An Autopsy Case of Reye Syndrome during a Remission of Malignant Lymphoma

This paper describes clinical and pathological studies on a patient who acutely died of Reye syndrome during a complete remission of malignant lymphoma. Although Reye syndrome is not a common disease in Japan, the number of case reports has been increasing. Yet such a combination with malignancy as in the present case seems extremely rare. The patient was a 4-year~old boy who developed a left mandibular swelling at 2 years of age, was diagnosed as undifferentiated malignant lymphoma by biopsy, and was treated with vincristine, methotrexate, prednisolone and adriamycin. One month after the completion of chemotherapy, the patient was readmitted to our hospital, because of sudden onset of recurrent vomiting, tachypnea, cyanosis, and consciousness disturbance. Laboratory studies revealed metabolic acidosis and hepatic dysfunction with elevated GOT (341 U), GPT(89U), CPK(983U), and blood ammonia (106μg/d/). Blood sugar was 5mg/d/. The CPK isozyme pattern was MM 79%, MB 21% and BB 1%. Serum virus titers including influenza B and chickenpox were within normal limits. Lumbar tap revealed a clear CSF with a mild pleocytosis (326/3/mm3), The patient died on the day of admission. Autopsy showed a gross appearance and histological evidence of fatty degeneration of liver and kidneys. Myocardium also showed fatty degeneration. The brain was edematous, and showed a tonsillar herniation of cerebellum. There was no evidence of relapse of malignant lymphoma. The clinical, laboratory, and pathological findings of the present case are consistent with Reye syndrome, although it took unusually acute course. It is suggested that such an acute course was somehow related with underlying malignant lymphoma or its chemotherapy.