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Transient myeloproliferative disorders and acute myelogenous leukemia in infants with Down's syndrome

Lookup NU author(s): Professor Hermann Josef Vormoor


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Transient neonatal myeloproliferative disorders (TMD's) indistinguishable from acute leukaemia by clinical and morphological criteria have been described in neonates with Down's syndrome. To analyse its clinical significance, 10 infants under 1 year of age presenting with Down's syndrome and the morphological picture of acute myelogenous leukaemia were reviewed. 3 of these children had true AML leading to death after 2, 8 and 11 months. In the other 7 children the diagnosis TMD was suggested as spontaneous or in one case interferon-induced remission occurred within 4 to 25 weeks after diagnosis. The interferon-treated patient died of SIDS at the age of 11 months. Another one of the TMD children developed fatal erythroleukaemia at the age of 2 years. Regarding initial clinical and haematological parameters, TMD was indistinguishable from true congenital leukaemie. In all patients classification according to the FAB criteria was difficult, as mainly undifferentiated or poorly differentiated myeloid blasts were seen, sometimes with erythro- or megakaryocytic features. Because of the difficulties in the differential diagnosis of TMD and true AML it is recommended to delay specific cytostatic therapy in neonates with Down's syndrome, until definite progression of the leukaemic process is observed or cytogenetic analyses suggesting true AML are available.

Publication metadata

Author(s): Creutzig U, Ritter J, Vormoor J, Eschenbach C, Dickerhoff R, Burdach S, Scheel-Walter HG, Kühl J, Schellong G

Publication type: Article

Publication status: Published

Journal: Klinische Pädiatrie

Year: 1990

Volume: 202

Issue: 4

Pages: 253-257

Print publication date: 01/07/1990

ISSN (print): 0300-8630

ISSN (electronic): 1439-3824


DOI: 10.1055/s-2007-1025529

Notes: 0300-8630 (Print) Journal Article


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