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Primary cutaneous CD30 + anaplastic large cell lympho-mas (pc-ALCL) belong to the group of rare, non-mycosis fungoides T-cell lymphomas (1). Similar to lymphomatoid papulosis the characteristic immunohistochemical finding of pc-ALCL is CD30-positivity of infiltrating neoplastic T cells. To date, only limited data is available on the treatment of pc-ALCL and current recommendations are largely based on case reports and small cohort studies (2). Surgical excision and/or radiotherapy are usually performed in limited disease. In cases of disseminated disease, multi-agent chemotherapy has shown good effects, but is accompanied with high toxicity and high recurrence rates. Herein, we report a case of refractory, widespread pc-ALCL that was successfully treated with brentuximab vedotin. A 56-year-old Caucasian man was initially referred to our department with a solid erythematous nodule 4 cm in diameter located in the left popliteal region. The patient's further medical history included coronary heart disease, peripheral arterial disease, and hyperuricemia. Histopathological evaluation of a lesional biopsy showed diffuse lymphocytic infiltrations of primarily large anaplastic cells within the entire dermis. Immunohistochemical analysis revealed CD4-, CD5-, and CD30-positivity in more than 70% and expression of Ki-67 in more than 50% of infiltrating cells (Fig. 1). The lymphocytes showed a loss of CD3 expression and were negative for anaplastic lymphoma kinase-1 (ALK-1) and T-cell beta chain antigen receptor F1 (beta F-1). CD8 was punctually expressed by small nuclei lymphocytic cells. At this time, there was no evidence for extracutaneous disease. Based on the clinical and histopathological findings, a diagnosis of CD30 + pc-ALCL was made and the tumour was completely excised. Seven months later the patient presented with a new nodule located at the preexisting scar as well as palpable, enlarged lymph nodes in the left inguinal region. Histological analysis of the lesions showed secondary infiltration of the CD30 + pc-ALCL (the histological evaluation of 5 out of 8 removed lymph nodes showed loss of normal lymph node architecture as well as large amounts of tumour infiltrates consisting of large, anaplastic, proliferating, CD30 + lymphoid cells). Complete computed tomography (CT) scan and bone marrow biopsy were unremarkable. We decided to initiate radiation therapy of the popliteal and inguinal area as well as low-dose therapy with methotrexate (15 mg/week). However, new skin lesions and lymph node metastases developed within a few weeks. Subsequent inguinal lymphadenectomy, a second course of radiation therapy, low-dose interferon alpha, bexarotene, and 6 cycles of mono-chemotherapy with gemcitabine did not result …

Complete Remission of Refractory, Ulcerated, Primary Cutaneous CD30+ Anaplastic Large Cell Lymphoma Following Brentuximab Vedotin Therapy