Primary Cutaneous CD8(+) CD30(+) Anaplastic Large Cell Lymphoma: An Unusual Case with a High Ki-67 index-A Short Review.

Abstract

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a part of the spectrum of CD30+ cutaneous lymphoproliferative disorder, characterized by variable degrees of CD2, CD3, CD4 and CD5 expression by lymphoid cells. PCALCLs with an expression of cytotoxic phenotype (CD8+) and cytotoxic proteins are uncommon. Cutaneous CD8+ CD30+ lymphoproliferative lesions are difficult to classify, diagnose and may be the cause of misdiagnose. CD8+ PCALCL must be distinguished from CD8+ mycosis fungoides, lymphomatoid papulosis type D and primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. Usually CD8+ PCALCL is an indolent disease with a favorable prognosis, except few cases can show poor outcomes. The high Ki-67 index points toward advanced PCALCL. Treatment modalities include surgical excision, radiotherapy and clinical monitoring. Chemotherapy is reserved for disseminated disease. We report a 59-year-old male presented with rapid development of multiple painful reddish-brown plaques and nodular ulcerative skin lesions over the left thigh region since 2 months. A diagnosis of CD8+ PCALCL with a high Ki-67 index was made on the basis of histology and immunohistochemistry, in co-relation with clinical presentation.