Follicular non-Hodgkin's lymphoma with refractory paraneoplastic pemphigus: Case report with review of novel treatment modalities

被引:32
作者
Van Rossum, MM
Verhaegen, NTM
Jonkman, MF
Mackenzie, MA
Koster, A
Van der Valk, PGM
Span, LFR
机构
[1] Univ Med Ctr Nijmegen, Dept Dermatol, NL-6500 HB Nijmegen, Netherlands
[2] Dept Haematol, Nijmegen, Netherlands
[3] Univ Groningen Hosp, Dept Dermatol, Groningen, Netherlands
关键词
paraneoplastic pempigus; non-Hodgkin's lymphoma; corticosteroids; intravenous immunoglobulin; plasma exchange; rituximab;
D O I
10.1080/10428190410001733781
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
In this paper a patient with a non-Hodgkin's lymphoma (NHL) and paraneoplastic pemphigus (PNP) is described. PNP is a very rare, painful mucocutaneous intraepithelial blistering disease associated with occult or confirmed malignancy. Patients with PNP show severe, progressive mucocutaneous disease with a high mortality rate, because of drug-induced infectious complications. The patients sometimes benefit from high doses of oral corticosteroids. However, pulse therapy with high doses of prednisolone (or dexamethasone) in combination with other immunosuppressants induces variable and inconstant results. Intravenous immunoglobulin (IVIg) has been applied in different cases of PNP with encouraging results. Plasmapheresis or plasma exchange (PE) in combination with corticosteroids and/or cyclophosphamide or azathioprine showed similar rapid and beneficial results in association with decreasing auto-antibody levels in this group of refractory pemphigus. Another interesting therapeutic option is rituximab, a chimeric monoclonal antibody directed against the CD20 antigen, which is found on the surface of normal and malignant B-lymphocytes. Administration of rituximab for patients with PNP in combination with follicular NHL is not always successful regarding oral lesions as we report in this case. PE leading to prompt depletion of autoreactive antibodies combined with immunosuppressants or synchronisation of PE with IVIg seems the best treatment modality for this refractory group, but the therapeutic value and appropriate timing of rituximab obviously deserve further evaluation in patients with low grade NHL and PNP.
引用
收藏
页码:2327 / 2332
页数:6
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