At a Glance
Primary cutaneous anaplastic large cell lymphoma is a primary cutaneous CD30 positive T-cell lymphoproliferative disorder. Lymphomatoid papulosis is a more indolent and self-healing condition. Cutaneous anaplastic large cell lymphoma is a type of cutaneous T-cell lymphoma.
Patients present at a median age of 60 with skin nodules or tumors, sometimes solitary, with rapid growth and ulceration, and patients often relapse. Only 10% of cutaneous anaplastic large cell lymphoma patients have dissemination to lymph nodes. It is a common form of T-cell lymphoma in HIV-infected patients. The diagnosis is made on skin biopsy, by the finding of sheets of large cells with anaplastic morphology. Mycosis Fungoides should be excluded, which can be done by clinical history and cellular features, as both can show expression of CD 30, a protein also expressed by the neoplastic cells of Hodgkin lymphoma. The neoplastic cells typically show aberrant loss of T-cell antigens, such as CD5, CD2, or CD3.
Cutaneous anaplastic large cell lymphoma usually lacks expression of anaplastic lymphoma kinase (ALK), usually tested by immunohistochemistry on paraffin embedded tissue. Distinction from a systemic form of ALK positive anaplastic large cell lymphoma is made by the lack of expression of the anaplastic lymphoma kinase and the absence of any evidence of lymphoma beyond the original skin lesion. Primary cutaneous anaplastic large cell lymphoma is an indolent disease. Up to 40% of cases show spontaneous regression, and 5-year survival rates are on the order of 90%.
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