How i treat primary haemophagocytic lymphohistiocytosis

British Journal of Haematology
Rebecca A Marsh, Elie Haddad

Abstract

Primary haemophagocytic lymphohistiocytosis (HLH) diseases are a collection of inherited genetic disorders that cause the syndrome of HLH. Great advances have been made in the last 20 years with regard to the discovery of many of the genetic aetiologies of disease. Several advances have also been made on the clinical stage. Accurate screening diagnostics for primary HLH diseases that are superior to traditional Natural Killer cell function testing have been developed and are now available in many countries. There is now grounded clinical experience on which to base routine treatment decisions for patients with HLH. Newer approaches to allogeneic haematopoietic cell transplantation have increased overall patient survival. Despite these advances, however, there is still much work to be done to further improve patient care. This 'How I Treat' article will focus on summarizing current diagnostic, treatment and transplant strategies for patients with primary HLH diseases.

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Citations

Nov 22, 2018·The Pediatric Infectious Disease Journal·Emily ChesshyreMarion R Roderick
Apr 7, 2019·Journal of Clinical Immunology·Thomas F MichniackiMark T Vander Lugt
May 7, 2020·The New England Journal of Medicine·Franco LocatelliCristina de Min
Oct 16, 2018·Frontiers in Immunology·Julie BarsalouFabien Touzot
Aug 10, 2019·Frontiers in Immunology·Samuel Cern Cher ChiangRebecca A Marsh
Nov 12, 2020·Journal of Medical Case Reports·Linn Hereide TrovikHåkon Reikvam
Dec 19, 2020·British Journal of Haematology·Thomas A Fox, Claire Booth
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Feb 10, 2021·Blood Reviews·James T EnglandLuke Y C Chen
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Jun 13, 2021·International Journal of Laboratory Hematology·Akriti GeraSumit Mehndiratta
Jul 5, 2021·The Journal of Allergy and Clinical Immunology. in Practice·Camille BeaufilsFabien Touzot

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