Abstract
We report a case of disseminated necrotizing leukoencephalopathy in a 43-year-old male with acute promyelocytic leukemia (APL). He was diagnosed as having APL in Sep. 22, 1980 and entered into a complete remission with administration of daunomycin, 6-MP and predonisolone. Because of meningeal relapse, he received several courses of intrathecal methotrexate, cytosine arabinoside and hydrocortisone. In addition, he received 2800 rad of whole skull irradiation over the period of 4 weeks. He developed rapidly progressive neurological symptoms such as salivation, dysarthria and dementia at the termination of whole skull irradiation. Brain CT scan films revealed diffuse low density area in cerebral white matter. Brain scintigram showed slightly ill-defined uptake of 99mTcO4- in bilateral frontal and temporal areas. EEG showed slow waves predominantly in frontal lobes. About one month after the termination of whole skull irradiation, he died of respiratory failure due to the disturbance of central nervous system.
The neuropathological examination of autopsied materials revealed the discrete, coalescent foci of coagulative necrosis in the cerebral white matter, pons, midbrain and cerebellar white matter. These lesions were accompanied by calcification and fibrinoid necrosis of the blood vessel walls. Striking axonal swellings and fragmentation were also noted.
In the prophylaxis and/or treatment of meningeal leukemia of adult patients with acute nonlymphocytic leukemia, we should always keep in mind the hazard of the development of disseminated necrotizing leukoencephalopathy related to therapy.
