Abstract Details

Title Histopathological and Electrophysiological Findings in Musculoskeletal Chronic Graft-Versus-Host Disease (cGVHD)

Topic Muscle Disease/Neuromuscular Junction

Presentation(s) P07 - (-)

Poster/Presentation
Number 048

Objective

Background BACKGROUND: Although fasciitis is a rare diagnostic manifestation of musculoskeletal cGVHD in NIH guideline, detailed histopathological and electrophysiological findings have not been described.

Design/Methods DESIGN/METHODS: A case report.

Results RESULTS: We presented a 38-year-old male who received bone marrow transplantation four years ago for acute lymphocytic leukemia. He felt muscle cramps the following year. He became a bed-bound state with gradually developing polyarthralgia. Examination revealed marked muscle atrophies and joint contractures in the whole body. Muscular weakness was mild and CK was not elevated. Nerve conduction studies revealed reduction in the amplitude of compound muscle action potentials and mild conduction velocity slowing. Sensory conduction studies were normal. No spontaneous discharges were observed in concentric needle electromyography in the tibialis anterior. Low amplitude polyphasic motor unit potentials fired with rapid frequencies in volitional contraction with complete interference. Mean muscle fiber conduction velocity measured by monopolar needle electrodes were delayed compared with normal control (2.0+-0.2 m/s). The electrodiagnosis was myopathic change which consists of cluster of atrophic fibers. Autopsy was performed because he died suddenly with pneumonia. The marked atrophied muscle fibers less than 10[mu]m in diameter were placed evenly with endomysial fibrosis in the biceps brachii. There were no necrotic processes, no reduced COX activities, and no lymphocytic infiltration. These findings were consistent with advanced myogenic changes different from typical myositis. In the fascia, small numbers of infiltrating CD8 positive T lymphocytes with proliferation of collagen fibers were seen, compatible with fasciitis. Skin changes resembled scleroderma.

Conclusions CONCLUSIONS: Severe muscle fiber atrophy and endomysial fibrosis without necrosis were particular muscle findings in fasciitis in cGVHD. The usefulness of these findings in diagnosis of fasciitis distinct from myositis must been validated in future.

Authors/Disclosures
Kenji Sekiguchi, MD, PhD (Kobe University Graduate School of Medicine) PRESENTER	Dr. Sekiguchi has nothing to disclose.
Fumio Kanda, MD, PhD, FAAN (Kusunoki Neurology Clinic)	No disclosure on file
Shane Raines	Shane Raines has received personal compensation for serving as an employee of 2b Analytics, LLC.
	No disclosure on file
	No disclosure on file
Hisatomo Kowa, MD, PhD (Kobe Univ Graduate School of Health Sciences)	No disclosure on file
Ichizo Nishino, MD, PhD, FAAN (National Institute of Neuroscience, NCNP)	The institution of Dr. Nishino has received research support from AMED.
	No disclosure on file

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