The spectrum of Evans' syndrome

Süreyya Savaşan, Indira Warrier, Yaddanapudi Ravindranath

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98 Scopus citations

Abstract

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 recovery-two 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.

Original languageEnglish
Pages (from-to)245-248
Number of pages4
JournalArchives of Disease in Childhood
Volume77
Issue number3
DOIs
StatePublished - 1997

Keywords

  • Evans' syndrome
  • Immune dysregulation
  • Lymphoproliferation
  • Serum immunoglobulins

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