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In hematology, hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis (British spelling), and hemophagocytic or haemophagocytic syndrome, [1] is an uncommon hematologic disorder seen more often in children than in adults.
Lymphohistiocytosis is a similar immune system disease characterized by the inappropriate activation of natural killer cells, CD8+ cytotoxic T-cells, and macrophages, involving principally the liver, spleen and central nervous system and associated with severe lymphoid atrophy. [9] [10]
Cytokine storm syndromes include familial hemophagocytic lymphohistiocytosis, Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis, systemic or non-systemic juvenile idiopathic arthritis–associated macrophage activation syndrome, NLRC4 macrophage activation syndrome, cytokine release syndrome and sepsis. [6]
Micrograph showing hemophagocytosis in the spleen. H&E stain.. Hemophagocytosis is a dangerous form of phagocytosis in which histiocytes engulf red blood cells, white blood cells, platelets, and their precursors [1] in bone marrow and other tissues.
Lymphoproliferative disorders are a set of disorders characterized by the abnormal proliferation of lymphocytes into a monoclonal lymphocytosis.The two major types of lymphocytes are B cells and T cells, which are derived from pluripotent hematopoietic stem cells in the bone marrow.
It is thought to be closely related and pathophysiologically very similar to reactive (secondary) hemophagocytic lymphohistiocytosis (HLH). [1] The incidence of MAS is unknown as there is a wide spectrum of clinical manifestations, and episodes may remain unrecognized.
Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or sometimes as Destombes–Rosai–Dorfman disease, [1] [2] [3] is a rare disorder of unknown cause that is characterized by abundant histiocytes in lymph nodes or other locations including the skin, sinuses, brain and heart.
Erdheim–Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis).