Hubbard MDS and MPD

Question 1

what is the general clinical picture of MDS

Answer 1

pancytopenia with hyperplastic marrow

Question 2

What are the drugs that can cause myelodysplasia

Answer 2

• Alkylating agents (cyclophosphamide, ifosfamide, cisplatin, carboplatin, nitrogen mustard)
• Anthracycline antibiotics (adriamycin, daunrubicin, epirubicin)

Question 3

Age group for MDS

Answer 3

mostly elderly

Question 4

cytogenetics for MDS

Answer 4

• partial or total loss of long arm of chromosome 5 or 7
• inversion of chromosome 16
• trisomy 8

Question 5

Lab abnormalities in MDS

Answer 5

• elevated serum LDH
• Evidence (in some) of iron overload
• increased serum ferritin
• serum Fe and TIBC often normal

Question 6

what do you suggest in patients with pancytopenia to look like a rockstar

Answer 6

• aplastic anemia
• hypersplenism
• MDS

Question 7

mitochondria laden with Fe encircling the nucleus of the erythroid precursors

Answer 7

refractory anemia with ringed sideroblasts (RARS)

Question 8

deficiency in what is sometimes associated with anemia with ringed sideroblasts

Answer 8

pyridoxine . . vitamin B6

Question 9

what are the adverse prognostic features for MDS

Answer 9

• marrow blasts >5%
• platelets <100k
• Hb < 10
• Neutrophils <2500
• Age over 60

Question 10

What cytogenetic abnormalities are associaed with poor prognosis in MDS?

Answer 10

• monosomy 7
• hyperdiploidy
• multiple abnormalities

Question 11

What cytogenetic abnormality has a favorable prognosis in MDS?
what drug does it respond to?

Answer 11

5q- syndrome

-Lenalidomide . . need to add thromboembolic prophylaxis . . aspirin

Question 12

what do you give a patient with iron overload and MDS

Answer 12

• desferrioxamine (Desferal) or

- Deferasirox (Exjade)

Question 13

what serum EPO level has poor response to Erythropoietin supplementation

Answer 13

> 500

Question 14

What are the hypomethylating agents used to treat MDS (low-intermediate therapy)

Answer 14

-Azacitidine and Decitabine

Question 15

What is the high intensity therapy for MDS

Answer 15

• AML induction style

- Hematopoietic stem cell transplantation

Question 16

when is hematopoietic stem cell transplantation considered in MDS

Answer 16

patient who is <60 and have an HLA-matched sibling donor

Question 17

a primary myeloproliferative disorder characterized by marrow fibrosis and extramedullary hematopoiesis

Answer 17

Myelofibrosis

Question 18

Triad in myelofibrosis

Answer 18

• leukoerythroblastic anemia
• Poikilocytosis
• Splenomegaly (HUGE)

Question 19

pathogenesis of myelofibrosis

Answer 19

• increased reticulin deposition in marrow
• suspected to be secondary to increased (PDGF) and other cytokine
• increased megakaryocytes can be seen
• dry tap

Question 20

what mutation in in 45-65% of myelofibrosis

Answer 20

JAK2

Question 21

treatment of pancytopenia in myelofibrosis

Answer 21

• anemia: transfusions
• EPO
• Transfuse platelets if bleeding occurs
• Growth factors for neutropenia
• treat infections aggressively

Question 22

What is most common cause of death in myelofibrosis

Answer 22

overwhelming infection

Question 23

treatment of splenomegaly in myelofibrosis

Answer 23

• hydroxyurea variably effective
• Radiation helpful
• in severe cases, splenectomy . . high mortality tho

Question 24

what is the JAK inhibitor approved for treatment of intermediate and high risk myelofibrosis

Answer 24

Ruxolitinib

Question 25

What is the only curative treatmentfor myelofibrosis

Answer 25

allogeneic stem cell transplantation

Question 26

what is seen on peripheral smear in MDS?

Answer 26

Pegler-Huet cells

-dwarfed megakaryocytes

Question 27

protein stains red with congo red and becomes apple green when light is polarized

Answer 27

amyloidosis

Question 28

Treatment of polycythemia vera

Answer 28

phlebotomy . . if not then hydroxyurea

-avoid Fe