抄録
A case of leukemic reticuloendotheliosis supposed to be chronic type was reported.
The patient (29-year old male) was admitted with chief complaints of nasal bleeding, general malaise and high fever. A huge spleen, pancytopenia and hypercellular bone marrow with maturation arrest were the most striking findings in physical and laboratory examinations on admission. The administration of betamethazone resulted in the temporary disappearance of the splenomegaly which reappeared after tapering down the drug.
Four months later, splenectomy and liver biopsy were performed, when 10% of atypical lymphoid cell was seen in the peripheral blood and 9.2% in the bone marrow. The portal pressure was normal and histology of the spleen showed atrophy of white pulp and infiltration of small to mediumsized mononuclear cells mainly in red pulp. Liver biopsy also showed diffuse scattering of simillar cells in the sinusoids.
Six months after splenectomy, he was readmitted because of progressive hepatomegaly and leucocytosis, when more than 90% of atypical lymphoid cell was seen both in the peripheral blood and the bone marrow. Cytological studies including electronmicroscopic and phase contrast microscopic examinations revealed the proliferating cells to be consistent with those of leukemic reticuloendotheliosis, and combination chemotherapy (VENP) was started. Four months later, hematological complete remission was obtained, which lasted for 6 months.
