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Primary Cutaneous Marginal Zone B-Cell Lymphoma
Clinical and Therapeutic Features in 50 Cases
J. J. Hoefnagel, MD;
M. H. Vermeer, MD, PhD;
P. M. Jansen, MD, PhD;
F. Heule, MD, PhD;
P. C. van Voorst Vader, MD, PhD;
C. J. G. Sanders, MD;
M. J. P. Gerritsen, MD, PhD;
M. L. Geerts, MD, PhD;
C. J. L. M. Meijer, MD, PhD;
E. M. Noordijk, MD, PhD;
R. Willemze, MD, PhD; for the Dutch Cutaneous Lymphoma Working Group
Arch Dermatol. 2005;141:1139-1145.
Background Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade B-cell lymphoma that originates in the skin, with no evidence of extracutaneous disease. Studies focusing on the optimal treatment of PCMZL have not been published thus far. We describe 50 patients with PCMZL to further characterize clinical characteristics and outcome and, in particular, to evaluate our current therapeutic approach.
Observations The majority of the patients (36/50 [72%]) presented with multifocal skin lesions, and 14 patients (28%) presented with solitary or localized lesions. The initial treatment of patients with solitary lesions consisted of radiotherapy or excision, whereas patients with multifocal lesions received a variety of initial treatments, most commonly radiotherapy and chlorambucil therapy. Cutaneous relapses developed in 19 (48%) of 40 patients who had complete remission and were more common in patients with multifocal disease. After a median period of follow-up of 36 months, 2 patients developed extracutaneous disease, but none of the patients died of lymphoma.
Conclusions Patients with PCMZL who have solitary lesions can be treated effectively with radiotherapy or excision. For patients with PCMZL who have multifocal lesions, chlorambucil therapy and radiotherapy are suitable therapeutic options. In case of cutaneous relapses, the beneficial effects of treatment should carefully be weighed against the potential adverse effects.
Author Affiliations: Departments of Dermatology (Drs Hoefnagel, Vermeer, and Willemze), Pathology (Dr Jansen), and Clinical Oncology (Dr Noordijk), Leiden University Medical Center, Leiden; the Departments of Dermatology, Erasmus Medical Center, Rotterdam (Dr Heule), University Hospital Groningen, Groningen (Dr van Voorst Vader), University Hospital Utrecht, Utrecht (Dr Sanders), Radboud University Nijmegen Medical Centre, Nijmegen (Dr Gerritsen), and University Hospital Gent, Gent (Dr Geerts); and the Department of Pathology, Vrije Universiteit Medisch Centrum, Amsterdam (Dr Meijer); the Netherlands.
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