Successful treatment of B cell chronic lymphocytic leukemia-associated severe paraneoplastic pemphigus with cyclosporin A

L. Gergely, László Váróczy, Györgyi Vadász, E. Remenyik, A. Illés

Research output: Contribution to journalArticle

26 Citations (Scopus)

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 paraneo-plastic 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.

Original languageEnglish
Pages (from-to)202-205
Number of pages4
JournalActa Haematologica
Volume109
Issue number4
DOIs
Publication statusPublished - 2003

Fingerprint

Pemphigus
B-Cell Chronic Lymphocytic Leukemia
Cyclosporine
B-Lymphocytes
Cyclophosphamide
Steroids
Chlorambucil
Plasmapheresis
Intravenous Immunoglobulins
B-Cell Lymphoma
Therapeutics
Blister
Combination Drug Therapy
Plastics
Publications
Lymphoma
Mucous Membrane
Cell Proliferation
Physicians
Recurrence

Keywords

  • B cell chronic lymphocytic leukemia
  • Cyclosporin A
  • Lymphoma
  • Paraneoplastic pemphigus
  • Plasmapheresis

ASJC Scopus subject areas

  • Hematology

Cite this

Successful treatment of B cell chronic lymphocytic leukemia-associated severe paraneoplastic pemphigus with cyclosporin A. / Gergely, L.; Váróczy, László; Vadász, Györgyi; Remenyik, E.; Illés, A.

In: Acta Haematologica, Vol. 109, No. 4, 2003, p. 202-205.

Research output: Contribution to journalArticle

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