Under myelodysplastic syndromes (MDS), a group of diseases is summarized, the (AML) are characterized by peripheral cytopenia, dysplastic hematopoietic progenitor cells, hypercellular bone marrow and an increased risk for the development of acute myeloid leukemia. The clinical symptoms depend on the most affected cell line and may include fatigue, weakness, pallor (due to anemia), increased susceptibility to infection and fever include (as a result of neutropenia) and tendency to bleeding and hematoma (as a result of thrombocytopenia). The diagnosis is prostate biopsy based on blood count, peripheral smears and bone marrow aspirate /. To treat azacytidine can be used. In the transition to AML treatment in accordance with the usual AML protocols should be.

Under myelodysplastic syndromes (MDS), a group of diseases is summarized, the (AML) are characterized by peripheral cytopenia, dysplastic hematopoietic progenitor cells, hypercellular bone marrow and an increased risk for the development of acute myeloid leukemia. The clinical symptoms depend on the most affected cell line and may include fatigue, weakness, pallor (due to anemia), increased susceptibility to infection and fever include (as a result of neutropenia) and tendency to bleeding and hematoma (as a result of thrombocytopenia). The diagnosis is prostate biopsy based on blood count, peripheral smears and bone marrow aspirate /. To treat azacytidine can be used. In the transition to AML treatment in accordance with the usual AML protocols should be. Pathophysiology MDS is a group of disorders, which are sometimes referred to as pre-leukemia, and result from a somatic mutation of hematopoietic precursors. MDS is similar to the Philadelphia chromosome-negative chronic myeloid leukemia, chronic myelomonocytic leukemia and myeloid metaplasia agnogenen, but is not identical with them. The etiology is often not known, however, the risk for the development of an MDS increases with exposure to benzene, ionizing radiation or chemotherapeutic agents (in particular for long-term or intensive treatment regimens, the alkylating or epipodophyllotoxins included). Characteristic of an MDS is the clonal proliferation of hematopoietic stem cells, which affects erythropoiesis granulocytopoese and megakaryocytopoiesis. The bone marrow is normotensive or hypercellular. As a result of ineffective hematopoiesis occurred on anemia (most common), neutropenia, thrombocytopenia, or a combination thereof. Because of the inappropriate cell production morphological changes of the cells show up in the bone marrow and peripheral blood. In case of extramedullary hematopoiesis can cause a hepatomegaly or splenomegaly. Occasionally there is a myelofibrosis already at diagnosis before or develops the MDS in the course. The classification is based on the findings in the blood and bone marrow (see table: Myelodysplastic syndrome: bone marrow findings and survival times). The unstable MDS clone tends to move into a AML. Myelodysplastic syndrome: bone marrow findings and survival classification criteria median survival (years) Refractory anemia anemia with reticulocytopenia normo- or hyper cellular bone marrow hyperplasia of erythropoiesis and dyserythropoiesis blasts ? 5% of NMC ? 5 Refractory anemia with sideroblasts findings as refractory anemia, but with ringed sideroblasts > 15% of NMC ? 5 Refractory anemia with excess blasts cytopenia in ? 2 cell lines with morphological changes of the blood cells hypercellular bone marrow with dyserythropoiesis and Dysgranulopoese blasts 5-20% of the NMC 1.5 Chronic myelomonocytic leukemia findings as refractory anemia with excess blasts and absolute monocytosis in blood Significant proliferation of Monozytenvorläuferzellen in 1.5 Refractory with excess blasts in transformation bone marrow anemia (Editor’s note: Please see the further developed and generally accepted classification according to wHO!) findings as refractory ary anemia with excess blasts, and with ? 1 of the following criteria: ? 5% blasts in the blood 20-30% blasts in the bone marrow Auer rods in the 0.5 Granulozytenvorläuferzellen NMC = nucleated bone marrow cells. Symptoms and signs The symptoms are an expression of the cell line most affected and can paleness, weakness, fatigue (anemia), fever and infection (neutropenia) and easy bruising, petechiae, nosebleeds, or mucosal bleeding (thrombocytopenia) include. Often, splenomegaly and hepatomegaly to occur. but existing symptoms may be attributable to other comorbidities. For example, there can be an increase in the pektanginösen complaints in elderly patients with pre-existing cardiovascular disease and anemia by the MDS. Diagnostic blood Peripheral blood smear bone marrow examination Suspicion of an MDS results, especially in the elderly, in the presence of refractory anemia, leukopenia or thrombocytopenia. Cytopenias as a result of congenital diseases, vitamin deficiency or medication side effects have to be excluded. To help diagnose the study of peripheral blood and bone marrow is necessary and morphological abnormalities must be detected in 10-20% of cells of the cell lines in question. Usually there is a macrocytic anemia with anisocytosis, which is indicated by the automatic cell counter as elevated MCV and broadening of the erythrocyte distribution. Frequently thrombocytopenia varying degrees occurs. In the peripheral blood smear, the platelets vary in size; some appear hypogranulär. The total white may be normal, increased or decreased. The cytoplasmic granulation of neutrophils is connected abnormally and with a anisocytosis and variable number of granules. Likewise abnormally granulated eosinophils or pseudo-Pelger Huet nuclear anomaly (hyposegmentierte neutrophils) can be found. The Monocytosis (> 1000 / uL) is characteristic of the subtype of chronic myelomonocytic leukemia. Immature myeloid cells may occur in less well-differentiated subtypes. Cytogenetic studies usually show pathological findings. Often have one or more clonal cytogenetic abnormalities often involving chromosomes 5 or 7. Forecast The prognosis depends to a large extent on the classification and present comorbidities from. Patients with refractory anemia or refractory anemia with ringed sideroblasts have a lower risk for progression to an aggressive form and eventually die of other causes. Therapy alleviating the symptoms Supportive treatment may stem cell azacytidine improves symptoms, reduces the rate of transformations in a leukemia and transfusion requirements, and probably survival. Other therapeutic strategies are purely supportive and include RBC and platelet transfusions and antibiotics for bacterial infections. The demethylating Desoxyazacytidin (decitabine) is sometimes effective even in patients who do not respond to azacitidine. In some patients, treatment with cytokines (erythropoietin in transfusion requirements, granulocyte colony-stimulating factor for severe symptomatic granulocytopenia and, if available, thrombopoietin for severe thrombocytopenia) represent an important support hematopoiesis, but they do not prolong life. Allogeneic stem cell transplantation is the treatment of choice in young patients. Currently, non-myeloablative allogeneic transplantation regimen for patients> 50 years are examined. The response of the MDS to chemotherapy (usually similar regimes, such as those used in AML) is comparable, taking into account age and karyotype with the AML. Summary A myelodysplastic syndrome is a disorder of hematopoiesis with clonal proliferation of abnormal precursor cell. Patients usually from a lack of red blood cells (is most common), white blood cells and / or platelets. The transformation to acute myeloid leukemia is common. Azacitidine may improve the symptoms and reduce the rate of transformation to acute leukemia. In young patients, stem cell transplantation is the treatment of choice.

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