Myelodysplasia in childhood may be a polyclonal disease.
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.
Laboratoire d'Hématologie, Hôpital Bicêtre, Le Kremlin Bicêtre, France.
G
G
Tchernia
B
B
Bader-Meunier
J M
JM
Lavergne
F
F
Miélot
J P
JP
Dommergues
D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D009190:Myelodysplastic Syndromes / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology