Clinical and laboratory observationAllogeneic bone marrow transplantation for erythrophagocytic lymphohistiocytosis
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Cited by (132)
Hemophagocytic lymphohistiocytosis: A retrospective analysis of 66 patients
2024, Revue de Medecine InterneStem cell transplantation for children with hemophagocytic lymphohistiocytosis: Results from the HLH-2004 study
2020, Blood AdvancesCitation Excerpt :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 TransplantationCitation Excerpt :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, BloodCitation Excerpt :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