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. 1975 Jul 26;49(32):1305-8.

Familial haemophagocytic reticulosis. Report of 2 cases in sibs

J B Botha, L B Kahn, R O Kaschula
  • PMID: 1154199

Familial haemophagocytic reticulosis. Report of 2 cases in sibs

J B Botha et al. S Afr Med J. .

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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