Brentuximab vedotin in multifocal cutaneous anaplastic large cell lymphoma in a patient with human immunodeficiency virus following Hodgkin lymphoma

Abstract

A 50-year-old Nigerian man presented in January 2017 with extensive widespread nodules on his face, trunk and limbs (Fig. 1). Some had become ulcerated or necrotic, while others had resolved spontaneously. The patient had some small volume inguinal lymphadenopathy but was systemically well. He had been diagnosed with human immunodeficiency virus (HIV) in 2010, but had not received treatment because he had a high CD4 count (1758 9 10/L) and this was the procedure in the guidelines at that time. His comorbidities included chronic hepatitis B and Type 2 diabetes. In 2014, he developed biopsy-proven eczema unresponsive to topical treatments, and was treated with two short courses of oral prednisolone, but the condition flared soon afterwards. The patient was started on oral ciclosporin 150 mg twice daily in January 2016, which was increased to 200 mg twice daily and then 250 mg twice daily (5 mg/kg/day) in March 2016. In April 2016, he developed left axillary lymphadenopathy, and following excision biopsy and positron emission tomography–computed tomography (PET-CT), he was diagnosed with stage IV Hodgkin lymphoma (HL) and his ciclosporin was stopped. He was then treated with six cycles of ABVD (doxorubicin, bleomycin, vinblastine and dacarbazine) chemotherapy and antiretroviral therapy with emtricitabine/tenofovir (Truvada; Gilead Sciences, Cambridge, UK) and raltegravir. His HIV viral load was suppressed within 2 months and his CD4 count remained at > 800 9 10/L. The end-of-treatment PET-CT scan showed a significant reduction in the size and fluorodeoxyglucose (FDG) avidity of the lymphadenopathy but without complete resolution. A repeat inguinal lymph node biopsy in December 2016 showed no evidence of HL. This was therefore interpreted as complete remission of HL, with the persistent lymphadenopathy due to active eczema. Skin biopsies were taken from the nodules and an inguinal lymph node and histological examination revealed a prominent dermal lymphoid infiltrate with a predominance of large lymphoid blasts, positive for CD30 expression, and negative for anaplastic lymphoma kinase (ALK). PET-CT showed widespread metabolic cutaneous activity with increased metabolic activity in the axillary and inguinal nodes but no deep lymphadenopathy. The differential diagnosis of CD30+ ALK-negative neoplasms involving the skin includes primary cutaneous anaplastic large cell lymphoma (cALCL), systemic anaplastic large cell lymphoma (sALCL) with secondary skin involvement, transformed mycosis fungoides (MF) and lymphomatoid papulosis (LyP). A review of the original eczematous biopsy excluded MF. LyP was considered unlikely due to the large tumours, peripheral lymphadenopathy and the lack of complete resolution of all lesions. As the patient was systemically well with ALK negativity and superficial lymphadenopathy and no organ involvement, these findings favoured a diagnosis of cALCL with secondary lymph node involvement. The patient was started on treatment with the antiCD30 agent brentuximab vedotin as an intravenous infusion once every 3 weeks at 1.8 mg/m. Following two cycles, the cutaneous lesions regressed and there were no new lesions or lymphadenopathy. He tolerated treatment well, with no infusion reactions or significant adverse effects (AEs). PET-CT after Cycle 4 Correspondence: Dr Sonia Wolf, Department of Haemato-Oncology, St Bartholomew’s Hospital, Barts Health NHS Trust, West Smithfield, London, EC1A 7BE, UK E-mail: [email protected]