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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.
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.
This regimen is currently recommended, particularly for primary HLH in young children, as induction therapy for EBV+ HLH except in patients with the macrophage activation syndrome where pulse methylprednisolone is the preferred treatment. Response rates are somewhat higher in young children than adults and in primary rather than secondary disease.
X-linked lymphoproliferative disease; Other names: Duncan disease, Purtilo syndrome: Specialty: Hematology : Symptoms: Reduced resistance to the Epstein-Barr virus (EBV), leading to infectious mononucleosis, hemophagocytic lymphohistiocytosis (HLH), dysgammaglobulinemia, non-Hodgkin lymphoma, aplastic anemia, vasculitis, chronic gastritis, skin lesions
There are several forms of Epstein–Barr virus (EBV) infection. These include asymptomatic infections, the primary infection, infectious mononucleosis, and the progression of asymptomatic or primary infections to: 1) any one of various Epstein–Barr virus-associated lymphoproliferative diseases such as chronic active EBV infection, EBV+ hemophagocytic lymphohistiocytosis, Burkitt's lymphoma ...
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.
Enjoy a classic game of Hearts and watch out for the Queen of Spades!
Emapalumab, sold under the brand name Gamifant, is an anti-interferon-gamma (IFNγ) antibody medication used for the treatment of hemophagocytic lymphohistiocytosis (HLH), [3] [4] [5] which has no cure. [6] The most common side effects include infections, hypertension, infusion-related reactions, and pyrexia. [3]