Familial haemophagocytic reticulosis.
نویسندگان
چکیده
منابع مشابه
Familial histiocytic reticulosis (familial haemophagocytic reticulosis).
The clinical and pathological findings are recorded in two siblings who died in early infancy from familial histiocytic (;haemophagocytic') reticulosis. The nature of this condition is obscure but probably represents a primary histiocytosis. The only other family on record is that described by Farquhar and Claireaux (1952) and by Farquhar, Macgregor, and Richmond (1958).
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Haemophagocytic reticulosis is characterized by pancytopenia and an increased mass of reticuloendothelial cells in various organs containing engulfed blood cells (see Buist, Jones, and Cavens, 1971; Oehmichen, Narita, and Roloff, 1972; Nezelof and Eliachar, 1973). The pathogenesis is obscure. Autoimmunity, primary histiocytic proliferation, and a graft versus host reaction have been suggested. ...
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A 15-year-old male developed features of Still's disease. He was treated, with incomplete response, with aspirin and corticosteroids. Some 10 months after the onset he contracted chickenpox and became gravely ill. A diagnosis of histiocytic medullary reticulosis was made because erythrophagocytic histiocytes were detected in marrow aspirate films. Necropsy studies showed systemic infiltration b...
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Familial haemophagocytic lymphohistiocytosis is a rare but invariably fatal disease without haematopoietic stem cell transplantation. Genetic defect identification is useful for confirming a clinical diagnosis, predicting the risk of future recurrence, and defining haemophagocytic lymphohistiocytosis predisposition in asymptomatic family members. Notably, familial haemophagocytic lymphohistiocy...
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In 1832 Thomas Hodgkin described ‘some morbid appearances of the absorbent glands and spleen’ in seven individuals with enlargements of lymph nodes and spleen without inflammation or other significant pathological findings. His initial description resolved into a multiplicity of pathological entities over the years. Indeed, among this wide group of disorders, the leucose, syphilitic and tubercu...
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 43 231 شماره
صفحات -
تاریخ انتشار 1952