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Hemophagocytic Lymphohistiocytosis After Initiation of Chemotherapy for Bilateral Adrenal Neuroblastoma.
J Pediatr Hematol Oncol
January 2016
*Department of Medicine, Toronto Western Hospital, University of Toronto, Toronto, ON, Canada †Department of Pediatrics, Pequeno Príncipe Hospital ‡Department of Pediatrics, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and aggressive syndrome characterized by overactivation of the immune system. Although secondary HLH has been frequently associated with malignancies, this entity is rarely triggered by solid tumors, such as neuroblastomas. Herein, we describe a 14-month-old girl with a late diagnosis of bilateral adrenal neuroblastoma who developed HLH 6 days after the initiation of chemotherapy.
View Article and Find Full Text PDFClonal CD8+ T Lymphocytic Proliferation and Karyotypical Abnormalities in an EBV Associated Hemophagocytic Lymphohistiocytosis.
Case Rep Pathol
October 2015
Department of Pathology and Laboratory Medicine, Indiana University, Indianapolis, IN 46202, USA.
EBV associated hemophagocytic lymphohistiocytosis and EBV-positive T cell lymphoproliferative disease of childhood share many histologic and clinical features, which sometimes makes it very difficult to render a definitive diagnosis. In this report, we present a 16-year-old male who developed symptoms clinically consistent with EBV associated hematophagocytic lymphohistiocytosis including fulfilling most of HLH diagnostic criteria and responding promptly to HLH targeted therapy. However, histologic and cytogenetics features of this case are very concerning for EBV-positive T cell lymphoproliferative disease of childhood.
View Article and Find Full Text PDFFamilial hemophagocytic lymphohistiocytosis in a 6-week-old male infant.
Coll Antropol
June 2010
Department of Hematology and Oncology, Pediatric Clinic, Zagreb Children's Hospital, Zagreb, Croatia.
Familial hemophagocytic lymphohistiocytosis (FLH) is an autosomal recessively inherited multisystem disease. This defect in cellular cytotoxicity is a life threatening condition characterized by fever, rash, splenomegaly, cytopenias and neurologic manifestations. PRF1, UNC13D and STX11 gene defects underlie in about 40-50% of primary cases.
View Article and Find Full Text PDFPerforin and the granule exocytosis cytotoxicity pathway.
Curr Opin Immunol
October 2003
Experimental Immunology Branch, National Cancer Institute, National Institutes of Health, Building 10, Room 4B36, 9000 Rockville Pike, Bethesda, MD 20892-1360, USA.
Perforin defects have been identified in humans with familial hematophagocytic lymphohistiocytosis. The pathology of these patients has dramatically illustrated an under-appreciated role for perforin in the regulation of T-cell responses in vivo, and experimental studies are shedding light on the mechanisms involved. The detailed molecular mechanisms of perforin's mandatory role in the cytotoxic T lymphocyte (CTL)-mediated granule exocytosis death pathway and of granzyme entry into target cells remain unclear.
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