Angiomatous-like Nodules on Chronic Lymphedema Revealing Systemic ALK-Negative ALCL

Introduction: Chronic lymphedema (CL) can lead to infectious complications, skin inflammation, and tumors. Consequently, patients with CL are at increased risk of developing several malignant cutaneous neoplasms, including Kaposi sarcoma, B-cell lymphoma, and angiosarcoma. T-cell lymphomas are rarely associated with CL. Additionally, systemic anaplastic large cell lymphomas (ALCLs) are usually positive for anaplastic lymphoma kinase (ALK). We present the case of a 73-year-old patient who exhibited angiomatous-appearing cutaneous lesions, ultimately leading to a diagnosis of ALK-negative systemic ALCL, exclusively localized to the CL of the left lower limb. Case Report: A 73-year-old man presented with a 12-year history of CL affecting his left lower limb. He subsequently developed, over a three-month period, an angiomatous-like plaque accompanied by multiple nodules on the same limb. Physical examination showed widespread lymphadenopathy. A skin and lymph node biopsy specimen displayed CD3+, CD30+, ALK-negative atypical lymphoid infiltrates consistent with ALK-negative systemic ALCL with secondary cutaneous involvement. A total body computed tomography (CT) scan showed hepatosplenomegaly and generalized lymphadenopathy. In the Department of Hematology, combination chemotherapy of cyclophosphamide, Adriamycin, vincristine, and prednisolone (CHOP) was started, but the patient was lost to follow-up. Conclusions: We report a unique case of ALK-negative systemic ALCL in a 73-year-old man who presented with angiomatous-like nodular lesions occurring at the site of chronic limb lymphedema. This case demonstrates the importance of a systemic evaluation of all CD30-positive cutaneous lymphoid infiltrates and suggests that chronic lymphatic stasis may be a predisposing factor in the development of ALCL.