Effect of anti-CD20 (rituximab) on resistant thrombocytopenia in autoimmune lymphoproliferative syndrome.
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.
Department of Immunology, Royal Free Hospital, Hampstead, London, UK. Bridget.Heelan@rfh.nthames.nhs.uk
Bridget T
BT
Heelan
Vincent
V
Tormey
Peter
P
Amlot
Elspeth
E
Payne
Atul
A
Mehta
A David B
AD
Webster
D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19 / Q000097:blood; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D002648:Child; D003131:Combined Modality Therapy; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008232:Lymphoproliferative Disorders / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D008297:Male; D009154:Mutation; D010976:Platelet Count; D011506:Proteins / Q000235:genetics; D000069283:Rituximab; D048029:TNF Receptor-Associated Factor 6; D013921:Thrombocytopenia / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D014750:Vincristine / Q000627:therapeutic use
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19; D000970:Antineoplastic Agents; D000972:Antineoplastic Agents, Phytogenic; D007074:Immunoglobulin G; D011506:Proteins; D048029:TNF Receptor-Associated Factor 6; D000069283:Rituximab; D014750:Vincristine