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- Title
Complete Response to Donor Lymphocyte Infusion for Primary Hemophagocytic Lymphohistiocytosis Relapse after Allogeneic Hematopoietic Cell Transplantation.
- Authors
Khanolkar, Rutvij A.; Kuehne, Nathan; Storek, Jan
- Abstract
Introduction: Primary hemophagocytic lymphohistiocytosis (HLH) is a hyper-inflammatory disorder characterized by dysregulation of inflammatory cells and cytokine signaling. Although first-line treatment consisting of immunosuppressive therapy and allogeneic hematopoietic cell transplantation (HCT) is often curative, it remains unknown whether any effective therapies exist for disease relapse/progression after HCT. Case Presentation: Here we present a case of a 29-year-old male with primary HLH who failed HLH-94 protocol and subsequently underwent myeloablative HCT. Disease relapse occurred at 9 months following HCT, and donor lymphocyte infusion (DLI) was initiated as salvage therapy. The patient subsequently achieved durable long-term disease-free survival following a DLI, without significant treatment-related complications. Conclusion: To our knowledge, this represents the first case demonstrating the efficacy of DLI for relapsed primary HLH.
- Subjects
HEMATOPOIETIC stem cell transplantation; HEMOPHAGOCYTIC lymphohistiocytosis; DISEASE relapse; PROGRESSION-free survival; SALVAGE therapy
- Publication
Acta Haematologica, 2024, Vol 147, Issue 4, p489
- ISSN
0001-5792
- Publication type
Article
- DOI
10.1159/000535449