Clinical, Diagnostic and Prognostic Characteristics of Primary Cutaneous Gamma Delta T-cell Lymphomas

Abstract

Primary cutaneous γδ T-cell lymphoma (PCGDTL) is a rare subtype of non-Hodgkin lymphoma (NHL) that arises from T-cells with γδ T-cell receptors. The exact incidence of PCGDTL is unknown, as it is usually lumped with other cutaneous lymphomas, which are also uncommon. It is one of the peripheral T-cell lymphoma (PTCL) subtypes which is known to have a dismal prognosis due to poor response and the paucity of available therapies. Despite the rarity and uncertainties of PCGDTL, a number of studies over the past decade were published about the pathologic, diagnostic, cytogenetic and clinical features of this disease. These diagnostic advances will open the doors to explore new therapeutics for this rare entity, specifically targeted and immune therapies. In this review, we highlight these advances, summarize the contemporary treatment approaches, and shed the light on future potential therapeutic targets.

Keywords: Cutaneous lymphomas; Gamma-delta; T-cell lymphoma.

Fig. 1

Suggested algorithm for management of primary cutaneous gamma-delta lymphoma. Allo-HCT allogeneic hematopoeitic cell transplant; BV-CHP: brentuximab vedotin, cyclophosphamide, hydroxydaunorubicin, prednisone; CHOP cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone; CR complete respone; EPOCH etoposide, prednisone, vicristine, cyclophosphamide, hydroxydaunorubicin; HCVAD hyperfractionated cycyclophosphamide, vincristine, adriamycin and dexamethasone; ICE ifosfamide, carboplatin, etoposide; PD progressive disease; PR partial remission