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Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q21.3-22 by homozygosity mapping.
Am J Hum Genet
January 1999
Department of Haematological Medicine, King's College School of Medicine and Dentistry, Denmark Hill, London SE5 9PJ, United Kingdom.
Familial hemophagocytic lymphohistiocytosis (FHL), also known as familial erythrophagocytic lymphohistiocytosis and familial histiocytic reticulosis, is a rare autosomal recessive disorder of early childhood characterized by excessive immune activation. Linkage of the disease gene to an approximately 7.8-cM region between markers D9S1867 and D9S1790 at 9q21.
View Article and Find Full Text PDFFamilial hemophagocytic lymphohistiocytosis (FHLH).
Pathology
February 1997
Department of Laboratory Medicine and Pathology, UMDNJ-New Jersey Medical School, USA.
Clinical course and histopathologic features of a typical case of familial hemophagocytic lymphohistiocytosis (FHLH) are presented. FHLH, initially known as familial hemophagocytic reticulosis (FHR), is rare and without proper treatment is invariably rapidly fatal, usually accompanied by fever, anorexia, vomiting, irritability and pallor. Sporadic examples with prolonged survival have been reported.
View Article and Find Full Text PDFAngiotropic B-cell lymphoma with hemophagocytic syndrome.
Pathol Res Pract
August 1994
Department of Pathology, Kanazawa University School of Medicine, Japan.
A unique autopsy case of angiotropic lymphoma, which is a rare lymphoma characterized by the lymphoma cells confined in blood vessels and with a minimal involvement of lymph nodes, is presented. The clinical diagnosis was a malignant histiocytosis (histiocytic medullary reticulosis), since clinical manifestations were muscle weakness of the extremities, pancytopenia, fever and hepatosplenonephromegaly, and biopsy exhibited infiltration by histiocytic cells showing hemophagocytosis in the liver sinusoids and the bone marrow, the latter with myelofibrosis. Postmortem examination revealed lymphoma cell proliferation within small blood vessels of the kidneys, perineural tissue of extremities and lungs.
View Article and Find Full Text PDFHematophagic histiocytosis: a clinicopathologic analysis of 23 cases with special reference to the association with peripheral T-cell lymphoma.
J Formos Med Assoc
May 1994
Department of Internal Medicine, National Taiwan University Hospital, Taipei, R.O.C.
The clinicopathologic features of 23 patients with hematophagic histiocytosis (HH) are described. All of them exhibited increased histiocytes associated with hemophagocytosis in the marrow. The patients usually presented with fever, hepatosplenomegaly, lymphadenopathy, and cytopenia.
View Article and Find Full Text PDFCutaneous lesions of hemophagocytic syndrome in a patient with T-cell lymphoma and active Epstein-Barr infection.
J Am Acad Dermatol
November 1991
Walter Reed Army Institute of Research, Washington, DC.
The hemophagocytic syndrome (HPS) was first described in immune-compromised patients with secondary viral infections. Immunodeficiency has not always been diagnosed before the onset of HPS, but most patients who develop HPS have known immune deficits. HPS has also been reported in association with lymphoreticular malignancies.
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