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International Urology and Nephrology
Erschienen in: International Urology and Nephrology 8/2016

20.04.2016 | Nephrology - Review

Hemophagocytic lymphohistiocytosis: an update for nephrologists

verfasst von: Edward J. Filippone, John L. Farber

Erschienen in: International Urology and Nephrology | Ausgabe 8/2016

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed.
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Metadaten
Titel
Hemophagocytic lymphohistiocytosis: an update for nephrologists
verfasst von
Edward J. Filippone
John L. Farber
Publikationsdatum
20.04.2016
Verlag
Springer Netherlands
Erschienen in
International Urology and Nephrology / Ausgabe 8/2016
Print ISSN: 0301-1623
Elektronische ISSN: 1573-2584
DOI
https://doi.org/10.1007/s11255-016-1294-z

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