Report of a patient with POEMS/Takatsuki/Crow-Fukase syndrome associated with focal spinal pachymeningeal amyloidosis.
The authors present the results of an autopsy of a 67-year-old Japanese man with POEMS/Takatsuki/Crow-Fukase syndrome (P/T/CFS) diagnosed in 1972. Each component of the syndrome was gradually recognized after the resection of lumbar vertebral solitary plasmacytoma in 1967. The patient died in 1989 of generalized infection and renal failure. Autopsy revealed in the vertebral canal between the fifth and seventh thoracic vertebrae dorsal pachymeningeal fibrosis, with prominent amyloid deposition that oppressed the spinal cord. This condition was consistent with the final neurologic manifestation of the patient, bilateral motor and sensory disturbance below the sixth thoracic level. Myelopathy remained clinically unnoticed because neurologic disturbance had begun as peripheral polyneuropathy. Normocellular marrow with heterogeneously scattered lambda light chain-positive plasma cells and degeneration of the myelinated fibers of sciatic nerve also were observed. This is the first report of focal spinal amyloidosis associated with P/T/CFS.
Toyokuni
S
S
Department of Pathology, Faculty of Medicine, Kyoto University Hospital, Japan.
Ebina
Y
Y
Okada
S
S
Yamabe
H
H
Ishikawa
T
T
Uchiyama
T
T
Yodoi
J
J
Uchino
H
H
Takatsuki
K
K
D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000150:complications*; D001344:Autopsy; D004388:Dura Mater; D006801:Humans; D008297:Male; D016878:POEMS Syndrome / Q000209:etiology*; D012584:Sciatic Nerve / Q000473:pathology; D013122:Spinal Diseases / Q000150:complications*; D013904:Thoracic Vertebrae