HISTIOCYTIC MEDULLARY RETICULOSIS

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Abstract
Histiocytic medullary reticulosis is a rare disease which is characterized by an infiltration of the lymphoreticular tissues by actively phagocytic histiocytes and their precursors. The dominant clinical features of this disorder are progressive splenic enlargement and pancytopenia with relatively severe neutropenia. All forms of therapy so far reported have been ineffective, death occurring in the majority of cases approximately six months after the onset of the disease. The diagnosis of histiocytic medullary reticulosis is a histological one, although the clinical features, peripheral blood count and radioisotopic studies may be highly suggestive. Two cases of histiocytic medullary reticulosis are reported in this paper. They illustrate the classical clinical, hæmatological and pathological features of the disease, but differ markedly from the majority of the reported cases in the patients' relatively long survival and their dramatic and sustained improvement following splenectomy.

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