Pediatric CNS-isolated 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...
متن کاملCNS Involvement in Hemophagocytic Lymphohistiocytosis: CT and MR Findings
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder that is characterized by proliferation of benign histiocytes, and this commonly involves the liver, spleen, lymph nodes, bone marrow and central nervous system (CNS). We report here on the CT and MR imaging findings in a case of CNS HLH that showed multiple ring enhancing masses mimicking abscess or another mass on the CT and MR imaging.
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 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...
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...
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ژورنال
عنوان ژورنال: Neurology - Neuroimmunology Neuroinflammation
سال: 2019
ISSN: 2332-7812
DOI: 10.1212/nxi.0000000000000560