Childhood monosomy 7 syndrome: clinical and in vitro studies.

Abstract:

:The clinical and cell growth characteristics of 11 children with monosomy 7 presenting as preleukemia (eight cases) or acute nonlymphoblastic leukemia (three cases) were studied. Anemia was common to all patients, with nine showing leukocytosis, seven thrombocytopenia, and one thrombocytosis. There was a striking predominance of males (M/F ratio, 10:1) and a young median age (3 years). Preleukemia evolved to acute nonlymphoblastic leukemia in five patients and to myelofibrosis in one. In vitro studies of bone marrow progenitor cells cultured in leukocyte feeder-stimulated agar revealed abnormal cell proliferative patterns, most often an increased number of small clusters, for all 11 subjects. The cells of some preleukemic patients showed increased growth even in the absence of an exogenous source of colony-stimulating factor, suggesting autonomous growth or possibly autocrine stimulation. Combination chemotherapy or bone marrow transplantation failed to induce complete remission in the seven patients who were treated. Our findings in these 11 cases confirm the poor prognosis of monosomy 7 presenting as preleukemia in children. The in vitro studies suggest an association between altered cell growth in vitro and clinical evolution to frank leukemia.

journal_name

Leukemia

journal_title

Leukemia

authors

Weiss K,Stass S,Williams D,Kalwinsky D,Dahl GV,Wang W,Johnson FL,Murphy SB,Dow LW

subject

Has Abstract

pub_date

1987-02-01 00:00:00

pages

97-104

issue

2

eissn

0887-6924

issn

1476-5551

journal_volume

1

pub_type

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