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Hemophagocytic lymphohistiocytosis and related disorders

6 Nov 2006

Abstract excerpt

PURPOSE OF REVIEW: The rate of diagnosis of hemophagocytic lymphohistiocytosis, a genetically heterogeneous and, frequently, rapidly fatal autosomal recessive disorder of immune regulation, is increasing worldwide. Awareness has grown through the Histiocyte Society and the publication of newly-recognized genetic causes. I summarize current knowledge regarding the pathophysiology, diagnosis and treatment of...

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PMID: 17088644
DOI: 10.1097/01.all.0000246626.57118.d9

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