Heme 2

Question 1

Pt NOT taking any medications. Does not have any Medical Problems. B12 is normal. Patient does not Drink. He has evidence of Anemia, leukopenia, thrombocytopenia, elevated MCV. Blood Smear shows Ovalmacrocystosis and HYPOsegmentated Neutrophils. Bone marrow biopsy/Aspirate Hypercellular Marrow with dysplasia. Normal B12 and folate levels.

Dx?
Test?
Rx?

Answer 1

MDS

Elevated MCV but normal B12 and Folate, No drugs, No ETOH.

Bone marrow biopsy (hypercellular marrow with dysplastic erythroid precursors) to confirm.

Supportive infusions.
STEM Cell Transplant < 50 years age.

Lenalidomide for older.

Question 2

Abdominal cramping, bloody diarrhea, haptoglobin undetectable, hgb 6.1, plt 34, retic count 9.8%, Cr 3.6, 3+ blood on UA, Coombs test negative.

Dx?
Rx?

Answer 2

Hemolytic Uremic Syndrome.
(Shiga toxin producing Ecoli)

Supportive

Question 3

Common cause of Heparin Resistance? ( failure of the aPTT to increase)

Answer 3

Antithrombin deficiency

(Heparin- Antithrombin complex Inactivates thrombin)
so they keep clotting

Question 4

Pt presenting with prolonged bleeding from venipuncture sites, new ecchymoses, poor oral intake on abx and has C. diff diarrhea. Dx?

Answer 4

Vit K deficiency

Give oral or IV Vit K

Question 5

1. Low ferritin, low iron, high TIBC, low % saturation ?
2. High ferritin, low iron, low TIBC , low sat?

Answer 5

1. Iron deficiency anemia
2. Anemia of inflammation

Question 6

Mild intramedullary hemolysis, elevated lDH, decrease haptoglobin, degreased reticulocyte count, increased indirect billi

1. Increased MMA and Homocysteine levels?
2. Normal MMA levels and Increased homocysteine levels.

Answer 6

B12 deficiency

Folate deficiency

Question 7

Similar to SS pt present with elevated reticulocyte count and tender abdomen?

Answer 7

Splenic Sequestration complication of hemoglobin SC disease.

Question 8

Main hemoglobin Elevated B thalassemia?

Answer 8

A2

Question 9

What diseases are important to rule out for ITP?

Management platelets > 30,000 ?
Management of platelets < 30,000 ?

Answer 9

Drugs,HIV, HCV, SLE, CLL, Lymphoma.

> 30,000 Observe

< 30,000 Steroids or IVIG or splenectomy

Question 10

Microangiopathic hemolytic anemia, Thrombocytopenia, FEVERS, Renal dysfunction.

Dx?
Test?
Rx?

Answer 10

TTP (FAT-RN)

Inherited gene defect in ADAMTS 13 or anti-ADAMTS13 from infection/drugs/preg/cancer

Plasmapharesis/Plasma exchange

Question 11

Difference from TTP and HUS?

Answer 11

HUS has more Renal involvement

Both can be caused by Shiga toxin

Question 12

Difference btw TTP and DIC?

Answer 12

PT and PTT are prolonged in DIC because the ALL clotting factors are affected

TTP will just have Prolonged Bleeding Time

Question 13

Life threatening bleeds, associated with solid tumors, CLL, post part, hemophiliacs. Mixing study does not correct PTT.

Answer 13

Factor 8 inhibitor

Question 14

Elevated PTT, Normal PT but No clinical bleeding disorder?

Answer 14

Factor 12 deficiency

Question 15

Bleeding, prolonged PTT, Corrects with mixing study, X-linked disorder?

Answer 15

Factor 8 deficiency

Question 16

Bleeding, decreased absorption in diet, associated Decreased levels 27910CS ?

Answer 16

Vit K deficiency

Question 17

Name the times you will use each:
1. Massive PE, unstable, renal failure, MI

2. HIT?
3. Malignancy and Pregnancy
4. Renal disease?

Answer 17

1. IV UFH (Heparin)
2. Agatroban, Bivalirudin
3. LMWH
4. LMWH, fondaparinux

Question 18

Pancytopenia due to Idiopathic, Drugs (phenytoin, sulfa) or Viral infection (Parovirus B19).
Pt presents with bleeding, infection, anemia.

Dx?
Test?
Rx?

Answer 18

Aplastic Anemia

Bone Marrow Tap is hypocellular

STEM Cell Transplant in Younger
Immunosuppression in Older patients

Question 19

Pt presenting with DIC, Auerods present.

Dx?
Rx?
Why to treat immediately?

Answer 19

APL with 15/17 translocation a subset of AML.

Rx with ATRA

Rx immediately, high mortality from hemorrhage

Question 20

Pt present with CNS symptoms, Cranial nerve Defects, associated with 9/22 BCR-ABL translocation.

Dx?

Answer 20

ALL

Question 21

Increased WBC in comparison to RBCs. Associated with thrombocytosis, early satiety, hepatosplenomegaly, 9/22 translocation, BCR/ABL .

Dx?
Rx?

Answer 21

CML

Imatinib, Tyrosine Kinase Inhibitor turn off tyrosine kinase to induce apoptosis

Question 22

Associated with very rapid growth, Tumor Lysis Syndrome, EBV in Africa?

Answer 22

Burkitt lymphoma (High Grade Lymphomas)

Question 23

Hairy cells seen on bone marrow biopsy. Associated with dry tap. Dx? Rx?

Answer 23

Hairy Cell leukemia

Cladribine

Question 24

Platelet Transfusion Threshold:

1. No other risk factors for bleeding.
2. Intracranial bleeding ?
3. Bleeding for planned surgery?

Answer 24

1. Transfuse < 10,000- 20,000 (Lowest Threshold)
2. < 100,000 (Higher threshold)
3. < 50,000