Background The Canale–Smith syndrome is a childhood disordercharacterized by lymphadenopathy and autoimmunity. The similaritybetween this syndrome and that in mice with the lymphoproliferation(lpr ) phenotype or the generalized-lymphoproliferative-disease(gld) phenotype led us to investigate whether it too is causedby mutations of the Fas gene (lpr mice) or the Fas ligand (gldmice), which regulate apoptosis in lymphocytes.
Methods We studied four patients with the syndrome and theirfamilies. T-lymphocyte phenotypes were analyzed, and the susceptibilityof activated T cells to Fas-mediated apoptosis in vitro wasdetermined. Mutations of Fas were sought by nucleotide-sequenceanalysis.
Results Patients with the Canale–Smith syndrome had increasednumbers of circulating double-negative T cells (>20 percent)and profoundly impaired apoptosis of activated T cells incubatedwith an anti-Fas antibody. Three novel Fas mutations were identified,all of which were heterozygous and predicted to impair signaltransduction by Fas. Autoimmune manifestations of the disease,such as hemolytic anemia and thrombocytopenia, persisted intoadolescence. Two patients followed into adulthood had intermittentlymphadenopathy, which diminished over time. Neoplasms developedin both, and one died of hepatocellular carcinoma at the ageof 43.
Conclusions Patients with the Canale–Smith syndrome havemutations in Fas — a fact that implicates this gene inthe accumulation of lymphocytes and the autoimmunity characteristicof the syndrome.
Source Information
From the Division of Rheumatology, Hospital for Special Surgery, Cornell University Medical Center, New York (J.D., A.K.V., J.-L.C., K.B.E.), and the Division of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia (K.E.S.).
Address reprint requests to Dr. Elkon at the Hospital for Special Surgery, 535 E. 70th St., New York, NY 10021.
The Canale–Smith Syndrome
Straus S. E., Lenardo M., Puck J. M., Vaishnaw A. K., Sullivan K. E., Elkon K. B.
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N Engl J Med 1997;
336:1457-1458, May 15, 1997.
Correspondence
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