Er is op dit moment geen behandelrichtlijn voor HLH.


  1. Emile JF et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016; 127(22): 2672-2681. doi: 10.1182/blood-2016-01-690636
  2. Henter JI et al. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. Medical and pediatric oncology 1997; 28(5): 342-347.
  3. Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood 2015; 125(19): 2908-2914. doi: 10.1182/blood-2015-01-551622
  4. Brisse E, et al. Understanding the spectrum of haemophagocytic lymphohistiocytosis: update on diagnostic challenges and therapeutic options. British journal of haematology 2016; 174(2): 175-187. doi: 10.1111/bjh.14144
  5. Brisse E et al. Advances in the pathogenesis of primary and secondary haemophagocytic lymphohistiocytosis: differences and similarities. British journal of haematology 2016; 174(2): 203-217. doi: 10.1111/bjh.14147
  6. Schram AM et al. Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years. British journal of haematology 2016; 172(3): 412-419. doi: 10.1111/bjh.13837
  7. Otrock ZK et al. Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. American journal of hematology 2014. e-pub ahead of print 2014/12/04; doi: 10.1002/ajh.23911
  8. Schram AM et al. Marked hyperferritinemia does not predict for HLH in the adult population. Blood 2015; 125(10): 1548-1552. doi: 10.1182/blood-2014-10-602607
  9. Schulert GS, Grom AA. Pathogenesis of macrophage activation syndrome and potential for cytokine- directed therapies. Annual review of medicine 2015; 66: 145-159. doi: 10.1146/annurev-med-061813-012806
  10. Chellapandian D et al. Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens. Br J Haematol 2013; 162(3): 376-382. e-pub ahead of print 2013/05/23; doi: 10.1111/bjh.12386
  11. Das R et al. Janus kinase inhibition lessens inflammation and ameliorates disease in murine models of hemophagocytic lymphohistiocytosis. Blood 2016; 127(13): 1666-1675. doi: 10.1182/blood-2015-12-684399
  12. Imashuku S. Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH); update 2010. Journal of pediatric hematology/oncology 2011; 33(1): 35-39. e-pub ahead of print 2010/11/23; doi: 10.1097/MPH.0b013e3181f84a52
  13. Jordan MB, Filipovich AH. Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: a journey of a thousand miles begins with a single (big) step. Bone marrow transplantation 2008; 42(7): 433-437. doi: 10.1038/bmt.2008.232
  14. La Rosee P. Treatment of hemophagocytic lymphohistiocytosis in adults. Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program 2015; 2015(1): 190-196. doi: 10.1182/asheducation-2015.1.190
  15. La Rosee P. Alleviating the storm: ruxolitinib in HLH. Blood 2016; 127(13): 1626-1627. doi: 10.1182/blood-2016-02-697151
  16. Maschalidi S et al. Therapeutic effect of JAK1/2 blockade on the manifestations of hemophagocytic lymphohistiocytosis in mice. Blood 2016; 128(1): 60-71. doi: 10.1182/blood-2016-02-700013
  17. Seo JJ. Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies. Blood research 2015; 50(3): 131-139. doi: 10.5045/br.2015.50.3.131


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