References in periodicals archive ?
The team are now planning a clinical trial to test the safety and activity of the antibody drug in people with HLH and MAS.
HLH can occur as a primary genetic disorder or a secondary consequence of another medical condition, including infection (9).
Studies demonstrate that CNS involvement, i.e., neurological symptoms, abnormal cranial magnetic resonance imaging (MRI), and/or cerebrospinal fluid (CSF), is indicators for a poor prognosis in HLH patients. However, in some HLH patients, CNS impairment is the first and only symptom resulting in difficulties and delays for the diagnosis.
The approval for the product is for paediatric, both new born and older, and adult HLH patients whose condition is refractory, recurrent or progressive or are intolerant to conventional HLH therapy.
Pathology report of her skin biopsy was in accordance with HLH.
The patient met diagnostic criteria for HLH, including fever, splenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis in bone marrow, and elevated ferritin.
As her condition deteriorated doctors diagnosed Layla with hemophagocytic lymphohistiocytosis (HLH), an immunodeficiency disease which, it is estimated, affects just one in 830,000 children a year.
Thereby, he was provisionally diagnosed with severe sepsis/septic shock - Salmonella-induced, probably hepatitis B-induced acute liver failure (ALF), hemophagocytic lymphohistiocytosis (HLH) (score five out of eight according to 2004 HLH guidelines), and severe coagulopathy.
Haemophagocytic lymphohistiocytosis (HLH) is a rare disease with a sepsis-like progression that leads to multiple organ dysfunction syndrome, especially in preterm infants.
Macrophage activation syndrome (MAS), also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and potentially fatal complication of several autoimmune diseases, including juvenile idiopathic arthritis and SLE.
Although most patients with infectious mononucleosis (IM) recover without major clinical sequelae, a number of harmful complications can occur, including hepatitis, splenic rupture, and hematological abnormalities ; among the latter, the hemophagocytic lymphohistiocytosis (HLH) is particularly harmful because it is characterized by the massive production and release of inflammatory mediators caused by the uncontrolled activation of T-lymphocytes, NK-cells, and macrophages; the ensuing clinical picture resembles septic shock and can determine a multiple organ dysfunction syndrome (MODS) [2-4].
Therefore, pathogenicity is unchecked, leading to uncontrolled lymphoproliferation and severe manifestations including fulminant infectious mononucleosis and hemophagocytic lymphohistiocytosis (HLH).
Acronyms browser ?
Full browser ?
- HLH (Hemophagocytic lymphohistiocytosis)