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
What is the only curative treatmentfor myelofibrosis
allogeneic stem cell transplantation
what is seen on peripheral smear in MDS?
Pegler-Huet cells
-dwarfed megakaryocytes
protein stains red with congo red and becomes apple green when light is polarized
amyloidosis
Treatment of polycythemia vera
phlebotomy . . if not then hydroxyurea
-avoid Fe
