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.
- anaplastic large cell lymphoma
- anaplastic lymphoma kinase
- cyclophosphamide, Adriamycin, vincristine, prednisone
ASJC Scopus subject areas