Management of Adult Onset Familial Hemophagocytic Lymphohistiocytosis (FHL): A Dilemma
نویسندگان
چکیده
منابع مشابه
First characterization of platelet secretion defect in patients with familial hemophagocytic lymphohistiocytosis type 3 (FHL-3).
Recent major improvement in long-term survival of younger patients with multiple myeloma. First characterization of platelet secretion defect in patients with familial hemophagocytic lymphohistiocytosis type 3 (FHL-3) Familial hemophagocytic lymphohistiocytosis (FHL), a rare auto-somal recessive disorder of lymphocyte cytotoxicity, is caused by mutations in genes encoding perforin (FHL-2) or pr...
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Wolman disease; Familial hemophagocytic lymphohistiocytosis; Hepatomegaly; Splenomegaly; Fever Abstract Background: Familial hemophagocytic lymphohistiocytosis is a rare autosomal recessive disease that is usually evident in the first few months or years of life. Major signs and symptoms include hepatomegaly, splenomegaly, anemia, leucopenia or thrombocytopenias which resemble many inborn error...
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BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome with both genetic and acquired causes characterized by elevated cytokine levels, hyperinflammation, and overactivation of lymphocytes and macrophages. It is typically a systemic disease with variable degrees of CNS involvement. Cases with predominantly central nervous system (CNS) involvement are very rare, with the vast...
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similar to that reported by Falini, and was associated with a higher proportion of normal karyotype (93% vs 60%; P .001), lower leukocyte count at diagnosis (32 109/L vs 69 109/L; P .01), and lower bone marrow infiltration (51% vs 72% blast cells, P .001). Interestingly, the frequency of NPM1 and FLT3 internal tandem duplication (FLT3-ITD) mutations did not differ between patients with and with...
متن کاملHypercytokinemia in familial hemophagocytic lymphohistiocytosis.
Familial hemophagocytic lymphohistiocytosis (FHL) is a frequently missed and almost uniformly fatal childhood disorder. It is characterized by fever, hepatosplenomegaly, cytopenia, coagulopathy, and hypertriglyceridemia. The pathogenesis of FHL is not known but the above clinical and laboratory findings are compatible with reported in vitro and in vivo effects of several inflammatory cytokines....
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ژورنال
عنوان ژورنال: Haematology International Journal
سال: 2018
ISSN: 2578-501X
DOI: 10.23880/hij-16000124