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History of Hemophagocytic Lymphohistiocytosis

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Cytokine Storm Syndrome

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a severe cytokine storm syndrome (CSS) which until the turn of the century was barely known but is now receiving increased attention. The history of HLH dates back to 1939 when it was first described in adults, followed by the first description of its primary, familial form in children in 1952. Secondary forms of HLH are far more frequent and occur with infections, malignancies, metabolic diseases, iatrogenic immune suppression, and autoinflammatory/autoimmune diseases. Identification of genetic defects leading to defective function of natural killer cells and cytotoxic T cells, as well as corresponding mouse models, have revolutionized our understanding of HLH and of immune function. Diagnosis relies on clinical and laboratory criteria; functional and genetic tests can help separate primary forms from secondary forms. Treatment with immunochemotherapy and hematopoietic stem cell transplantation has considerably improved survival in children with primary HLH, a formerly uniformly fatal disease.

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Authors and Affiliations

  1. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg, Hamburg, Germany

    Gritta E. Janka

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  1. UAB School of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA

    Randy Q. Cron

  2. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Edward M. Behrens

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Janka, G.E. (2019). History of Hemophagocytic Lymphohistiocytosis. In: Cron, R., Behrens, E. (eds) Cytokine Storm Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-22094-5_1

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