Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment

Figure 1 Diagnostic work up algorithm for a patient with primary hemophagocytic lymphohistiocytosis (familial hemophagocytic lymphohistiocytosis and X-linked lymphoproliferative syndrome)[6,22,23]. Degranulation assay is useful for familial hemophagocytic lymphohistiocytosis (FHL) type 3-5. It is advised to perform flow cytometry of peripheral blood mononuclear cells to detect the expansion of CD8+ T cells as first step of diagnosing hemophagocytic lymphohistiocytosis (HLH) prior to the work up algorism.