An autopsy case of HTLV-i associated myelopathy (HAM) with adult T-cell leukemia (ATL)

Murata, M.; Mizusawa, H.; Kanazawa, I.; Yazawa, T.; Uchida, Y.

Rinsho Shinkeigaku 30(7): 754-759


ISSN/ISBN: 0009-918X
PMID: 2242629
Document Number: 366788
An autopsy case of HTLV-I associated myelopathy (HAM) combined with adult T-cell leukemia (ATL) was reported. Although HAM and ATL are reported to be caused by an identical virus, a combination of these two conditions is extremely rare. This is the first report of an autopsy of the case. A 42-year-old female born in Kumamoto noticed gait disturbance at age 20. Dysesthesia in her lower limbs and bladder bowel disturbance (BBD) gradually appeared at age 29 and at age 39, respectively, and slowly progressed. Neurological examination revealed spastic paraparesis, sensory disturbance in her lower limbs in all modalities and BBD. Furthermore, titers of the anti HTLV-I antibody were increased both in serum and CSF. She was diagnosed as HAM at this stage. She responded to the oral administration of prednisolone. Ten months after the initiation of prednisolone therapy, a tumor shadow appeared in the right lung and characteristic ATL cells were found in the pleural effusion. She died with respiratory failure. ATL cells were not found either in the peripheral blood or CSF throughout the course of her illness. Autopsy revealed solid tumors in both lungs, liver, kidneys, spleen, thyroid gland and para-aortic lymphnodes, composed of ATL cells with extensive necrosis. The spinal cord showed a marked loss of myelin and axons, and perivascular fibrosis in the lateral and anterior columns. These changes were most severe at the 6th and 7th thoracic segments. No ATL cell was found in the spinal cord. Concerning the sensory system, the posterior root, ganglion and posterior column were preserved, whereas the axonal degeneration was found in the biopsied specimen of the sural nerve.

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