Primary Cutaneous CD30+/ALK- ALCL with Transition into sALCL: Favourable Response after Systemic Administration with Brentuximab Vedotin! Unique Presentation in a Bulgarian Patient!

BACKGROUND: Modern drugs could sometimes be a good solution even to problematic patients. The cutaneous and systemic forms of the CD30 positive anaplastic large T-cell lymphoma could often be described as a suitable target for therapy with Brentuximab vedotin. CASE REPORT: We present the first case of a Bulgarian patient with a histologically confirmed primary cutaneous T-cell CD30+/ALK- large anaplastic cell lymphoma-cALCL (therapeutically resistant to therapy with Methotrexate, radiation therapy and systemic corticosteroid therapy) who was successfully treated with Brentuximab vedotin. In several years, the patient has developed a comparatively fast skin progression as well as an initial systemic one which impacts inguinal and mediastinal nodes. After the implementation of 4 therapy cycles with Brentuximab vedotin, complete regression of the described by previous hospitalisations lymph nodes as well as 80% reduction of the cutaneous and subcutaneous located tumour formations were observed. CONCLUSION: The therapy of CD30+/ALK- anaplastic large T-cell lymphoma is a significant challenge for oncologists and dermatologists because it requires maximally efficient and minimally traumatic treatment in parallel. Therapy with Brentuximab is a new direction which shows extremely good clinical results and can be applied to the cutaneous as well as to the systemic form of anaplastic large-cell CD30 positive lymphoma. The key element by treatment with Brentuximab is suppression of the CD30- expression which, in turn, could be the cause of relapses. On that ground, patients with these lymphomas should be strictly monitored.