Myelodysplastic Syndrome

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Alvin Houston
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1 Myelodysplastic Syndrome Marsha N. Rehm, MSN, RN-BC Oncology Clinical Nurse Specialist Vidant Medical Center Greenville, North Carolina Objectives Describe the etiology of Myelodysplastic Syndrome (MDS) Identify treatment and management strategies MDS is A type of cancer, in which the bone marrow does not make enough health cells. Characterized by immature cells in the bone marrow 1

$bleeding Abnormal Cells Blast cells crowd out healthy/mature cells leading to: Refractory Anemia - RBC, normal WBC and Plts Refractory Anemia with ring sideroblasts - Same as above with excess iron$

2 Normal Bone Marrow Normal Cell Function Red Blood Cells (RBC)-Carry oxygen and other substances to tissues White Blood Cells (WBC)-Fight infection and diseases Platelets (Plts)-Form clots to stop bleeding Abnormal Cells Blast cells crowd out healthy/mature cells leading to: Refractory Anemia - RBC, normal WBC and Plts Refractory Anemia with ring sideroblasts - Same as above with excess iron 2

3 Abnormal Cells Refractory anemia with excess blasts - RBCs, 5-19% blasts in marrow, can progress to Acute Myeloid Leukemia (AML) Refractory Cytopenia with multilineage dysplasia - in one of the 3 types of marrow cells - <5% blasts in marrow, <1% in blood: RBCs may or may not have extra iron-can progress to AML Abnormal Cells Refractory cytopenia with unilineage dysplasia - in one type of cell. Changes in > 10% in others (still < 5% blasts in marrow and 1% in blood) Unclassified myelodysplastic syndrome -marrow is normal and doesn t fit another category MDS associated with an isolated del(5q)chromosome abnormality - RBCs, <5% marrow, 1% blood with chromosomal change Chronic Myelomonocytic Leukemia (CMML) Increases with age White males Incidence of MDS >10,000 new cases of MDS occur in the United States annually 60,000 individuals with MDS currently reside in the country. Am J Med Jul;125(7 Suppl):S2-5. doi: /j.amjmed

Risk Factors Past treatment with chemotherapy or radiation therapy Exposure to chemicals Tobacco smoke Pesticides Fertilizers Solvents (Benzene) Exhaust gases Exposure to heavy metals Mercury Lead

4 Risk Factors Past treatment with chemotherapy or radiation therapy Exposure to chemicals Tobacco smoke Pesticides Fertilizers Solvents (Benzene) Exhaust gases Exposure to heavy metals Mercury Lead Signs and Symptoms Shortness of breath Weakness and fatigue Pallor Easy bruising or bleeding Petechiae Testing and Diagnosis Physical exam and history CBC with diff Cytogenics BMP Bone marrow aspiration and biopsy Immuno-cytochemistry Immuno-phenotyping Flow cytometry FISH (fluorescence in situ hybridization) 4

5 Prognosis and Treatment (Revised International Prognostic Scoring System-IPSS-R) Depends on: Number of blasts in the bone marrow Whether one or more types of cells are affected Presenting symptoms (anemia, bleeding, infection) Risk of leukemia Changes in chromosomes Whether post chemo/radiation related Age and general health of the patient R-IPSS Cytogenic Risk Groups Cytogenetic prognostic subgroups Very good Good Intermediate Poor Very poor Cytogenetic abnormalities -Y, del(11q) Normal, del(5q), del(12p), del(20q), double including del(5q) del(7q), +8, +19, i(17q), any other single or double independent clones -7, inv(3)/t(3q)/del(3q), double including -7/del(7q), Complex: 3 abnormalities Complex: >3 abnormalities IPSS-R Prognostic Score Values Prognostic variable Cytogenetics Very Good Good Intermediate Poor Very Poor BM Blast % <=2 >2-<5% 5-10% >10% Hemoglobin =>10 8-<10 <8 Platelets => <100 <50 ANC =>0.8 =>0.8 5

6 IPSS-R Prognostic Risk Categories/Scores RISK CATEGORY RISK SCORE Very Low <=1.5 Low >1.5-3 Intermediate >3-4.5 High >4.5-6 Very High >6 IPSS-R: Prognostic Risk Category Clinical Outcomes* No. pts Very Low Low Intermediate High Very High Patients (%) Survival* ** % 38% 20% 13% 10% AML/25 %***,^ NR ***Medians, years ^Median time to 25% AML evolution *Greenberg, Tuechler, Schanz et al, Revised International Prognostic Scoring System (IPSS-R) for Myelodysplastic Syndrome, Blood 120: 2454, **Schanz J et al, J Clin Oncology 2012; 30:820 Supportive Care Drug Therapy Treatment Options Stem Cell Transplant 6

7 Supportive Care Transfusion therapy Erythropoiesis stimulation Antibiotic therapy Transfusion Therapy RBC s (CMV neg and leukoreduced)- Maintain Hgb > 8 or symptomatic Platelets Maintain Plt count > 10,000 or active/potential bleeding G-CSF-for recurrent or resistant infections Erythropoiesis-Stimulating Agents Given to increase number of mature RBCs Nursing care - Monitor for site reactions, monitor lab values 7

8 Drug Therapy Lenalidomide for del(5q) chromosome abnormality, lessens need for RBC transfusions Immunosuppressive therapy- Antithymocyte globulin/cyclosporine Azacitidine and decitabine Antibiotic Therapy Febrile neutropenia Considered a medical emergency Broad-spectrum antibiotics Chemotherapy Similar treatment for AML High dose Cytarabine Daunorubicin Fludarabine Topotecan Patient education for side effect management 8

9 Stem Cell Transplant Can consider if remission is achieved Stem cells are harvested High dose chemotherapy is used Stem Cells are infused Supportive care until counts recover Role of the Infusion Nurse Monitor and maintain access devices Patient and family education Monitor vital signs Monitor lab values especially pre and post transfusions Monitor transfusion reactions Monitor for fluid overload Ensure infection control practices Monitor pan-culture results Questions?? 9

Acute Myeloid Leukemia

Acute Myeloid Leukemia Introduction Leukemia is cancer of the white blood cells. The increased number of these cells leads to overcrowding of healthy blood cells. As a result, the healthy cells are not