Hemophagocytic Lymphohistiocytosis (HLH) – Market Outlook, Epidemiology, Competitive Landscape, and Market Forecast Report – 2023 To 2033
Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome (HPS), is a rare, life-threatening hematologic disorder manifested by clinical findings of extreme inflammation and unregulated immune activation. In both its congenital (primary) and adult (secondary) forms, an underdiagnosed syndrome is caused by the overwhelming dysregulation of the immune system, resulting in end organ damage or even death if untreated. It is described as a hyperinflammatory reaction with uncontrolled, persistent activation of lymphocytes and macrophages engulfing hematopoietic cells, creating a vicious cycle of a cytokine storm. The diagnostic criteria of HLH involve a molecular diagnosis or meeting five of the eight criteria, including fever, splenomegaly, bicytopenia, hypertriglyceridemia and hypofibrinogenemia, hemophagocytosis in the bone marrow, low or absent NK cell activity, elevated ferritin levels and elevated soluble CD25 levels. Hyperferritinemia and elevated soluble CD25 a