How is hemophagocytic lymphohistiocytosis (HLH) treated in X-linked lymphoproliferative (XLP) syndrome?

Updated: Jan 27, 2019
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Hemophagocytic lymphohistiocytosis is treated according to standardized protocols (HLH 94 and 2004) based on the use of dexamethasone, etoposide, and cyclosporine, with the addition of intrathecal methotrexate and steroids if there is neurological involvement. This is a highly suppressive regime and can be associated with significant toxicity. The protocol follows different stages, starting with an intense period of treatment initially, with reducing doses of steroids and frequency of etoposide over time if a response is seen. Re-intensification of therapy is occasionally required. [18]

Other immunosuppressive agents have been used to control HLH, either in combination with steroids or as rescue therapy, including ATG (anti-thymocyte globulin) in combination with etoposide and the anti-CD52 antibody alemtuzumab (Campath). In addition, newer biologics are now available, and some are being tested in HLH, including the interleukin-6 receptor agonist tocilizumab (Actemra). [23]  

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