Hubbard MDS and MPD Flashcards
what is the general clinical picture of MDS
pancytopenia with hyperplastic marrow
What are the drugs that can cause myelodysplasia
- Alkylating agents (cyclophosphamide, ifosfamide, cisplatin, carboplatin, nitrogen mustard)
- Anthracycline antibiotics (adriamycin, daunrubicin, epirubicin)
Age group for MDS
mostly elderly
cytogenetics for MDS
- partial or total loss of long arm of chromosome 5 or 7
- inversion of chromosome 16
- trisomy 8
Lab abnormalities in MDS
- elevated serum LDH
- Evidence (in some) of iron overload
- increased serum ferritin
- serum Fe and TIBC often normal
what do you suggest in patients with pancytopenia to look like a rockstar
- aplastic anemia
- hypersplenism
- MDS
mitochondria laden with Fe encircling the nucleus of the erythroid precursors
refractory anemia with ringed sideroblasts (RARS)
deficiency in what is sometimes associated with anemia with ringed sideroblasts
pyridoxine . . vitamin B6
what are the adverse prognostic features for MDS
- marrow blasts >5%
- platelets <100k
- Hb < 10
- Neutrophils <2500
- Age over 60
What cytogenetic abnormalities are associaed with poor prognosis in MDS?
- monosomy 7
- hyperdiploidy
- multiple abnormalities
What cytogenetic abnormality has a favorable prognosis in MDS?
what drug does it respond to?
5q- syndrome
-Lenalidomide . . need to add thromboembolic prophylaxis . . aspirin
what do you give a patient with iron overload and MDS
- desferrioxamine (Desferal) or
- Deferasirox (Exjade)
what serum EPO level has poor response to Erythropoietin supplementation
> 500
What are the hypomethylating agents used to treat MDS (low-intermediate therapy)
-Azacitidine and Decitabine
What is the high intensity therapy for MDS
- AML induction style
- Hematopoietic stem cell transplantation
when is hematopoietic stem cell transplantation considered in MDS
patient who is <60 and have an HLA-matched sibling donor
a primary myeloproliferative disorder characterized by marrow fibrosis and extramedullary hematopoiesis
Myelofibrosis
Triad in myelofibrosis
- leukoerythroblastic anemia
- Poikilocytosis
- Splenomegaly (HUGE)
pathogenesis of myelofibrosis
- increased reticulin deposition in marrow
- suspected to be secondary to increased (PDGF) and other cytokine
- increased megakaryocytes can be seen
- dry tap
what mutation in in 45-65% of myelofibrosis
JAK2
treatment of pancytopenia in myelofibrosis
- anemia: transfusions
- EPO
- Transfuse platelets if bleeding occurs
- Growth factors for neutropenia
- treat infections aggressively
What is most common cause of death in myelofibrosis
overwhelming infection
treatment of splenomegaly in myelofibrosis
- hydroxyurea variably effective
- Radiation helpful
- in severe cases, splenectomy . . high mortality tho
what is the JAK inhibitor approved for treatment of intermediate and high risk myelofibrosis
Ruxolitinib
