抄録
A case of T-cell malignancy with clinical manifestations of chronic leukemic reticuloendotheliosis was reported. The patient (37-year-old male) was admitted to our hospital because of abdominal mass, fever and weight loss. A huge spleen, pancytopenia and atypical cells in the bone marrow (24%) were the most prominent findings in physical and laboratory examinations on admission. Temporary reduction in size of the huge spleen was brought about with the administration of prednisolone, and then, splenectomy and liver biopsy were performed.
Histology of the excised spleen and liver biopsy were consistent with so called chronic leukemic reticuloendotheliosis. Ribosome-lamella-complex and tartrate-resistant acid phosphatase were absent in atypical cells. In addition, there were no hairy cells. Surface-marker analysis identified them as T-lymphocytes.
Following splenectomy, the number of atypical cells increased in the bone marrow as well as in the blood. Hepatomegaly, lymphadenopathy and extranodal lesions such as the left testicular and multiple subcutaneous tumors developed. The patient died of respiratory failure 3 years and 6 months after the onset. Autopsy disclosed leukemic infiltration to the bone marrow, heart, liver. kidneys, adrenal glands, accessory spleen and lymph nodes (thoracic, portal, perigastric, peripancreatic and retroperitoneal).
