Case & Review
Management of multifocal primary cutaneous CD30+ anaplastic large cell lymphoma

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Abstract

Primary cutaneous CD30+ anaplastic large cell lymphoma in adults is rare, but the prognosis is generally excellent. Skin lesions may be localized or, less commonly, multifocal. Although not extensively reported, multifocal primary cutaneous anaplastic large cell lymphoma tends to relapse after systemic chemotherapy and is generally considered more prone to progress to extracutaneous involvement than the localized disease. We report the case of a 21-year-old woman with primary cutaneous CD30+ anaplastic large cell lymphoma manifesting as widespread papules and nodules. Despite remaining localized to the skin, the disease relapsed after multiple chemotherapy regimens and autologous stem-cell transplantation. Treatment with an experimental anti-CD30 monoclonal antibody was successful. Review of this case and similar cases illustrates that traditional combination chemotherapy may not be best. Newer treatments, including anti-CD30 monoclonal antibodies, show promise. However, further study is needed to develop optimal therapeutic strategies.

Section snippets

Case report

A 21-year-old woman presented with a 4-month history of erythematous papules that began on her elbows, subsequently enlarging to form nodules and involving her arms, legs, and trunk. Initial therapy included 3 courses of oral antibiotics prescribed by her primary care physician because the condition was presumed to be infectious. No improvement was noted, and the patient experienced an unintentional 5-kg weight loss along with night sweats.

Physical examination revealed multiple red-violet,

Background for literature review

Three main categories of CD30+ lymphoproliferative disorders of the skin are recognized: (1) primary cutaneous disorders (eg, ALCL, lymphomatoid papulosis); (2) CD30+ lymphoproliferative disorders developing with pre-existing mycosis fungoides–type cutaneous T-cell lymphoma or lymphomatoid papulosis; and (3) cutaneous involvement from a primary systemic ALCL.1., 11., 14., 15. This case is a primary cutaneous CD30+ lymphoproliferative disorder of the ALCL type. The patient had multifocal skin

Patients and methods

As part of this review, we searched the MEDLINE database (1966-present) for reports of representative cases of multifocal primary cutaneous CD30+ ALCL. Basic exclusion criteria included cases for patients younger than 18 years, cases with certain pre-existing disorders (mycosis fungoides–type cutaneous T-cell lymphoma, lymphomatoid papulosis, and other lymphoproliferative disorders), and cases with extracutaneous disease at presentation. Before 1985, ALCL cases were typically described as

Clinical features

The clinical characteristics of this group of adult patients with multifocal primary cutaneous CD30+ ALCL are somewhat heterogeneous. A male predominance is notable, with only 4 of the cases occurring in women (male-female ratio, approximately 3:1). Age at disease onset ranged from 20 to 88 years (mean: 52 years). Within this range, the ages at onset were distributed relatively uniformly by decade. The most frequently involved regions were the trunk and extremities, with the head and neck

Discussion

Therapy for solitary or localized disease is well delineated, in contrast to therapy for multifocal primary cutaneous ALCL. Some authors have recommended an initial 4- to 8-week period of observation for all patients with primary cutaneous CD30+ lymphomas because spontaneous regression is possible.14., 24. Furthermore, radiotherapy and simple excision, used individually or in combination, are accepted as the treatments of choice for solitary or localized disease.1., 12., 14.

Until recently,

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    Funding sources: None.

    Conflicts of interest: None identified.

    Presented at the Gross and Microscopic Dermatology Symposium of the American Academy of Dermatology 61st Annual Meeting, San Francisco, Calif, March 21-26, 2003.

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