title: Successful treatment of B cell chronic lymphocytic leukemia-associated severe paraneoplastic pemphigus with cyclosporin A.
abstract: Since the first description of paraneoplastic pemphigus, several cases have been described in the literature. However, curative therapy is usually a challenge to the physicians treating this disease. Several publications are available discussing the efficacy of steroids, cyclophosphamide and cyclosporin A. Recently, a report of the successful use of rituximab was also published. However, the use of cyclosporin A is controversial in the case of B cell malignancies, as there are reports showing the cytotoxic effect of this drug on B cells. However, other authors report no effect, or even unwanted effects resulting in B cell proliferation. We report the case of a 50-year-old Caucasian male. He developed a B cell lymphoma consisting of CD5/CD20-double-positive cells, and 2 months later, it was followed by a very severe paraneoplastic pemphigus affecting the mucosa and the skin. The lymphoma was well managed with CHOP and CVP polychemotherapy, followed by oral chlorambucil; however, the bullous eruptions did not disappear. Oral steroids, cyclophosphamide, plasmapheresis and IVIG therapy were only partially successful, so we decided to use oral cyclosporin A. Starting with 7 mg/kg and maintaining a steady plasma level of no less then 110 ng/l, the bullae completely disappeared within 6 weeks, and the patient has been in remission for 17 months now, taking the oral cyclosporin A continuously. The underlying B cell disorder did not relapse during the therapy.
authors.affiliation: 3rd Department of Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary. gergely@iiibel.dote.hu
authors.forename: Lajos
authors.initials: L
authors.lastname: Gergely
authors.forename: László
authors.initials: L
authors.lastname: Váróczy
authors.forename: Györgyi
authors.initials: G
authors.lastname: Vadász
authors.forename: Eva
authors.initials: E
authors.lastname: Remenyik
authors.forename: Arpád
authors.initials: A
authors.lastname: Illés
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D002699:Chlorambucil / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D016572:Cyclosporine / Q000627:therapeutic use*; D004317:Doxorubicin / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000188:drug therapy; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000188:drug therapy* / Q000209:etiology / Q000628:therapy; D010392:Pemphigus / Q000188:drug therapy* / Q000209:etiology / Q000628:therapy; D010951:Plasma Exchange; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D007166:Immunosuppressive Agents; D002699:Chlorambucil; D014750:Vincristine; D004317:Doxorubicin; D016572:Cyclosporine; D003520:Cyclophosphamide; D011241:Prednisone; D008775:Methylprednisolone