Transplantation in the Treatment of Primary Cutaneous Aggressive Epidermotropic Cytotoxic CD8-Positive T-Cell Lymphoma

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Abstract

Background

Primary cutaneous aggressive epidermotropic cytotoxic CD8 positive T-cell lymphoma (CD8+ PCAETL) is a rare subtype of peripheral T-cell lymphoma with poor outcomes and without a standardized treatment strategy. Allogeneic hematopoietic stem cell transplantation (HSCT) has been suggested as a potential curative therapy.

Patients and Methods

We conducted a retrospective case series. We identified 8 patients with the diagnosis of CD8+ PCAETL, 4 of whom also underwent allogeneic HSCT.

Results

Eight patients were treated at our center with combination chemotherapy and several novel agents, including histone deacetylase inhibitors, brentuximab, and pralatrexate. Patients underwent a median of 8.5 treatments before HSCT. Six of the 8 patients examined, including all 4 who received an HSCT, were alive at their last follow-up.

Conclusion

Allogeneic HSCT is a promising treatment modality for CD8+ PCAETL. Because of the aggressive nature of this disease and lack of sustained remission with currently available therapies, HSCT should be considered early in the course of treatment. Two novel agents, brentuximab and pralatrexate, showed significant activity against CD8+ PCAETL, and may be incorporated earlier in the treatment course.

Micro-Abstract

Several studies have suggested that hematopoietic stem cell transplantation (HSCT) might provide a cure for primary cutaneous aggressive epidermotropic cytotoxic CD8-positive T-cell lymphoma (CD8+ PCAETL). We summarize published literature on this disease and present outcomes of 8 patients with CD8+ PCAETL treated at our institution. In our experience, allogeneic HSCT and the novel agents brentuximab and pralatrexate show substantial activity against this disease.

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