Histiocyte Society. Hemophagocytic Lymphohistiocytosis Study Group – Treatment Protocol of the Second International HLH Study 2004.
The term histiocytoses identifies a group of disorders that have in common the proliferation of cells of the mononuclear phagocyte system, with the histiocyte as a central cell. The histiocytic disorders are, at present, in brief classified as follows (1):
Disorders of Varied Biological Behavior
– Dendritic cell-related disorders (incl Langerhans cell histiocytosis);
– Macrophage-related disorders Malignant Disorders;
Hemophagocytic lymphohistiocytosis (HLH) includes the great majority of patients with macrophage-related disorders (1-5). HLH comprises two different conditions that may be difficult to distinguish from each other (4-7)
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