Juliet Fraser Gibson1, Onder Alpdogan2, Antonio Subtil3, Michael Girardi4, Lynn D Wilson5, Kenneth Roberts5, Francine Foss6. 1. Yale School of Medicine, New Haven, Connecticut. Electronic address: Juliet.gibson@yale.edu. 2. Department of Medical Oncology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania. 3. Department of Dermatology and Pathology, Yale University School of Medicine, New Haven, Connecticut. 4. Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut. 5. Department of Therapeutic Radiology, Yale University School of Medicine, New Haven, Connecticut. 6. Hematology and Bone Marrow Transplantation, Yale University School of Medicine, New Haven, Connecticut.
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.
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.
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