Abstract
The 8-year-old boy had been repeatedly hospitalized since November 1968 because of intermittent high fever, associated with exanthem. Every admission failed to establish a definite diagnosis and to improve clinical symptoms inspite of steroid administration. To evaluate further his disease, he was admitted to Nagoya University Hospital in October 1973. Hepatomegaly, splenomegaly, moderate hepatic dysfunction and leukocytosis (11,300/cmm) including 48% atypical lymphoid cells without anemia or thrombocytopenia were the remarkable findings in physical and laboratory examinations. On May 20 1974, splenectomy and liver biopsy were performed because of rapidly increasing splenomegaly and development of pancytopenia. The portal pressure was normal. The histology of the spleen showed atrophy of the white pulp and infiltration of lymphoid cells as well as of histiocytes, plasmacytoid cells and “reticulum cells” mainly in the red pulp, with erythrophagocytosis in some of the histiocytes. Liver biopsy also revealed diffuse scattering of mainly lymphoid cells in the sinusoids and portal areas. Soon after the surgery, high fever developed and persisted. Liver was found to enlarge rapidly, accompanied by generalized exanthem. At that time, atypical lymphoid cells were increased gradually in the peripheral blood as well as in the bone marrow. Cytological studies including electron-microscopy and phase contrast microscopy were done. The skin biopsy showed infiltration of the same atypical cells as those in the peripheral blood. In July 1974, combination chemotherapy (VEMP and VENP) was started. He was discharged at the time when a transient remission was achieved clinically, not hematologically. On June 4 1975, he died unexpectedly after a short period of high fever.
The case was presented as a form of “leukemic reticuloendotheliosis” of relatively chronic course developed in a child, which exhibited rather unusual features in many respects.
