PURPOSE OF REVIEW Hemophagocytic lymphohistiocytosis (HLH) is a condition of uncontrolled immune activation with a high mortality rate. The recommended therapeutic guideline for HLH was published by the Histiocyte Society in 1994 and revised in 2004, which greatly improved the survival in patients with HLH. However, HLH is still a refractory disease for which the search for novel treatments con...

Hemophagocytic lymphohistiocytosis is a rare condition characterized by highly stimulated but inactive immune response. The disease may be inherited or acquired due to infections, collagen vascular diseases and malignancies. The pathological hallmark of the syndrome is aggressive proliferation of macrophages and histiocytes. Decreased NK cell activity results in increased T cell activation resu...

Macrophage activation syndrome is a form of secondary hemophagocytic lymphohistiocytosis in the setting autoimmune diseases. It’s rare, hyperinflammatory complication requiring prompt institution therapy to prevent organ dysfunction and death. We report young female presenting with macrophage multiorgan failure diffuse alveolar hemorrhage inaugural systemic lupus erythematosus. The patient was ...