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      Hemophagocytic syndromes--an update.

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          Abstract

          Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome and not an independent disease. HLH represents the extreme end of a severe uncontrolled hyperinflammatory reaction that can occur in many underlying conditions. Genetic forms of HLHs are due to defects in transport, processing and function of cytotoxic granules in natural killer cells and cytotoxic T lymphocytes, and are not restricted to manifestation in childhood. Acquired forms of HLH are encountered in infections, autoinflammatory and autoimmune diseases, malignancies, acquired immune deficiency. Functional tests allow for differentiation between genetic and acquired HLH. Treatment aims at suppressing hypercytokinemia and eliminating activated and infected cells. It includes immunomodulatory and immunosuppressive agents, cytostatics, T-cell and cytokine antibodies. In genetic HLH cure can only be achieved with hematopoietic stem cell transplantation. Reduced-intensity conditioning regimens have considerably improved survival.

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          Author and article information

          Journal
          Blood Rev.
          Blood reviews
          1532-1681
          0268-960X
          Jul 2014
          : 28
          : 4
          Affiliations
          [1 ] Pediatric Hematology and Oncology, University Medical Center Eppendorf, Hamburg, Germany. Electronic address: janka@uke.uni-hamburg.de.
          [2 ] Pediatric Hematology and Oncology, University Medical Center Eppendorf, Hamburg, Germany.
          Article
          S0268-960X(14)00028-9
          10.1016/j.blre.2014.03.002
          24792320
          f1ca3cd0-d100-421d-ab47-658762bcde16
          Copyright © 2014 Elsevier Ltd. All rights reserved.
          History

          Adult HLH,Hemophagocytic lymphohistiocytosis,Immune deficiency,Macrophage activation syndrome,Reduced-intensity conditioning,Stem cell transplantation

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