Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disorder of the immune system. While familial HLH is usually seen in children, secondary HLH is more common in adults. Secondary HLH is associated with a wide variety of underlying conditions including infections, malignancy and autoimmune disorders. While HLH 94/04 protocol-based chemotherapy can be used for initial treatment, allogeneic hematopoietic stem cell transplant (allo-HSCT) remains the only potentially curative treatment modality for this disorder. In this systematic review, we analyzed available literature on the role of allo-HSCT in adolescent and adult patients using PubMed, Cochrane, Embase and ClinicalTrials.gov. A total of 11 retrospective studies evaluated the role of allo-HSCT for HLH. Allo-HSCT, when compared to non-HSCT approach, appears to be associated with reasonable efficacy and acceptable safety for adolescent and adult patients with HLH.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$259.00 per year
only $21.58 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
Data availability
The data that support the findings of this study are available from the corresponding author, AM.
References
Morimoto A, Nakazawa Y, Ishii E. Hemophagocytic lymphohistiocytosis: Pathogenesis, diagnosis, and management. Pediatrics Int. 2016;58:817–25.
Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014;383:1503–16.
Masood A, Wahab A, Clifford T, Weaver EJ, Ehsan H, El Ayass W. Secondary hemophagocytic lymphohistiocytosis due to nivolumab/ipilimumab in a renal cell cancer patient-A case report. Clin Case Rep. 2021;9:e05184.
Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH‐2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124–31.
Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: pathogenesis and treatment. Hematol Am Soc Hematol Educ Program. 2013;2013:605–11.
Seo JJ. Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies. Blood Res. 2015;50:131–9.
Jin Z, Wang Y, Wang J, Zhang J, Wu L, Gao Z, et al. Primary hemophagocytic lymphohistiocytosis in adults: the utility of family surveys in a single-center study from China. Orphanet J Rare Dis. 2018;13:17.
Lai W, Wang Y, Wang J, Wu L, Jin Z, Wang Z. Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults and adolescents-a life-threatening disease: analysis of 133 cases from a single center. Hematology. 2018;23:810–6.
Li Z, Wang Y, Wang J, Zhang J, Wang Z. Haploidentical hematopoietic stem cell transplantation for adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Leuk Lymphoma. 2018;59:77–84.
Park H-S, Lee J-H, Lee J-H, Choi E-J, Ko S-H, Seol M, et al. Fludarabine/melphalan 100 mg/m2 conditioning therapy followed by allogeneic hematopoietic cell transplantation for adult patients with secondary hemophagocytic lymphohistiocytosis. Biol Blood Marrow Transplant. 2019;25:1116–21.
Park HS, Kim DY, Lee JH, Lee JH, Kim SD, Park YH, et al. Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors. Ann Hematol. 2012;91:897–904.
Fu L, Wang J, Wei N, Wu L, Wang Y, Huang W, et al. Allogeneic hematopoietic stem-cell transplantation for adult and adolescent hemophagocytic lymphohistiocytosis: a single center analysis. Int J Hematol. 2016;104:628–35.
Jin Z, Wang Y, Wang J, Wu L, Pei R, Lai W, et al. Multivariate analysis of prognosis for patients with natural killer/T cell lymphoma-associated hemophagocytic lymphohistiocytosis. Hematology. 2018;23:228–34.
Lee DE, Martinez-Escala ME, Serrano LM, Zhou XA, Kaplan JB, Pro B, et al. Hemophagocytic lymphohistiocytosis in cutaneous T-cell lymphoma. JAMA Dermatol. 2018;154:828–31.
Yu JT, Wang CY, Yang Y, Wang RC, Chang KH, Hwang WL, et al. Lymphoma-associated hemophagocytic lymphohistiocytosis: experience in adults from a single institution. Ann Hematol. 2013;92:1529–36.
Henter JI, Samuelsson-Horne A, Aricò M, Egeler RM, Elinder G, Filipovich AH, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002;100:2367–73.
Marsh RA, Hebert K, Kim S, Dvorak CC, Aquino VM, Baker KS, et al. Comparison of hematopoietic cell transplant conditioning regimens for hemophagocytic lymphohistiocytosis disorders. J Allergy Clin Immunol. 2022;149:1097–104.E2.
Ouachée-Chardin M, Elie C, de Saint Basile G, Le Deist F, Mahlaoui N, Picard C, et al. Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single-center report of 48 patients. Pediatrics. 2006;117:e743–50.
Hartz B, Marsh R, Rao K, Henter J-I, Jordan M, Filipovich L, et al. The minimum required level of donor chimerism in hereditary hemophagocytic lymphohistiocytosis. Blood. 2016;127:3281–90.
Marsh RA, Vaughn G, Kim MO, Li D, Jodele S, Joshi S, et al. Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation. Blood. 2010;116:5824–31.
Arnaout K, Patel N, Jain M, El-Amm J, Amro F, Tabbara IA. Complications of allogeneic hematopoietic stem cell transplantation. Cancer Invest. 2014;32:349–62.
Nikiforow S, Berliner N, Kim HT, Ho VT, Joyce A, Glotzbecker B, et al. Genetic predispositions, management strategies, and clinical outcomes in adults with hemophagocytic lymphohistiocytosis (HLH) after reduced-intensity conditioning (RIC) hematopoietic stem cell transplantation (HSCT) at dana-farber cancer institute. Biol Blood Marrow Transplant. 2019;25:S398.
Li Z, Wang Y, Wang J, Zhang J, Wang Z. Successful haploidentical stem cell transplantation for three adults with primary hemophagocytic lymphohistiocytosis. Bone Marrow Transplant. 2017;52:330–3.
Marsh RA, Allen CE, McClain KL, Weinstein JL, Kanter J, Skiles J, et al. Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab. Pediatr Blood Cancer. 2013;60:101–9.
Cheson BD, Pfistner B, Juweid ME, Gascoyne RD, Specht L, Horning SJ, et al. Revised response criteria for malignant lymphoma. J Clin Oncol. 2007;25:579–86.
Author information
Authors and Affiliations
Contributions
All authors equally contributed to the conception and design of the work that led to this submission, including acquiring the data and drafting the manuscript. The final version of the manuscript has been approved by all authors who have agreed to be accountable for all aspects of the work.
Corresponding author
Ethics declarations
Competing interests
The authors declare no competing interests.
Additional information
Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Masood, A., Wahab, A., Iqbal, Q. et al. Efficacy and safety of allogeneic hematopoietic stem cell transplant in adults with hemophagocytic lymphohistiocytosis: a systematic review of literature. Bone Marrow Transplant 57, 866–873 (2022). https://doi.org/10.1038/s41409-022-01668-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1038/s41409-022-01668-9
This article is cited by
-
Second Allogeneic Hematopoietic Stem Cell Transplantation for Hemophagocytic Syndrome with Engraftment Failure
Indian Journal of Hematology and Blood Transfusion (2023)