Familial histiocytic reticulosis (familial haemophagocytic reticulosis)

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).

Familial haemophagocytic reticulosis.

Splenectomy for histiocytic medullary reticulosis.

ScoTT & Robb-Smith (1939) introduced the term histiocytic medullary reticulosis (HMR) in 1939 to describe a disorder of the reticulo-endothelial system characterized by fever, wasting and generalized lymphadenopathy, associated with splenic and hepatic enlargement and in the final stages jaundice, purpura and anaemia. Necropsy in their patients revealed an intense erythrophagocytosis by histioc...

Haemophagocytic reticulosis. A state of chimerism?

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. ...

Histiocytic medullary reticulosis in a child.

Scott and Robb-Smith (1939), using the name 'histiocytic medullary reticulosis', reported four cases of a subacute illness characterized by fever, anaemia, leucopenia, jaundice and enlargement of the lymph nodes, spleen and liver. Microscopically there was a proliferation of phagocytes and giant cells in the reticulo-endothelial system, the affected tissues showing a tendency to undergo haemorr...