title: Treatment of refractory thrombotic thrombocytopenic purpura using multimodality therapy including splenectomy and cyclosporine.
abstract: ADAMTS13 mediated thrombotic thrombocytopenic purpura (TTP) is an immunological disease that is very difficult to treat. Plasma exchange, with plasma replacement and steroids have been the first line of treatment for this condition. Ten to 20% of the patients either have no response or a partial response to the treatment. Refractory TTP has been treated in few case reports with anti-CD20 agents, intravenous gamma globulin, vincristine and splenectomy. We report two cases of refractory TTP that responded to multimodality immunosuppressive therapy that included splenectomy, intravenous gamma globulin, and cyclosporine after numerous plasma exchange treatments, steroids, rituximab and vincristine had failed to induce remission. Combining drugs that target T and B lymphocytes is a standard in organ transplantation and deserves more consideration in the treatment of severe and refractory autoimmune diseases such as TTP.
authors.affiliation: Department of Medicine, New York Presbyterian Hospital, Weill Cornell Medical Center, New York, NY 10065, USA. kdj200@gmail.com
authors.forename: Kenar D
authors.initials: KD
authors.lastname: Jhaveri
authors.forename: Abby
authors.initials: A
authors.lastname: Scheuer
authors.forename: Jules
authors.initials: J
authors.lastname: Cohen
authors.forename: Bruce
authors.initials: B
authors.lastname: Gordon
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000328:Adult; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008776:Methylprednisolone Hemisuccinate / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy* / Q000601:surgery*; D013156:Splenectomy; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D008776:Methylprednisolone Hemisuccinate; D016572:Cyclosporine; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human