Pediatric hemophagocytic lymphohistiocytosis
نویسندگان
چکیده
منابع مشابه
PEDIATRIC HEMATOLOGY Hemophagocytic lymphohistiocytosis
HLH was first described by Farquhar and Claireaux in 1952 as a familial disease [1]. Interestingly in their cases hemophagocytosis, which has given the disease its name, could not be found during lifetime but was prominent on autopsy. Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive disorder with an estimated frequency of 0.12/100 000 children per year [2]. Several g...
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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...
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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...
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 in adults
Dear Sir, I read with interest a recent article by Antonodimitrakis and colleagues in your journal presenting a case of acquired hemophagocytic lymphohistiocytosis (HLH) in a 60-year-old woman suffering from diabetes mellitus type 2 (1). The reported patient developed a life-threatening HLH associated with a reactivation of an Epstein–Barr virus (EBV) infection and was successfully treated by m...
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ژورنال
عنوان ژورنال: Blood
سال: 2020
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.2019000936