Hemophagocytic lymphohistiocytosis (hlh), a heterogeneous spectrum of cytokine-driven immune disorders

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder that was first described by Scott and Robb-Smith in 1939 [1]. It is characterized by severe systemic inflammation caused by uncontrolled proliferation and activation of lymphocytes and macrophages, secreting large amounts of cytokines, creating a so-called cytokine storm. The hallmark of HLH is the emergence of hemophagocytosis, a process in which histiocytes actively engulf blood cells and their precursors. Table 1 provides a comprehensive overview of symptoms that are most commonly observed in HLH [2–4], together with their application in the current diagnostic criteria [5].
Source: Cytokine and Growth Factor Reviews - Category: Molecular Biology Authors: Tags: Survey Source Type: research