Incapacitating peripheral neuropathy as a manifestation of chronic graft-versus-host disease.

Incapacitating peripheral neuropathy as a manifestation of chronic graft-versus-host disease.
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DOI:
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发表时间:
1990-05
影响因子:
4.8
通讯作者:
A. Greenspan;Deeg Hj;Michele Cottler-Fox;M. Sirdofski;Thomas R. Spitzer;J. Kattah
A. Greenspan;Deeg Hj;Michele Cottler-Fox;M. Sirdofski;Thomas R. Spitzer;J. Kattah
中科院分区:
医学3区
文献类型:
--
作者:
A. Greenspan;Deeg Hj;Michele Cottler-Fox;M. Sirdofski;Thomas R. Spitzer;J. Kattah

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一名 35 岁的白人男子在骨髓移植后 5 个月出现轻微且短暂的慢性移植物抗宿主病 (GVHD) 症状。 16 个月大时,他出现手、脚和躯干肌肉疼痛性痉挛。电生理学研究揭示了全身感觉神经病。在不自主的肌肉收缩和周围肌肉群的激动剂/拮抗剂共同收缩期间(不连续),偶尔会出现肌束颤动和高频运动单位。腓肠神经活检显示施万细胞和巨噬细胞带有髓磷脂碎片,施万细胞堆带有胶原蛋白袋。患者的症状对糖皮质激素和硫唑嘌呤治疗有反应。我们认为该患者的失能性多发性神经病是慢性 GVHD 的主要表现,表明周围神经系统和可能的中枢神经系统可能是慢性 GVHD 的目标。
A 35-year-old caucasian man developed mild and transient signs of chronic graft-versus-host disease (GVHD) 5 months after bone marrow transplantation. At 16 months he presented with painful cramps in hands, feet, and truncal muscles. Electrophysiological studies revealed generalized sensory neuropathy. There was occasional fasciculation and high frequency motor units during involuntary muscle contractions, and agonist/antagonist cocontractions of peripheral muscle groups (non-continuous). A sural nerve biopsy showed Schwann cells and macrophages with myelin debris, and Schwann cell stacks with collagen pockets. The patient's symptoms responded to treatment with glucocorticoids and azathioprine. We propose that this patient's incapacitating polyneuropathy was a major manifestation of chronic GVHD, suggesting that the peripheral and possibly central nervous systems can be targets of chronic GVHD.