The DOI matches its Crossref record; 151 of the 151 references it cites were found in a registry or an index.
Hemophagocytic lymphohistiocytosis: review of etiologies and management
Open accessAbstract
Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection. Familial forms result from genetic defects in natural killer cells and cytotoxic T-cells, typically affecting perforin and intracellular vesicles. HLH is likely under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy to be made. Current treatment regimens include immunosuppression, immune modulation, chemotherapy, and biological response modification, followed by hematopoietic stem-cell transplant (bone marrow transplant). A number of recent studies have contributed to the understanding of HLH pathophysiology, leading to alternate treatment options; however, much work remains to raise awareness and improve the high morbidity and mortality of these complex conditions.
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Cites
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Cited by
Karki, R., Sharma, B. R., Tuladhar, S., Williams, E. P., Zalduondo, L., Samir, P., Zheng, M., Sundaram, B., Banoth, B., Malireddi, R. K. S., et al. (2021) · Cell · doi:10.1016/j.cell.2020.11.025 · Cited by 1,734
Otrock, Z. K., & Eby, C. S. (2015) · American Journal of Hematology · doi:10.1002/ajh.23911 · Cited by 338
Brisse, E., Wouters, C. H., & Matthys, P. (2016) · British Journal of Haematology · doi:10.1111/bjh.14147 · Cited by 197
Metadata
- Authors
- George, M.
- Venue
- Journal of Blood Medicine
- Publisher
- Informa UK Limited
- Published
- 2014-06
- Volume
- 5
- Pages
- 69
- ISSN
- 1179-2736 (ISSN-L)
- OpenAlex
- W2063048890
- Cited by
- 488
- References
- 151
- FWCI
- 14.29
- Type
- journal article
- Language
- en
- Open access
- gold
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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