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What is the cause and treatment for myelodysplastic syndrome?

Q: My wife has MDS RA from the past 2 and half years. Her latest counts Hb - 7.0, WBC – 1000 and platelets - 36,000. She is under hospital care for fever and throat infection. She does no have any other disorder. Her menstrual bleeding exceeds to more days. Primolut course is suggested. Epo + G-CSF protocol started 4 weeks ago. Four Wepox were given, 14 Grafeel have been given till now and is given daily in the hospital. Her reports say Creatinine, blood culture, karyotype and B12 levels are normal. No malignancy in BM study before 2 years. Two per blast and iron negative in BM. The WBC has lowered to 900, which G-CSF will help? Doctor suggests Grafeel daily. What is the treatment of MDS? What is the prognosis and how can routine life be established?

A:Myelodysplastic syndromes (MDS) are a varied group of hematologic disorders arising from a problem in the stem cells. They are characterised by ineffective blood cell production resulting in varying degrees of anaemia (low haemoglobin), leukopaenia (reduced white cell count) and thrombocytopaenia (low platelets). The bone marrow and peripheral blood examination shows cells with abnormal features. The postulated causes include prior chemotherapy, exposure to radiation or chemicals, viral infection or genetic predisposition. The condition is considered to be a pre-malignant one as some forms of MDS may transform to leukaemia. Refractory anaemia has a prolonged clinical course and a low prevalence of progression to acute leukaemia. Most treatment of MDS is not standard and is considered experimental and is constantly changing. It is typically tailored according to the individual’s age, general health, type of MDS, and symptoms. In the early phases of ineffective haematopoiesis, retinoids and haematopoietic growth factors are indicated. In late stages, with inevitable leukaemic transformation, cytotoxic chemotherapy and bone marrow transplantation may be necessary. All of these modes of therapy are undergoing clinical trials to determine the overall benefit to quality of life and survival. Supportive therapy like transfusions of the cells that are low (red cells or platelets), and treatment of infections form the cornerstone. Symptomatic therapy improves quality of life but these measures are temporary and more long-term measures are necessary to stimulate the patient's bone marrow. These include erythropoietin for anaemia and granulocyte colony-stimulating factor for neutropaenia. Transition to acute leukaemia is prevented by administration of the newly approved demethylating agent azacitidine. New promising drugs such as Revimid are completing clinical trials. The treatment being given to your wife is as per currently accepted protocols. Patients who have single or mild cytopaenias, normal chromosomes or a single chromosomal abnormality (except those involving chromosome 7) and less than 5% myeloblasts in the bone marrow have a better prognosis.


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