Hematopoietic stem cell transplantation for primary cutaneous γδ T-cell lymphoma and refractory subcutaneous panniculitis-like T-cell lymphoma

Abstract

Background: The panniculitic T-cell lymphomas (TCLs) comprise 2 distinct entities, αβ subcutaneous panniculitis-like TCL (SPTCL) and the γδ cutaneous TCLs with pannicular involvement primary cutaneous γδ (PCGD)-TCL. Although outcomes for most patients with SPTCL are favorable, those with PCGD-TCLs generally have an inferior outcome, and treatment strategies have not been well defined. Allogeneic hematopoietic stem cell transplantation (HSCT) has been shown to be a potentially curative strategy in aggressive TCLs and in refractory and advanced-stage mycosis fungoides.

Objective: We sought to analyze the outcomes of HSCT for panniculitic cutaneous TCL.

Results: Fourteen patients (4 SPTCL, 10 PCGD-TCL) presented with primarily pannicular T-cell infiltrates. Seven patients underwent allogeneic HSCT from matched-related donors and matched-unrelated donors of which 4 (57%) are alive (1 SPTCL, 3 PCGD-TCL) at 7.8, 6.9, 6.2, and 0.25 years. Two patients underwent autologous HSCT (1 SPTCL, 1 PCGD-TCL) and both are alive at a median follow-up of 1.91 years.

Limitations: This study is limited by its retrospective nature and small sample size because of the rarity of SPTCL and PCGD-TCL.

Conclusion: Aggressive therapy followed by allogeneic HSCT is a promising treatment modality for patients with PCGD-TCL.

Keywords: T-cell lymphoma; hematopoietic stem cell transplantation; primary cutaneous γδ T-cell lymphoma; subcutaneous panniculitis-like T-cell lymphoma; γ-δ.