Dear Editor, Primary cutaneous lymphomas are defined as non-Hodgkin lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. Primary cutaneous diffuse large B cell lymphoma-leg type (PCDLBCL-LT) is a rare type of lymphoma (1 to 3 % of all cutaneous lymphomas), of poor prognosis with a median survival of 5 years for 50 to 60 % of patients [1, 2]. While rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone(R-CHOP) with or without IFRT is recommended as the first line of treatment in these lymphomas, nowadays, there are insufficient studies on relapsed PCDLBCL-LT [3, 4]. Oral lenalidomide monotherapy is active in relapsed or refractory aggressive NHL, with manageable side effects [5, 6]. We describe the clinical course of a woman, aged 83, presented to our hospital with a 6-month history of asymptomatic, continuously growing, and hyperpigmented nodules and plaques at her right lower leg and thigh. The patient underwent a complete evaluation including clinical history, physical examination with identification of all skin lesions, laboratory analyses, as well as histological and imaging examination including computed tomography of the chest and abdomen and a bone marrow aspiration to exclude a systemic lymphoma. An excisional biopsy was performed and histological examination revealed an infiltrate with the predominance of large neoplastic CD20-positive B cells (CD20, CD79, MUM1, BCL2+; CD30, CD10, BCL6−). The serum LDH level and the computed tomography scans of the chest and abdomen were normal. Being confirmed as PCDLBCL-LT, we initiated immunochemotherapy with R-CHOP protocol in 75 % dose reduction. A partial remission (defined as >50 % reduction in tumor burden) was achieved after two courses of modified R-CHOP. However after the fifth course, the patient refrained from further treatment against medical advice. After 6 months of disease stabilization, progressing lymphoma at both legs, multiple, rapidly growing tumors with spontaneous ulceration (Fig. 1a), without other pathological findings, forced her to seek treatment again. Lenalidomide (25 mg once daily) on days 1 to 21 of every 28-day cycle, administered orally, was initiated about 5 months ago. Objective responses were achieved already throughout the first cycle (Fig. 1b). Partial remission with disease stabilization was obtained after the fourth course of lenalidomide and currently continues at the fifth cycle. The tolerance was excellent, so no dose reductions was necessary; no thrombosis prophylaxis was performed. PCDLBCL-LT is a rare type of lymphoma of poor prognosis, characterized by a very aggressive clinical course with high recurrence rates and a median survival of 5 years for 50 to 60 % of patients. ISCL/EORTC recommendations for the management of PCDLBCL-LT suggest that immunochemotherapy with R-CHOP currently represents the most effective treatment. For recurring PCDLBCL-LT, no standardized treatment recommendations exist. Here, we present the first example, to our knowledge, of a major response to lenalidomide in relapsed/refractory PCDLBCL-LT. Lenalidomide has demonstrated significant clinical activity and a manageable safety profile. We believe that the use P. Savini :A. Lanzi (*) : F. G. Foschi :G. Marano : G. F. Stefanini U.O. Medicina Interna, Ospedale di Faenza, Via Stradone no. 9, 48018 Faenza, RA, Italy e-mail: lanziari@yahoo.com
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Open Access
Aug 28, 2019
Hussein M Khaled + 4
