Published in:
01-02-2004 | Case Report
The autoimmune lymphoproliferative syndrome (Canale–Smith) in adulthood
Published in: Clinical Rheumatology | Issue 1/2004
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The autoimmune lymphoproliferative syndrome (ALPS) or Canale–Smith syndrome is a recently described clinical entity consisting of chronic, non-malignant lymphadenopathy and hepatosplenomegaly together with hypergammaglobulinemia, positive autoantibodies and/or overt autoimmune diseases. It is caused by a genetic defect in the mechanism of programmed cell death (apoptosis) and is characterized by the presence of double-negative (TCR α/β CD4− CD8−) T lymphocytes (DNT). Although well known in pediatric patients, ALPS is an unusual diagnosis in adults. The oldest reported patient was aged 54. We describe another two adult patients in whom a presenting autoimmune disease led to the diagnosis of ALPS.