(Last Updated On: October 29, 2018)

Authors: Elisabeth Ryan, MD, Mohammad Yoga Adi Waskito, MD, Johannes F. Dayrit, MD, FPDS

Abstract

Introduction:  Anaplastic large-cell lymphoma (ALCL) is a CD30-positive T-cell lymphoma, a rare subtype of Cutaneous T-cell lymphoma (CTCL). It is classified into primary cutaneous ALCL and systemic ALCL subtypes. Primary cutaneous CD30-positive T-cell lymphoma account for 25% of all cutaneous T-cell lymphomas (CTCL). It characterized by localized to several nodules/tumors  with ulceration, may mimic other common skin disease and histologically presents as diffuse sheets of large tumor cells with an anaplastic, pleomorphic or immunoblastic cytomorphology, and largely expressing (>75 %) CD30.

Case summary: A 50-year old woman presented with 5-month history of asymptomatic multiple erythematous plaques with satellite papules on the upper extremities. There were no systemic symptoms. The lesion slowly evolved into nodules with ulceration. Dermatologic examination revealed multiple erythematous plaques and nodule with satellite papules on the upper extremities. Skin punch biopsy showed exocytosis of lymphocyte with no atypia, dense inflammatory infiltrate consists of numerous blastic lymphoid cells with pale pink cytoplasm, and high mitotic activity. By immunohistochemistry, tumor cells are diffusely positive for CD2, CD4 and CD30 and was negative for CD3, CD5 and CD7. CD8 highlights a number of small round lymphocytes. EMA stains about 5% of the cells in the infiltrate and ALK1 is negative consistent with Primary cutaneous CD30-positive Anaplastic large-cell lymphoma. Patient was referred to an oncologist for co-management and chemotherapy.

Conclusion: Primary cutaneous CD30-positive T-cell lymphoma is a rare subtype of Cutaneous T-cell lymphoma (CTCL). The clinical presentation of this disease may mimic other common skin disease and early detection is important.

 

Citation

 

Keywords

Primary cutaneous CD30-positive anaplastic large cell lymphoma, ALCL, CTCL

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