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HTLV-I-associated peripheral neuropathy with smoldering-type adult T-cell leukemia
Hideaki Matsui1, Fukashi Udaka, Tamotsu Kubori
1Department of Neurology, Sumitomo Hospital, Nakanoshima, Osaka, Japan. matsui-hideaki@sumitomo-hp.or.jp
Background:
Peripheral neuropathy with adult T-cell leukemia/lymphoma (ATLL) has seldom been reported.
Review Summary:
A 69-year-old woman with smoldering-type ATLL presented with pain and muscle weakness of the bilateral upper and lower limbs and gait disturbance. Anti-human T lymphotropic virus type I antibody was positive in the serum but negative in the cerebrospinal fluid. Magnetic resonance imaging (MRI) showed no abnormal signals in the spinal cord or brain. Nerve conduction studies disclosed severe peripheral neuropathy. Sural nerve biopsy disclosed both axonal degeneration and demyelinated fibers, and marked perivascular inflammatory infiltrates mainly consisting of T lymphocytes without malignant changes were seen. Steroid therapy was markedly effective, and the patient became able to walk without assistance.
Conclusions:
This is the first reported case of ATLL and peripheral neuropathy in which a nerve biopsy was performed. In our patient, the marked perivascular inflammatory infiltrates mainly consisting of T lymphocytes suggested either immune-mediated vasculitis or tumor invasion. Further studies are needed.
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