Fatal Cutaneous γ/δ T-Cell Lymphoma with Central Nerve System Metastasis

Abstract

Cutaneous γ/δ T-cell lymphoma is an extremely rare and highly aggressive tumor that is often resistant to treatment, such as polychemotherapy and radiotherapy. Due to the complexity of clinical, pathologic, and immunohistochemical features of this disease entity, the physician should perform a careful evaluation; however, treatment should be rapid and aggressive. We present a case of fatal cutaneous γ/δ T-cell lymphoma of a 55-year old woman who died after recurrence with central nerve system metastasis.

Cutaneous gamma‐delta T‐cell lymphoma with central nervous system involvement: report of a rarity with review of literature

“…Accompanying hemophagocytic lymphohistiocytosis/syndrome was also observed in both cases. The average clinical course from the initial presentation to CNS manifestations was 17.2 months (ranging from 6.5 to 30 months) . In addition, a recent large study that included 53 patients with γδ T‐cell lymphoma first presenting in the skin, reported three (<5%) cases that had central nervous system involvement .…”

“… M, male; F, female; CNS, central nerve system; CSA, cyclosporine; FND, fludarabine, Mitoxantrone, Dexamethasone; MOF, multiple organ failure; Ref No., reference number. Cases 2–5 were literature review from Refs respectively. …”

“…The average clinical course from the initial presentation to CNS manifestations was 17.2 months (ranging from 6.5 to 30 months). 5,11,12 In addition, a recent large study that included 53 patients with γδ T-cell lymphoma first presenting in the skin, reported three (<5%) cases that had central nervous system involvement. 3 These cases are not shown in Table 1 due to lack of detailed clinical information.…”

“…CNS involvement and hemophagocytic syndrome are complications that can arise in cutaneous γδ T‐cell lymphoma, both of which could be fatal. In WHO classification, it is defined as mature, activated γδ T‐cells with a cytotoxic phenotype . Clinical manifestations of cutaneous γδ T‐cell lymphoma are variable and some could be mistaken as MF as patients frequently present with patches, plaques, nodules, or necrotic tumors, which are often ulcerated and primarily affect the extremities .…”

“…In WHO classification, it is defined as mature, activated γδ T-cells with a cytotoxic phenotype. 2,3,5 Clinical manifestations of cutaneous γδ T-cell lymphoma are variable and some could be mistaken as MF as patients frequently present with patches, plaques, nodules, or necrotic tumors, which are often ulcerated and primarily affect the extremities. 3,6 Indolent cutaneous γδ T-cell lymphoma associated with localized atypical lymphocytic lobular panniculitis often resembles subcutaneous panniculitis-like T-cell lymphoma.…”