBC mass
2. Normal arterial oxygen saturation
3. Splenomegaly

Question 2

What is seen in the CBC and bone marrow in aplastic anemia?

Answer 2

• CBC = pancytopenia

- bone marrow = hypocellular hone marrow

Question 3

3 Features of Autoimmune hemolytic anemia?

Answer 3

1. Anemia
2. Elevated reticulocyte count
3. Microspherocytes on peripheral blood smear

Question 4

Serum methylmalonic acid and homocysteine concentrations in vitamin B12 deficiency v. Folate deficiency?

Answer 4

• B12: both methylmalonic acid and homocysteine concentrations are elevated
• Folate: only homocysteine concentrations are elevated, not methylmalonic acid

Question 5

What is the mean corpuscular hemoglobin concentration in hereditary sphereocytosis?

Answer 5

-elevated

Question 6

3 Hematologic findings in iron deficiency anemia on the peripheral blood smear?

Answer 6

1. Hypochromatic RBCs
2. Abnormalities in the size and shape of RBCs
3. Occasional bizarre shaped (ex cigar) RBCs

Question 7

What is seen on the peripheral blood smear in a pt with alpha-thal trait?

Answer 7

-target cells

Question 8

Initial tx in pts with warm-antibody autoimmune hemolytic anemia?

Answer 8

-corticosteroid tx

Question 9

What is required for the dx of myelodysplastic syndrome?

Answer 9

-cytopenia of at least one of the cell lines

Question 10

In what 3 settings can an acquired factor VIII inhibitor coagulopathy occur?

Answer 10

1. Some malignancies –> ex lymphomas
2. Some autoimmune diseases
3. Postpartum

Question 11

Dx for an acquired factor VIII inhibitor coagulopathy?

Answer 11

• mixing study
• patient’s plasma and normal plasma are mixed in 1:1 ratio & the abnormal test is repeated –> if the abnormality is due to a factor VIII deficiency, the abnormality will correct with mixing, but if the abnormality is due to an inhibition, the abnormality will not correct with mixing!

Question 12

What is the leading cause of death in a pt with sickle cell anemia?

Answer 12

-acute chest syndrome

Question 13

5 Ssx of acute chest syndrome?

Answer 13

1. Fever
2. Chest pain
3. Shortness of breath
4. Hypoxia
5. Radiographically detected pulmonary infiltrate
   * * in a pt with a sickling disorder (SS)

Question 14

2 Most common infectious causes of acute chest syndrome?

Answer 14

1. Chlamydia

2. Mycoplasma

Question 15

What drug should be used to prevent renal dz in sickle cell pts that show signs of developing kidney dysfunction?

Answer 15

-ACEi

Question 16

5 Ssx of TTP?

Answer 16

1. Fever
2. Neurologic abnormalities
3. Thrombocytopenia
4. Microangiopathic hemolytic anemia –> see: anemia, schistocytes, and elevated lactate dehydrogenase concentration
5. Renal insufficiency

Question 17

3 Ssx of HUS?

Answer 17

1. Thrombocytopenia
2. Microangiopathic hemolytic anemia
3. Renal disease

Question 18

4 Signs of DIC?

Answer 18

1. Elevated prothrombin time
2. Elevated activated partial thromboplastin time
3. Low fibrinogen levels
4. Thrombocytopenia

Question 19

Tx of HIT?

Answer 19

• stop heparin immediately

- use an alternative anticoagulation tx = direct thrombin inhibitor

Question 20

Ssx of ITP?

Answer 20

1. Severe thrombocytopenia
2. Normal hemoglobin
3. Normal leukocytes
4. Absence of other sx, such as fever, headache, other constitutional manifestations

Question 21

What is the most common cause of thrombocytopenia during pregnancy? Tx?

Answer 21

• incidental thrombocytopenia of pregnancy

- tx: none, its a benign condition, just monitor the pt and platelet levels

Question 22

What does HELLP stand for?

Answer 22

• Hemolysis with microangiopathic blood smear
• Elevated Liver enzymes
• Low Platelets

Question 23

Tx for TTP

Answer 23

-plasma exchange ASAP

Question 24

2 Most common mutations that predispose to venous thrombosis? What ethnicities are they most common in?

Answer 24

1. Factor V Leiden
2. Prothrombin G20210A
   - most common in whites, rare in Asian and black populations

Question 25

What 2 proteins are decreased during pregnancy? When else are they decreased?

Answer 25

-protein C and protein S are decreased during pregnancy and when using oral anticoagulants

Question 26

In what 2 situations are protein C & S concentrations decreased in patients?

Answer 26

1. Pregnancy

2. When they are on oral anti-coagulants

Question 27

Tx for Philadelphia chromosome positive CML?

Answer 27

-imantinib mesylate –> targets BCR-ABL

Question 28

2 Micro bio signs of Chronic myeloid leukemia?

Answer 28

1. Elevated leukocyte count

2. Increased numbers of granulocytic cells in all phases of development on peripheral blood smear

Question 29

2 Microbio signs of Acute Myeloid Leukemia?

Answer 29

1. Pancytopenia

2. Increased myeloid blasts

Question 30

What are patients with refractory anemia and an excessive amount of blasts at risk for?

Answer 30

-transforming into an acute leukemia during their lifetime

Question 31

4 Common ssx of multiple myleoma?

Answer 31

1. Hypercalcemia
2. Bone pain
3. Anemia
4. Clusters of Large plasma cells on bone marrow aspirate