Familial Hemophagocytic Lymphohistiocytosis (Fhl) – A Case Series
نویسندگان
چکیده
منابع مشابه
Hemophagocytic Lymphohistiocytosis Case Series
Hemophagocytic Lymphohistiocytosis (HLH) is a rare, lifethreatening syndrome of excessive multisystem inflammation and tissue destruction due to abnormal immune activation. It is more frequently seen in the pediatric population, either as a genetic disorder or secondary to infection or autoimmune disease, but can present in acquired forms at any age. It is thought to be due to an absence of dow...
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Hemophagocytic lymphohistiocytosis is a rare complication of dengue. We present 8 cases of dengue associated hemophagocytic lymphohistiocytosis diagnosed in our hospital during the dengue outbreak of 2012. All the cases were treated with a short (4 weeks) course of steroids along with supportive measures, and showed an excellent response.
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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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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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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...
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ژورنال
عنوان ژورنال: Pediatric Hematology Oncology Journal
سال: 2018
ISSN: 2468-1245
DOI: 10.1016/j.phoj.2018.11.044