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Non-Hodgkin's Lymphoma following Acute Myeloid Leukemia in Complete Remission

A 57-year-old female was admitted with low grade fever, leukocytosis, anemia and thrombocytopenia. Her white blood cell count was 59.7 ! 109/l, with 83% leukemic cells, some of which had Auer rods. The blasts were myeloperoxidase-positive with a myeloid immunophenotype (CD33: 99.1%, CD13: 8.5%, CD19: !1.0%, CD20: !1.0%, HLA-DR: 18.4%). She was diagnosed as having AML (M2) and underwent combination chemotherapy (the Japan Adult Leukemia Study GroupAML89 regimen which includes enocitabine, cytarabine, mercaptopurine, methotrexate, daunorubicin, aclarubicin, mitoxantrone, vindesine, etoposide, prednisolone), resulting in complete remission. She was well thereafter and was followed as an outpatient without treatment. Thirty-seven months following the diagnosis of AML, however, she complained of nausea and abdominal pain. Her serum lactate dehydrogenase level rose from 313 to 844 and 1,371 IU/l within 2 months (normal: less than 310). When she was readmitted for investigation, the peripheral blood counts were normal and examination of her bone marrow showed complete remission. Serum CA 19-9 and CA 125 levels were elevated to 145.6 ng/ml (normal: less than 60) and 315 U/ml (normal: less than 35), respectively. The chest X-ray revealed a small amount of bilateral pleural effusion, and the computed tomography scan of the abdomen showed multiple intrahepatic tumors and a moderate amount of ascites (fig. 1). There was no evidence of another tumor or lymphadenopathy. We suspected that she had carcinoma of the digestive or gynecologic system because of the high levels of the two characteristic tumor markers. However, no abnormality was detected by the gastrointestinal and gynecologic study. Examination of the pleural effusion and ascites showed no malignant or clonal cells, but 1 month later, reexamination of the patient’s ascites showed atypical cells (fig. 2). She died within 1 week of disseminated intravascular coagulation. The autopsy revealed diffuse, medium-sized, B cell non-Hodgkin’s lymphoma [LCA (CD 45); L26 (CD20)and UCHL1 (CD45RO)-positive but neutrophilic esterase-negative] in the liver tumors (maximum diameter 6 cm). There was also microscopic infiltration by the lymphoma cells into the diaphragm, omentum, peritoneum, stomach, parapancreatic region and para-aortic lymph nodes.