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Lymphoproliferative Disorders in Children with Primary Immunodeficiencies: Immunological Status May Be More Predictive of the Outcome Than Other Criteria by D Canioni; N Jabado; E MacIntyre; N Patey; J F Emile; N Brousse is a Medicine article available to read on EtoBox.

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## Aims: Lymphoproliferative disorders (LPDs) are a severe complication in primary immunodeficiency and post‐transplant patients. In primary immunodeficiency patients, LPDs are not well‐known and, thus, we tried to evaluate their distinctive features and to determine prognostic factors predictive of clinical outcome by comparison with LPDs in post‐transplant children. ## Methods and results: Clinical records and histopathology of 18 LPDs occurring in primary immunodeficieny children were compared with those of 10 LPDs in post‐transplant children, together with results of in‐situ hybridization for the detection of Epstein–Barr virus (EBV)‐RNA and molecular biological techniques. LPDs were frequently extranodal, EBV‐associated, and were more commonly pleomorphic in primary immunodeficiency than in post‐transplant patients. A low T‐cell count and abnormal T‐cell function indicated bad prognosis in both groups. Polymorphic LPDs (PLPDs) were most frequent (__n__ = 19), whereas lymphomas were rare (__n__ = 7), and pseudo‐tumoral lymphoid hyperplasias (__n__ = 2) were observed only in primary immunodeficiency. Comparative p53/bcl‐2 staining revealed a p53 overexpression in lymphomas compa

Who reads Lymphoproliferative Disorders in Children with Primary Immunodeficiencies: Immunological Status May Be More Predictive of the Outcome Than Other Criteria?

Author

D Canioni; N Jabado; E MacIntyre; N Patey; J F Emile; N Brousse

Publisher

John Wiley and Sons; Wiley (Blackwell Publishing); Blackwell Publishing Inc.; Wiley (ISSN 0309-0167)

Published

2001

Language

EN

Field

Medicine (Health Sciences)