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Treatment of Multifocal Primary Cutaneous B-Cell Lymphoma: A Clinical Follow-Up Study of 29 Patients

From the Departments of Dermatology and Pathology, Free University Hospital, Amsterdam; Department of Radiation Oncology, Leiden University Medical Center, Leiden; Department of Dermatology, University of Rotterdam, Rotterdam; Department of Dermatology, University of Groningen, Groningen; Department of Dermatology, University of Utrecht, Utrecht, the Netherlands; and Department of Dermatology, University of Ghent, Ghent, Belgium.

Address reprint requests to Rein Willemze, MD, Department of Dermatology, Free University Hospital, De Boelelaan 1117, 1081 HV Amsterdam, the Netherlands; email r.willemze{at}azvu.nl

PURPOSE: Although patients with primary cutaneous B-cell lymphoma (CBCL) and localized skin lesions are generally treated with radiotherapy and have an excellent prognosis, the clinical behavior and optimal treatment of CBCL presenting with multifocal skin lesions are less well defined. In this study, we evaluated the clinical behavior of and results of treatment for multifocal CBCL in 29 patients, and we formulated therapeutic guidelines.

PATIENTS AND METHODS: The study group included 16 patients with primary cutaneous follicular center-cell lymphoma (PCFCCL), eight with primary cutaneous immunocytoma (PCI), and five with primary cutaneous large B-cell lymphoma presenting on the legs (PCLBCL of the leg).

RESULTS: Only one of the 24 patients with multifocal PCFCCL or PCI developed extracutaneous disease, and no patient died from lymphoma (median follow-up, 54 months). In patients with PCFCCL, treatment with either multiagent chemotherapy (nine patients) or radiotherapy directed toward all skin lesions (five patients) proved equally effective in terms of complete remission, relapse, and survival. In contrast, all five patients with PCLBCL of the leg developed extracutaneous disease, and four of the five died from systemic lymphoma, 8 to 36 months (median, 21 months) after diagnosis.

CONCLUSION: The results of these preliminary studies suggest that patients with PCFCCL or PCI presenting with multifocal skin lesions have the same excellent prognosis that patients with localized PCFCCL or PCI have and that radiotherapy directed toward all skin lesions is as effective as multiagent chemotherapy. Patients with PCLBCL of the leg have a more unfavorable prognosis, particularly patients presenting with multifocal skin lesions. This last group should always be treated with multiagent chemotherapy.

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