Myelodysplasia in childhood may be a polyclonal disease |
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Authors: | G. Tchernia B. Bader-Meunier J. M. Lavergne F. Miélot J. P. Dommergues |
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Affiliation: | 1. Laboratoire d’Hématologie and Service de Pédiatrie Générale, H?pital Bicêtre, 78, rue du Général Leclerc, F-94275, Le Kremlin Bicêtre, France
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Abstract: | Myelodysplasia in childhood can be associated with constitutional abnormalities. Two main situations can be observed: constitutional diseases such as Down’s Syndrome may be the first step of a malignant stem cell transformation leading to monoclonal hematopoiesis. However, in other situations such as mitochondrial cytopathies or other polymalformative syndromes, myelodysplasia may simply be the hematological expression of a multi-tissue constitutional disease. In such cases, the bone marrow karyotype is usually found to be normal and, in affected females, clonality studies show a polyclonal pattern. Clonality assessment should be, when possible, a mandatory step before any major therapeutic decision during the course of childhood myelodysplasia. |
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