Efficacy and safety of allogeneic hematopoietic stem cell transplant in adults with hemophagocytic lymphohistiocytosis: a systematic review of literature

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...

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Bibliographic Details
Published in:Bone marrow transplantation (Basingstoke) 2022-06, Vol.57 (6), p.866-873
Main Authors: Masood, Adeel, Wahab, Ahsan, Iqbal, Qamar, Davis, James, Ehsan, Hamid, Hashmi, Hamza
Format: Article
Language:English
Subjects:
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Adolescent
Adolescents
Adult
Adults
Autoimmune diseases
Bone marrow
Cell Biology
Chemotherapy
Child
Children
Clinical trials
Cytokines
Disease
Hematology
Hematopoietic Stem Cell Transplantation
Hematopoietic stem cells
Histiocytosis
Humans
Immune system
Infections
Internal Medicine
Literature reviews
Lymphatic diseases
Lymphocytosis
Lymphohistiocytosis, Hemophagocytic - drug therapy
Malignancy
Medicine
Medicine & Public Health
Mutation
Neoplasms - complications
Oncology
Patients
Pediatrics
Public Health
Retrospective Studies
Review Article
Safety
Stem cell transplantation
Stem Cells
Systematic review
Teenagers
Viral infections
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Description
Summary: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.
ISSN:0268-3369
1476-5365
1476-5365
DOI:10.1038/s41409-022-01668-9