ISSN:
1279-8509
Keywords:
Myelodysplastic syndromes
;
Clonality
;
Mitochondrial cytopathies
;
Malformative syndromes
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s00282-996-0325-0
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