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Arch Dis Child 1997;77:245-248 ( September )

The spectrum of Evans' syndrome

Süreyya Savaan, Indira Warrier, Yaddanapudi Ravindranath

Division of Hematology/Oncology, Children's Hospital of Michigan, Wayne State University, School of Medicine, Detroit, Michigan, USA

Correspondence to: and reprint requests to: Dr I Warrier, Children's Hospital of Michigan, Division of Hematology/Oncology, 3901 Beaubien Boulevard, Detroit, MI 48201, USA.

Accepted 17 June 1997

Eleven patients (10 boys, one girl) with Evans' syndrome with a median follow up time of 8.0 years were evaluated retrospectively. Six patients had either persistent hepatosplenomegaly or generalised lymphadenopathy, or both. In five patients, an increase in lymph node and/or spleen size was observed during the exacerbations of cytopenias. Seven patients had quantitative serum immunoglobulin abnormalities at the time of presentation. There were associated systemic manifestations in nine patients. Various forms of treatment were used with mixed results. Four patients died from sepsis and haemorrhage; four had complete recoverytwo after splenectomy. These findings show that Evans' syndrome is a heterogeneous disorder with significant morbidity and mortality. High incidence of quantitative serum immunoglobulin abnormalities, lymphoid hyperplasia, and associated systemic manifestations suggest that Evans' syndrome may represent a stage of a more broad spectrum, generalised immune dysregulation.

Keywords: Evans' syndrome; lymphoproliferation; serum immunoglobulins; immune dysregulation

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© 1997 by Archives of Disease in Childhood

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