Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a type of severe systemic inflammatory syndrome. HLH occurs when histiocytes and lymphocytes become overactive and mediate attack on body tissues rather than only on microbes or cancer cells. When histiocytes and lymphocytes are overactive, they produce excess of cytokines, which lead to a “cytokine storm”. These over-reactive cells attack normal cells of the body like blood cells and bone marrow cells, as well as the spleen, lymph nodes, skin, liver and brain. Many genes have been identified whose defect leads to primary HLH. These genes include PRF1, STXBP2, UNC13D, STX11, RAB27A, SH2D1A, LYST, AP3B1 and XIAP/BIRC4. When the HLH occurs because of some severe illness like viral infection or malignancy then it is termed as secondary HLH. These diseases trigger the inflammatory response which cannot be shut off. Treatments for HLH include immunosuppressants and anti-inflammatory agents such as glucocorticoids and etoposide. A hematopoietic stem cell transplant (SCT) is the only permanent cure for primary HLH.
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Sharma, S.K. (2023). Hemophagocytic Lymphohistiocytosis. In: Basics of Hematopoietic Stem Cell Transplant. Springer, Singapore. https://doi.org/10.1007/978-981-19-5802-1_57
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DOI: https://doi.org/10.1007/978-981-19-5802-1_57
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