Complete Response to Donor Lymphocyte Infusion for Primary Hemophagocytic Lymphohistiocytosis Relapse after Allogeneic Hematopoietic Cell Transplantation
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Published:2023-11-23
Issue:
Volume:
Page:1-4
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ISSN:0001-5792
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Container-title:Acta Haematologica
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language:en
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Short-container-title:Acta Haematol
Author:
Khanolkar Rutvij A.,Kuehne Nathan,Storek Jan
Abstract
<b><i>Introduction:</i></b> 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. <b><i>Case Presentation:</i></b> 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. <b><i>Conclusion:</i></b> To our knowledge, this represents the first case demonstrating the efficacy of DLI for relapsed primary HLH.
Subject
Hematology,General Medicine