0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:17
1030 Myelodysplastic Syndrome■Viral marrow suppression
➣Epstein-Barr, hepatitis, parvovirus B19, HIV and others
■Marrow infiltration
➣Acute and chronic leukemias
➣Metastatic solid tumor infiltration
■Paroxysmal Nocturnal Hemoglobinuria
■Hypersplenism
management
What to Do First
■Assess severity of pancytopenia; treat with supportive care
■Evaluate candidacy for allogeneic stem cell transplantation, espe-
cially in high-risk patients (see Prognosis)
General Measures
■Immediate attention to signs or symptoms of infection. Consider
prophylactic antibiotics for repeat infections.
■Transfuse PRBC for symptomatic anemia
➣Treat iron overload with chelation therapy
■Minimize platelet transfusion to avoid alloimmunization. Transfu-
sion guidelines: platelets <10,000/mcl, clinically significant bleed-
ing, surgical procedure.
specific therapy
Indications
■Symptomatic cytopenias are a general indication for treatment of
MDS. Treatment options have improved recently, with FDA approval
of methyltransferase inhibitors (5-Azadytidine, and anti-angiogenic
agents (lenalidomide, Revlimid, Celgene).
➣Acute infection with neutropenia: Consider granulocyte colony-
stimulating factor (G-CSF,Neupogen)
➣Symptomatic anemia: 15–30% of MDS patients with erythro-
poietin (Epo) level <2,500 mU/mL will respond to erythropoi-
etin (Procrit) therapy. For patients refractory to monotherapy,
dose escalation and/or c-administration of G-CSF may further
increase response rate. Most recently, darbepoietin-alpha (DA,
Aransep), a hypersialylated erythropoietic stimulating protein
with a prolonged half-life, has shown erythroid responses com-
parable to rhuEP, with less frequent dosing, although optimal
dosing and schedule of DA for MDS have not yet been estab-
lished.