Improved transplant outcomes with myeloablative conditioning for hemophagocytic lymphohistiocytosis in HLA-matched and mismatched donors: a national multicenter retrospective study

Ness, Yarden Greental; Kuperman, Amir A.; Stein, Jerry; Yacobovich, Joanne; Even‐Or, Ehud; Zaidman, Irina; Gefen, Aharon; Nevo, Neta; Oberman, Bernice; Toren, Amos; Stepensky, Polina; Bielorai, Bella; Jacoby, Elad

doi:10.1038/s41409-021-01290-1

Cited by 6 publications

(3 citation statements)

References 46 publications

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“…Therefore, we inferred that the prognosis of early transplant following partial response yields a better prognosis than delayed transplant following complete remission in children with hemophagocytic syndrome. At the same time, Greental et al (28) showed that 5-year EFS rates were higher with MAC regimen for transplant from either HLA matched or alternative donors, so the patient used MAC as conditioning regimen. Certainly controlling hemophagy was also a vital factor for achieving long-term survival.…”

Section: Literature Searchmentioning

confidence: 99%

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Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review

Bingjie,

Linlin,

Futian

et al. 2024

Front. Immunol.

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ObjectivesThis study aims to discuss the clinical manifestations and treatment of Familial hemophagocytic lymphohistiocytosis (FHL) caused by a mutation in the UNC13D gene.MethodsA 6-year-old female child presented with unexplained febricity, splenomegaly, pancytopenia, hemophagocytic lymphohistiocytosis in bone marrow, decreased NK cell activity, soluble CD25 levels > 44000ng/ml. Genetic sequencing revealed a mutation in the UNC13D gene. Additionally, the patient experienced intermittent fever with seizures characterized by involuntary twitching of the left upper limb. Head magnetic resonance imaging (MRI) showed white matter lesions.ResultsAccording to the HLH-2004 diagnostic criteria revised by the International Society of Histiocytosis the patient was diagnosed with FHL. Despite receiving HLH-2004 treatment, the disease relapsed. However, after a salvage allogeneic Hematopoietic Stem Cell Transplant (HSCT), febricity, abnormal blood cells, and neurological symptoms significantly improved.ConclusionsPrompt performance of allogeneic HSCT is crucial upon diagnosis of FHL, especially when neurological involvement is present.

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Section: Literature Searchmentioning

confidence: 99%

“…At the same time, Greental et al. ( 28 ) showed that 5-year EFS rates were higher with MAC regimen for transplant from either HLA matched or alternative donors, so the patient used MAC as conditioning regimen. Certainly controlling hemophagy was also a vital factor for achieving long-term survival.…”

Section: Literature Searchmentioning

confidence: 99%

Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review

Bingjie,

Linlin,

Futian

et al. 2024

Front. Immunol.

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“…Better survival results initially achieved with reduced intensity conditioning (three-year survival of 92% after RIC vs 43% after myeloablative conditioning--MAC) [12], raised interest in this method, but problems with engraftment and mixed chimerism cause a need for its optimization [11,[13][14][15][16][17][18]. MAC approach can be more beneficial in terms of EFS (event-free survival) [19].…”

Section: Introductionmentioning

confidence: 99%

Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT

Machowicz

Suarez

Wiktor-Jedrzejczak

et al. 2022

Bone Marrow Transplant

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Hemophagocytic lymphohistiocytosis (HLH; hemophagocytic syndrome) is a rare syndrome of potentially fatal, uncontrolled hyperinflammation. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is indicated in primary, recurrent or progressive HLH, but information about its outcomes in the adult population is limited. We obtained data about 87 adult (≥18 years of age) patients retrospectively reported to the EBMT. The median survival time was 13.9 months. The three and five-year overall survival (OS) was 44% (95% CI 33-54%). Among 39 patients with a follow-up longer than 15 months, only three died. Relapse rate was 21% (95% CI 13-30%), while NRM reached 36% (95% CI 25-46%). Younger patients (<30 years of age) had better prognosis, with an OS of 59% (95% CI 45-73%) at three and five years vs 23% (95% CI 8-37%) for older ones. No difference in survival between reduced and myeloablative conditioning was found. To our knowledge, this is the largest report of adult HLH patients who underwent allo-HSCT. Patients who survive the first period after this procedure can expect a long disease-free survival. Both reduced intensity and myeloablative conditioning have therapeutic potential in adult HLH.

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Isolated Full Donor T-Cell Chimerism After Haploidentical TCRαβ/CD19 Depleted HSCT Maintains Remission of Familial HLH

Consonni,

Coniglio,

Martini

et al. 2023

J Clin Immunol

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Improved transplant outcomes with myeloablative conditioning for hemophagocytic lymphohistiocytosis in HLA-matched and mismatched donors: a national multicenter retrospective study

Cited by 6 publications

References 46 publications

Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review

Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review

Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT

Isolated Full Donor T-Cell Chimerism After Haploidentical TCRαβ/CD19 Depleted HSCT Maintains Remission of Familial HLH

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