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[混合性冷球蛋白血症与血管炎性神经肌病]

[Mixed cryoglobulinemia and vasculitic neuromyopathy].

作者信息

Schlieper M, Kurrer M, Strebel U, Moll C

机构信息

Institut für Neuropathologie, Departement Pathologie, Universität Zürich.

出版信息

Dtsch Med Wochenschr. 1993 Jan 29;118(4):94-9. doi: 10.1055/s-2008-1059305.

Abstract

Over a period of 25 years, a 67-year-old man again and again developed purpura over the lower legs. When hospitalized because of splenomegaly with thrombocytopenia the diagnosis of idiopathic thrombocytopenic purpura was made. Four years later, after an infection of the upper respiratory tract, purpura again developed, together with a symmetrical sensory-motor polyneuropathy with flaccid paraparesis. Biopsy of the N. suralis revealed a florid leukocytoclastic vasculitis. Morphologically there was severe nerve damage with Wallerian degeneration and subtotal nerve fibre loss. Immunological tests demonstrated essential mixed cryoglobulinaemia of IgG/IgM type. Cryoprecipitates must be considered as the cause of the vasculitis, which in turn produced the nerve lesions and purpura. This case underlines the importance of biopsy diagnosis of peripheral nerve and skeletal muscle, because it may demonstrate a treatable cause of a severe acute neuromyopathy.

摘要

在25年的时间里,一名67岁男性反复在小腿出现紫癜。因脾肿大伴血小板减少症住院时,诊断为特发性血小板减少性紫癜。四年后,在上呼吸道感染后,紫癜再次出现,同时伴有对称性感觉运动性多发性神经病及弛缓性截瘫。腓肠神经活检显示为明显的白细胞破碎性血管炎。形态学上存在严重的神经损伤,伴有华勒氏变性和神经纤维部分缺失。免疫检测显示为IgG/IgM型原发性混合性冷球蛋白血症。冷沉淀物被认为是血管炎的病因,进而导致了神经病变和紫癜。该病例强调了外周神经和骨骼肌活检诊断的重要性,因为它可能揭示严重急性神经肌肉病的可治疗病因。

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