Elsevier

The Journal of Pediatrics

Volume 108, Issue 2, February 1986, Pages 267-270
The Journal of Pediatrics

Clinical and laboratory observation
Allogeneic bone marrow transplantation for erythrophagocytic lymphohistiocytosis

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  • Stem cell transplantation for children with hemophagocytic lymphohistiocytosis: Results from the HLH-2004 study

    2020, Blood Advances
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    The most frequent severe sequelae in pediatric HLH are associated with central nervous system (CNS) involvement, often leading to neurologic deficits.1,14,15 Treatment of HLH aims to downregulate the hyperactive immune system; in addition, for primary HLH, hematopoietic stem cell transplantation (HSCT) is required for cure, as first shown by Fischer et al.16-18 HSCT is also needed for certain forms of sHLH, such as chronic active Epstein-Barr virus infection, and some cases of malignancy-associated HLH.19-21 As a result of collaborations worldwide and new treatment protocols, survival in HLH has improved dramatically.1,22-26

  • Outcomes of Children with Hemophagocytic Lymphohistiocytosis Given Allogeneic Hematopoietic Stem Cell Transplantation in Italy

    2018, Biology of Blood and Marrow Transplantation
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    However, in patients with familial/genetic, relapsing, or severe and persistent disease, allogeneic hematopoietic stem cell transplantation (HSCT) is the only established curative treatment [8]. HSCT in a patient with HLH was first reported in 1986, and many case series have since then been described [9]. Significant transplant-related mortality (TRM) was reported in earlier experiences, with an overall survival (OS) ranging between 45% and 65% [10-12].

  • Confirmed efficacy of etoposide and dexamethasone in HLH treatment: Long-Term results of the cooperative HLH-2004 study

    2017, Blood
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    It can be difficult to distinguish between primary HLH and sHLH at onset, and both often require chemotherapy and/or immunotherapy. For primary cases, hematopoietic stem cell transplantation (HSCT) is required for cure,17-19 and may also be necessary for some forms of sHLH such as chronic active Epstein-Barr virus (EBV) infection.20,21 Three decades ago, long-term survival in HLH was <5%.1

  • Hemophagocytic lymphohistiocytosis in children

    2016, Acta Haematologica Polonica
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