Long-Term Remission After Matched Sibling Donor Hematopoietic Cell Transplantation in a Patient With Primary Cutaneous CD8 Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma

摘要

Primary cutaneous CD8 aggressive epidermotropic cytotoxic T-cell lymphoma (CD8 PCAECTL) is an extremely rare neoplasm with a poor prognosis. Chemotherapy typically does not result in a sustained response, and hematopoietic stem cell transplant (HSCT) is the only therapy that has been shown to produce a durable response of any kind. Here, we report a case of a 25-year-old?previously healthy male who presented with a painful ulcerative lesion on the bottom of his right great toe and local lymphadenopathy. The biopsy of the lesion was consistent with CD8 PCAECTL. He received immediate chemotherapy followed by matched related donor HSCT (MRD-HSCT) and remained in complete remission (CR) for eight years post-transplant, longer than any CR reported in the literature. In conclusion, our report provides clinical evidence that early transplant consult and donor search is one of the key factors in the management of CD8 PCAECTL.