International Journal of Hematology

DOI: 10.1007/s12185-018-02568-9 Pages: 187-196

Hematopoietic stem cell transplantation for subcutaneous panniculitis-like T-cell lymphoma: single center experience in an Asian population

1. Taipei Veterans General Hospital, Division of Hematology, Department of Medicine

2. Taipei Veterans General Hospital, Department of Pathology and Laboratory Medicine

3. National Yang-Ming University, Faculty of Medicine

4. Taipei Veterans General Hospital, Division of Transfusion Medicine, Department of Medicine

5. Taipei Veterans General Hospital, Department of Pediatrics

Correspondence to:
Jyh-Pyng Gau
Tel: 886-2-28757529
Email: jpgau@vghtpe.gov.tw

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Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare form of cytotoxic T-cell lymphoma. It is believed that SPTL in patients without hemophagocytic syndrome (HPS) follows an indolent course; in contrast, SPTL in patients with HPS has been associated with unfavorable survival. To provide more clinical data on SPTL in Asian populations and to identify optimal therapeutic strategies for SPTL, we assessed the clinicopathological features and long-term follow-up data of 10 Taiwanese SPTL patients diagnosed at a single center. Our study demonstrates a group of patients with high incidence of HPS (50%), rather aggressive courses, and early progression. A total of eight patients underwent hematopoietic stem cell transplant (HSCT), including one autologous HSCT and seven allogeneic HSCT. Seven of eight patients receiving HSCT achieved durable remission and maintained in remission for over 30 months (range 30–132 months). There was no difference in 3-year survival of patients with HPS (80%) compared with patients without HPS (80%). Of long-term survivors in the HPS group, three of four received HSCT (autologous HSCT, n = 1; allogeneic HSCT, n = 2). Our study indicated that HSCT is a curative option for eligible SPTL patients with HPS.

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