Immune disorder in the blood leading to hyperinflammation
Medical condition
Hemophagocytic lymphohistiocytosis
Other names
HLH
Micrograph showing red blood cells within macrophages. H&E stain.
Specialty
Hematology, immunology
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. It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of HLH.
^Fisman, David N. (2000). "Hemophagocytic syndromes and infection". Emerging Infect. Dis.6 (6): 601–8. doi:10.3201/eid0606.000608. PMC 2640913. PMID 11076718.
and 18 Related for: Hemophagocytic lymphohistiocytosis information
hematology, hemophagocyticlymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis (British spelling), and hemophagocytic or haemophagocytic...
pathophysiologically very similar to reactive (secondary) hemophagocyticlymphohistiocytosis (HLH). The incidence of MAS is unknown as there is a wide...
anti-interferon-gamma (IFNγ) antibody medication used for the treatment of hemophagocyticlymphohistiocytosis (HLH), which has no cure. The most common side effects include...
leukemia, neuroblastoma, and ovarian cancer. It is also used for hemophagocyticlymphohistiocytosis. It is used by mouth or injection into a vein. Side effects...
in hemophagocyticlymphohistiocytosis (HLH) there is marked inappropriate and ineffective T cell activation that leads to an increased hemophagocytic activity...
syndromes include familial hemophagocyticlymphohistiocytosis, Epstein-Barr virus–associated hemophagocyticlymphohistiocytosis, systemic or non-systemic...
marrow and other tissues. It is part of the presentation of hemophagocyticlymphohistiocytosis and Macrophage activation syndrome. It has also been seen...
general as well as nasopharyngeal and gastric carcinoma. Hemophagocyticlymphohistiocytosis Once the acute symptoms of an initial infection disappear...
following groups:: 714–724 X-type histiocytoses Non-X histiocytoses Lymphohistiocytosis is a similar immune system disease characterized by the inappropriate...
thrombocytopenia, and autoimmune thyroid disease. Familial hemophagocyticlymphohistiocytosis, an autosomal recessive primary immunodeficiency, is another...
LDH, IL-6, and ferritin. Hemophagocyticlymphohistiocytosis and Epstein-Barr virus-related hemophagocyticlymphohistiocytosis are caused by extreme elevations...
hemosiderosis. Adult-onset Still's disease, some porphyrias, and hemophagocyticlymphohistiocytosis/macrophage activation syndrome are diseases in which the ferritin...
thrombocytopenia and autoimmune thyroid disease. Familial hemophagocyticlymphohistiocytosis, an autosomal recessive primary immunodeficiency, is another...
respectively. NI-0501, an anti-IFNγ antibody, is in development for hemophagocyticlymphohistiocytosis. A phase II/III trial began in 2013 and is ongoing. The FDA...
due to alleged incompatible blood transfusion (Toddler had Hemophagocyticlymphohistiocytosis). Durru Shehvar Children's & General Hospital Nizam's Institute...
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