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Blood, Vol. 93 No. 2 (January 15), 1999:
pp. 459-466
Myelodysplastic and Myeloproliferative Disorders of Childhood: A
Study of 167 Patients
Sandra Luna-Fineman,
Kevin M. Shannon,
Susan K. Atwater,
Jeffrey Davis,
Margaret Masterson,
Jorge Ortega,
Jean Sanders,
Peter Steinherz,
Vivian Weinberg, and
Beverly J. Lange
From the University of California, San Francisco, San Francisco, CA;
the British Columbia Children's Hospital, Vancouver, British Columbia,
Canada; the Children's Hospital Medical Center of Cincinnati,
Cincinnati, OH; the Children's Hospital of Los Angeles, Los Angeles,
CA; the Fred Hutchinson Cancer Research Center, Seattle, WA; the
Memorial Sloan-Kettering Cancer Center, New York, NY; and the
University of Pennsylvania and Children's Hospital of Philadelphia,
Philadelphia, PA.
Myelodysplastic syndromes (MDS) and myeloproliferative syndromes
(MPS) of childhood are a heterogeneous group of clonal disorders of
hematopoiesis with overlapping clinical features and inconsistent nomenclature. Although a number of genetic conditions have been associated with MDS and MPS, the overall contribution of inherited predispositions is uncertain. We report a retrospective study examining
clinical features, genetic associations, and outcomes in 167 children
with MDS and MPS. Of these patients, 48 had an associated
constitutional disorder. One hundred one patients had adult-type
myelodysplastic syndrome (A-MDS), 60 had juvenile myelomonocytic leukemia (JMML), and 6 infants with Down syndrome had a transient myeloproliferative syndrome (TMS). JMML was characterized by young age
at onset and prominent hepatosplenomegaly, whereas patients with A-MDS
were older and had little or no organomegaly. The most common
cytogenetic abnormalities were monosomy 7 or del(7q) (53 cases); this
was common both in patients with JMML and those with A-MDS. Leukemic
transformation was observed in 32% of patients, usually within 2 years
of diagnosis. Survival was 25% at 16 years. Favorable prognostic
features at diagnosis included age less than 2 years and a hemoglobin F
level of less than 10%. Older patients tended to present with an
adult-type MDS that is accommodated within the French-American-British
system. In contrast, infants and young children typically developed
unique disorders with overlapping features of MDS and MPS. Although the
type and intensity of therapy varied markedly in this study, the
overall outcome was poor except in patients with TMS.
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