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| The spectrum of Evans' syndrome |
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| Author(s): Savasan S, Warrier I, Ravindranath Y |
| Source: ARCHIVES OF DISEASE IN CHILDHOOD Volume: 77 Issue: 3 Pages: 245-248 Published: SEP 1997 |
| Times Cited: 43 References: 33 |
| 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. |
| Document Type: Article |
| Language: English |
Addresses:
1. WAYNE STATE UNIV, CHILDRENS HOSP MICHIGAN, SCH MED, DIV HEMATOL ONCOL, DETROIT, MI 48201 USA |
| Publisher: BRITISH MED JOURNAL PUBL GROUP, BRITISH MED ASSOC HOUSE, TAVISTOCK SQUARE, LONDON, ENGLAND WC1H 9JR |
| Subject Category: Pediatrics |
| IDS Number: XX932 |
| ISSN: 0003-9888 |
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