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Case Reports
. 2002 Sep;118(4):1078-81.
doi: 10.1046/j.1365-2141.2002.03753.x.

Effect of anti-CD20 (rituximab) on resistant thrombocytopenia in autoimmune lymphoproliferative syndrome

Bridget T Heelan  1 , Vincent Tormey, Peter Amlot, Elspeth Payne, Atul Mehta, A David B Webster
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Case Reports

Effect of anti-CD20 (rituximab) on resistant thrombocytopenia in autoimmune lymphoproliferative syndrome

Bridget T Heelan et al. Br J Haematol. 2002 Sep.

Abstract

Fas (CD95) plays an important role in apoptosis. Patients with defects in Fas have an autoimmune lymphoproliferative syndrome (ALPS) characterized by lymphadenopathy, autoimmune cytopenias and an increased incidence of lymphomas. There are approximately 70 known cases described worldwide. The autoimmune cytopenias are difficult to treat in this group. We describe a patient with a defect in the death domain of the FAS molecule who had autoimmune thrombocytopenia resistant to conventional therapy but which responded to a combination of rituximab and vincristine.

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