Genetic Deficiency of Interferon‐γ Reveals Interferon‐γ–Independent Manifestations of Murine Hemophagocytic Lymphohistiocytosis
نویسندگان
چکیده
منابع مشابه
Biology and Treatment of Hemophagocytic Lymphohistiocytosis
Hemophagocytic lymphohistocytosis (HLH) is a hyperinflammatory syndrome that occurs at all ages and is characterized by high levels of cytokines, secreted by activated T-lymphocytes and macrophages. All symptoms and laboratory changes can be explained by organ infiltration by these cells and hypercytokinemia. HLH occurs as an inherited form (genetic, primary HLH) with mutations primarily in the...
متن کاملTreatment of hemophagocytic lymphohistiocytosis.
PURPOSE OF REVIEW Hemophagocytic lymphohistiocytosis (HLH) is a condition of uncontrolled immune activation with a high mortality rate. The recommended therapeutic guideline for HLH was published by the Histiocyte Society in 1994 and revised in 2004, which greatly improved the survival in patients with HLH. However, HLH is still a refractory disease for which the search for novel treatments con...
متن کاملHemophagocytic Lymphohistiocytosis
To investigate the clinical features of adult patients with hemophagocytic lymphohistiocytosis (HLH) and to explore possible risk factors for death, we retrospectively reviewed the medical records of 103 adult HLH patients hospitalized from 1997 to 2012. We analyzed the underlying diseases, clinical characteristics, 1aboratory findings, outcomes, and prognostic factors. The most common cause of...
متن کاملGenetic analysis of B-cell lymphomas associated with hemophagocytic lymphohistiocytosis.
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. HLH clinically presents with fever, pancytopenia, splenomegaly, and hemophagocytosis in the bone marrow, lymph nodes, or liver. A proposed mechanism for HLH is a paradoxical downregulation of various aspects of the immune response, including B-cell development and function, Toll-...
متن کاملLymphoma-associated hemophagocytic lymphohistiocytosis.
A 34-year-old male with a history of T cell–rich B-cell lymphoma relapsed 1 year after high-dose therapy with recurrent fevers, abdominal pain, and jaundice. He was cachetic with hepatosplenomegaly and ascites. Laboratory findings showed a white blood cell count of 2.7 109/L, hemoglobin 8.7 g/dL, platelets of 21 109/L, hyperbilirubinemia, and no evidence of hemolysis including a negative Coombs...
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ژورنال
عنوان ژورنال: Arthritis & Rheumatology
سال: 2019
ISSN: 2326-5191,2326-5205
DOI: 10.1002/art.41076