P100 Primary cutaneous peripheral T-cell lymphoma, unspecified: a case report

Abstract

Introduction Primary cutaneous T-cell lymphomas (PCTCLs) represent 76,9% of all primary cutaneous lymphomas. MF/Sezary and CD30+ T cell lymphoproliferative disorders are the most common, while the histological entity of PTCL unspecified (NOS) represents<10% of all CTCLs [1–4]. Case history A 44-year-old male patient presented to our department with several (up to 10) asymptomatic, isolated, sharply demarcated violet red nodules on trunk, extremities, as well as, on his face, and neck. The lesions were generally oval and ranged size from 1 to 4 cm in diameter. According to the patient, the skin lesions appeared on his face 9 months ago, and become multiple and more generalized. Initial skin biopsy was consistent with the diagnosis of pseudo-T-cell lymphoma. Three months later, a new skin biopsy was performed due to progression of the disease, and the diagnosis of peripheral T cell lymphoma unspecified was reached. Histological examination revealed diffuse infiltration of dermis with medium and large size lymphoid cells. Immunophenotypic analysis showed expression of the following markers: CD3+, CD4+, CD43+, while there was no expression of CD30. Ki67 was expressed on 60–70% at large lymphoid cells. Tissue clonality studied by PCR detected positive TCR rearrangement. Physical examination, complete blood cell counts, computed tomography of chest and abdomen and bone marrow aspirate failed to demonstrate extracutaneous disease. After all the adequate staging procedures the diagnosis of a primary cutaneous peripheral T cell lymphoma unspecified was established. The patient was treated with combined chemotherapy (cycloposphamide, Mitoxantron, Vincristine, and Prednisolone). Complete remission of skin lesions was achieved after the second course of therapy. Discussion Most patients with unspecified PTL presented with nodular or tumorous skin lesions, solitary, localized or multifocal [1]. The duration of skin lesions prior to diagnosis varied between 1 and 100 months (median 6 months) [1] Therapy consists in most cases of Doxorubicin-based chemotherapy (CHOP or CHOP-like), radiotherapy, or a combination of both. Complete remission can be achieved but are generally short-lived, requiring additional multiagent chemotherapy soon afterward. Prognosis is poor, with a 5-year survival rate of<20%. Conclusion PTCLs unspecified, presenting in the skin are rare and have an unfavorable prognosis. It is fair to conclude that it is as yet unknown how such patients with a PTL unspecified, can best be treated. Because of the inefficacy of current standard regimens, new therapies should be investigated.