Familial haemophagocytic reticulosis. Report of 2 cases in sibs
- PMID: 1154199
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Familial haemophagocytic reticulosis. Report of 2 cases in sibs
S Afr Med J.
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Abstract
Two cases of familial haemophagocytic reticulosis occurring in sibs are described. The disease is characterised by a widespread proliferation of histocytes showing haemophagocytosis and it usually manifests clinically with haematological abnormalities. The initial presentation may be that of a meningo-encephalitic illness, the result of a lymphohistiocytic infiltration of the meninges and cerebral tissues. The clinical course and pathological features are described and the differential diagnosis is discussed.
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