Postępy w diagnostyce i leczeniu zespołów mielodysplastycznych
© Borgis - Postępy Nauk Medycznych 4/2000, s. 40-43
Kazimierz Kuliczkowski, Maria Podolak-Dawidziak, Donata Urbaniak-Kujda
Summary
The myelodysplastic syndromes (MDS) are heterogenous group of clonal stem cell disorders characterized by bone marrow failure, abnormal haemopoiesis, and proliferation of myeloid blast cells. MDS is typically characterized by pancytopenias, in the setting of bone marrow hypercellularity with evidence of dysplasia of three lineages. High level of apoptosis, a premature programmed cell death has been demonstrated in dysplastic cells.
The FAB classification used since 1982 divided MDS into five subsets: refractory anemia, refractory anemia with ring sideroblasts, refractory anemia with excess of blasts, refractory anemia with excess of blasts in transformation, and chronic myelomonocytic leukaemia.
A new international scoring system for predicting outcome in myelodysplasia patients focuses on: age, marrow blast percentages, degree of cytopenia, and genetic abnormalities. Therapy of MDS is complex and difficult. Different drugs are used to encourage differentiation of malignant cell lines and thereby inhibit both the proliferation of blasts and the supression of abnormal cell maturation, and diminish apoptosis.
The role of bone marrow transplant in MDS is not yet wholly estimated.
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