LY12 Primary cutaneous large cell anaplastic lymphoma and lymphomatoid papulosis: clinical characteristics, treatment patterns and outcomes from a UK supraregional skin lymphoma centre

Abstract

Primary cutaneous anaplastic large cell lymphoma (ALCL) is a CD30-positive cutaneous T-cell lymphoma that presents in the skin. It is associated with an excellent prognosis, although relapses can occur. Lesions are often solitary, and do not normally progress to systemic disease. Management is therefore usually skin directed, most commonly with surgical excision or local radiotherapy. Lymphomatoid papulosis (LyP) is a chronic, relapsing–remitting CD30-positive cutaneous lymphoproliferative disorder. The histology is similar to that of ALCL, but it is benign, characterized by self-resolving papules and nodules and a low risk of progression to systemic disease. Treatment is frequently conservative, ranging from observation to topical corticosteroids, with systemic treatment reserved for patients with widespread or symptomatic disease. We performed a retrospective analysis of patients with a confirmed diagnosis of either ALCL or LyP, identified from hospital records. The data pro forma included the type of diagnosis, age at diagnosis, treatments given and whether patients had concomitant mycosis fungoides or another malignancy. In total, 62 patients were identified; 60% of patients had LyP, 37% had ALCL and 3% developed both types of lesions. Out of the patients who had LyP, 54% were confirmed to have type A. The mean age at ALCL diagnosis was 55 years (range 21–91), and the mean age at LyP diagnosis was 47 years (range 22–74). There was heterogeneity in treatment approaches, with topical corticosteroids (39%), localized radiotherapy (37%) and methotrexate (19%) most commonly used. One patient, with systemic disease, required chemotherapy and bone marrow transplantation. Concomitant mycosis fungoides occurred in 20% of patients with ALCL and 31% with LyP. Other malignancies, including skin malignancies, occurred in 10% of patients. ALCL and LyP usually run a benign course and rarely progress to systemic disease. The mainstays of treatment are topical cortico­steroids, localized radiotherapy, methotrexate and observation for recurrent lesions. It is important to monitor these patients for concomitant malignancies.