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Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory clinical syndrome of uncontrolled immune response which results in hypercytokinemia due to underlying primary or secondary immune defect. A number of genetic defects in transport, processing and function of cytotoxic gr...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis
2015
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4595578/ https://www.ncbi.nlm.nih.gov/pubmed/26457279 http://dx.doi.org/10.5045/br.2015.50.3.131 |
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author | Seo, Jong Jin |
author_facet | Seo, Jong Jin |
author_sort | Seo, Jong Jin |
collection | PubMed |
description | Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory clinical syndrome of uncontrolled immune response which results in hypercytokinemia due to underlying primary or secondary immune defect. A number of genetic defects in transport, processing and function of cytotoxic granules which result in defective granule exocytosis and cytotoxicity of cytotoxic T lymphocytes (CTL) and natural killer (NK) cells have been well identified at the cellular and molecular level. Important advances have been made during the last 20 years in the diagnosis and treatment of HLH. The Histiocyte Society has proposed diagnostic guideline using both clinical and laboratory findings in HLH-2004 protocol, and this has been modified partly in 2009. HLH used to be a fatal disease, but the survival of HLH patients has improved to more than 60% with the use of chemoimmunotherapy combined with hematopoietic cell transplantation (HCT) over the past 2 decades. However, HCT is still the only curative option of treatment for primary HLH and refractory/relapsed HLH after proper chemoimmunotherapy. The outcome of HCT for HLH patients was also improved steadily during last decades, but HCT for HLH still carries significant mortality and morbidity. Moreover, there remain ongoing controversies in various aspects of HCT including indication of HCT, donor selection, timing of HCT, conditioning regimen, and mixed chimerism after HCT. This review summarized the important practical issues which were proven by previous studies on HCT for HLH, and tried to delineate the controversies among them. |
format | Online Article Text |
id | pubmed-4595578 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis |
record_format | MEDLINE/PubMed |
spelling | pubmed-45955782015-10-09 Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies Seo, Jong Jin Blood Res Review Article Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory clinical syndrome of uncontrolled immune response which results in hypercytokinemia due to underlying primary or secondary immune defect. A number of genetic defects in transport, processing and function of cytotoxic granules which result in defective granule exocytosis and cytotoxicity of cytotoxic T lymphocytes (CTL) and natural killer (NK) cells have been well identified at the cellular and molecular level. Important advances have been made during the last 20 years in the diagnosis and treatment of HLH. The Histiocyte Society has proposed diagnostic guideline using both clinical and laboratory findings in HLH-2004 protocol, and this has been modified partly in 2009. HLH used to be a fatal disease, but the survival of HLH patients has improved to more than 60% with the use of chemoimmunotherapy combined with hematopoietic cell transplantation (HCT) over the past 2 decades. However, HCT is still the only curative option of treatment for primary HLH and refractory/relapsed HLH after proper chemoimmunotherapy. The outcome of HCT for HLH patients was also improved steadily during last decades, but HCT for HLH still carries significant mortality and morbidity. Moreover, there remain ongoing controversies in various aspects of HCT including indication of HCT, donor selection, timing of HCT, conditioning regimen, and mixed chimerism after HCT. This review summarized the important practical issues which were proven by previous studies on HCT for HLH, and tried to delineate the controversies among them. Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis 2015-09 2015-09-22 /pmc/articles/PMC4595578/ /pubmed/26457279 http://dx.doi.org/10.5045/br.2015.50.3.131 Text en © 2015 Korean Society of Hematology http://creativecommons.org/licenses/by-nc/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Article Seo, Jong Jin Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies |
title | Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies |
title_full | Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies |
title_fullStr | Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies |
title_full_unstemmed | Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies |
title_short | Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies |
title_sort | hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4595578/ https://www.ncbi.nlm.nih.gov/pubmed/26457279 http://dx.doi.org/10.5045/br.2015.50.3.131 |
work_keys_str_mv | AT seojongjin hematopoieticcelltransplantationforhemophagocyticlymphohistiocytosisrecentadvancesandcontroversies |