Abstract
An autopsy case of histiocytic medullary reticulosis (HMR) in a seventeen-old girl, clinically characterized by fever, jaundice, hemorrhagic diathesis, hepatosplenomegaly, impaired liver function and pancytopenia, was described. A small number of atypical cells, some of which showed erythrophagocytosis, were seen in the smears of peripheral blood and bone marrow. Electron microscopic examination of these cells allowed for their categorization as histiocytes of varying maturity, because of their overt phagocytotic properties and of the presence of pseudopodal extensions, pinocytotic vesicles and phagocytotic vacuoles. She went very rapidly downhill to death eleven days after onset.
The pathological examination showed generalized proliferation of histiocytic series in the lymph nodes, liver, spleen and bone marrow. Essentially medullary proliferations of these cells in lymph nodes was noted as a characteristic topographical picture. The nature of the case was considered as a malignant neoplastic disease because of the marked proliferation of atypical cells and of the absence of old infectious focus. Pertinent literatures were reviewed mainly from the histopathological point of view. The characteristic histopathological appearances of HMR and the nosological differentiation of presented case from the other neoplastic diseases of reticuloendothelial system were discussed.
