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Managing the CD30+lymphoproliferative diseases

Bagot, M.

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doi: 10.1097/01.cmr.0000382787.89923.d9
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Primary cutaneous CD30+lymphoproliferative diseases are the second most common type of cutaneous T-cell lymphoma (CTCL), representing approximately 30% of CTCLs. The spectrum of primary cutaneous CD30-positive lymphoproliferative disorders includes primary cutaneous anaplastic large cell lymphoma (C-ALCL), lymphomatoid papulosis (LyP), and borderline cases with overlapping clinical, histologic, and immunophenotypic features. The diagnosis is performed on a combination of clinical, histological and immunophenotypic criteria. LyP is characterized by recurrent, self-healing papulonecrotic or papulonodular skin kesions that may leave superficial scars. Three overlapping histologic subtypes of LyP (types A, B, and C) have been described. The prognosis is excellent. However, LyP is associated with development of malignant lymphoma (mycosis fungoides, Hodgkin lymphoma, or ALCL) in 20% of cases. No extensive staging is needed. Patients with relatively few lesions can be clinically followed without treatment. Topical chemotherapy, PUVA or low-dose oral methotrexate can be advised in patients with extensive lesions. C-ALCL is characterized by solitary or localized nodules or tumors, that can be ulcerated and that may regress spontaneously. Multifocal lesions are also possible. Histological analysis shows a diffuse infiltrate composed of large cells with an anaplastic, pleomorphic, or immunoblastic cytomorphology with cohesive sheets and expression of the CD30 antigen by more than 75% of tumor cells. The (2;5) (p23;q35) translocation, which is often found in systemic ALCL, is not found in C-ALCL Minimal clinical staging is recommended. The prognosis is usually very good. Primary cutaneous ALCL must be distinguished from secondary skin lesions of systemic ALCL, which have a less favourable prognosis. Solitary or localized lesions can be treated by radiotherapy or surgical excision. Multifocal skin lesions can best be treated with low-dose methotrexate or bexarotene. Relapses are frequent in the skin. Extracutaneous dissemination occurs in approximately 10% of the patients, and mainly involves the regional lymph nodes. Patients with extracutaneous disease or with rapidly progressive skin disease should be treated with multiagent chemotherapy.

© 2010 Lippincott Williams & Wilkins, Inc.