9/22/2009. Treating Low-Risk Myelodysplasia. When myelodysplasia may be suspected. Common initial evaluations

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1 Treating Low-Risk Myelodysplasia Michael H. Rosove, M.D. Clinical Professor of Medicine UCLA Division of Hematology-Oncology When myelodysplasia may be suspected Anemia (low hemoglobin) normal gm/dl Macrocytosis (large RBCs) normal MCV fl Thrombocytopenia (low platelet count) normal 140,000 to 400,000/µL Thrombocytosis (high platelet count) Leukopenia (neutropenia) normal 4,000-10,000/µL (about 1,800-6,000/µL) Leukocytosis (neutrophilia) evaluate for immature neutrophils or myeloblasts Blood cell morphologic abnormalities on blood smear Common initial evaluations CBC (and comparison to prior CBCs) Review of the blood smear Blood tests to rule out iron, folic acid, vitamin B12 deficiencies, and other causes of anemia Serum erythropoietin level Bone marrow aspirate and biopsy (assessment of cellularity, dysmorphology, % myeloblasts, and chromosome/fish abnormalities) Clinical assessment especially fatigue and other symptoms referable to anemia; also bruising and bleeding, infections 1

2 Myelodysplasia Question: Which is/are principal determinant(s) of prognosis in the myelodysplastic syndromes? 1. Number of cytopenias 2. Percent myeloblasts in the bone marrow 3. Results of chromosome/fish analysis 4. Age 5. All are correct 6. 2 and 3 only are correct Myelodysplasia IPSS Points # of cytopenias* 0/1 2/3 BM myeloblast % < Karyotype** Good Int Poor * Hgb <10 (normal 12-16), ANC <1,800 (1,800-6,000), platelets <100,000 (140, ,000) ** Good normal, (del)y, 5q-, 20q- Poor chromosome 7, or complex (3 or more) abnormalities Intermediate all others Myelodysplasia IPSS and prognosis Median survival (yrs) by age Points Risk group <60 >60 <70 >70 0 Low Int Int >2.5 High <0.5 <0.5 <0.5 <0.5 2

3 Treatment options in low-risk myelodysplasia For stable patients with no symptoms observation alone For patients with symtomatic anemia erythropoietic stimulating agents (ESAs, Procrit, Epogen, Aranesp) (EPO level >200 not likely to respond well; treatment not appropriate if level >500); G-CSF (Neupogen) may improve ESA response For patients with 5q- myelodysplasia Revlimid (lenalidomide) For patients with markedly symptomatic anemia red blood cell transfusion(s) Treatment options in higher risk myelodysplasia Hypomethylating agents decitabine and azacytidine Experimental drug therapies Allogeneic bone marrow transplantation 70 y/o lady, increasingly fatigued over half a year, cannot walk 50 feet level without resting, no other medical problems, on exam quite pale. WBC 5,400, normal distribution ib ti of WBCs, Hgb 5.8, MCV 105, plat.ct. 530,000, iron, folic acid, vitamin B12 normal, serum EPO level 350 (normal 5-25). 3

4 Red cell transfusion is given, and a bone marrow exam is performed: Mild hypercellularity (50%), mild trilineage dysplastic changes, myeloblasts 2%, chromosome and FISH show 5q-. IPSS score: 0 (low risk). Points # of cytopenias* 0/1 2/3 BM myeloblast % < Karyotype** Good Int Poor * Hgb <10 (normal 12-16), ANC <1,800 (1,800-6,000), platelets <100,000 (140, ,000) ** Good normal, (del)y, 5q-, 20q- Poor chromosome 7, or complex (3 or more) abnormalities Intermediate all others For stable patients with no symptoms observation alone For patients with symptomatic anemia erythropoietic stimulating agents (ESAs, Procrit, Epogen, Aranesp); (EPO level l >200 not likely l to respond well; treatment not appropriate if level >500); G-CSF (Neupogen) may improve ESA response For patients with 5q- myelodysplasia Revlimid (lenalidomide) For patients with markedly symptomatic anemia red blood cell transfusion(s) 4

5 Question: Which is the single best treatment choice for this patient with 5q- myelodysplasia, IPSS score of 0 (low risk), and EPO level 350? 1. Erythropoietic stimulation, with red cell transfusional support as needed 2. Lenalidomide (Revlimid) 3. Hypomethylating agent azacytidine (Vidaza) or decitabine (Dacogen) 4. Allogeneic stem cell transplantation Case #2 A 60 y/o man sees his primary care physician for his annual check-up and is feeling fine. WBC 3,800, ANC 2,000, Hgb 11.0, MCV 96, platelets 180,000. One year prior, WBC 4,200, ANC 2,200, Hgb 12.2, MCV 95, platelets 200,000. A hematologist rules out other common causes of anemia. A bone marrow shows normal cellularity, mild dysplastic changes, myeloblasts <5%, chromosome/fish analyses normal. The hematopathologist declares this compatible with myelodysplasia. Question: How should he be managed? Case #3 A 75 y/o man sees his primary care physician for fatigue. He has noticed reduced activities over the past year. WBC 3,800, ANC 2,000, Hgb 8.7, MCV 110, platelets 120,000. One year prior, WBC 4,200, ANC 2,200, Hgb 11.7, MCV 95, platelets 200,000. Other common causes of anemia ruled out. Serum EPO 56. Bone marrow shows hypercellularity (70%), moderate dysplastic changes, myeloblasts <5%, chromosome/fish analyses 20q-. Question: How should he be managed? 5

Understanding Myelodysplastic Syndromes: A Patient Handbook

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