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Coagulation and vascular abnormalities in Crow-Fukase syndrome

✍ Scribed by Kyoko Saida; Hideji Kawakami; Mitsuhiro Ohta; Kyoko Iwamura

Publisher: John Wiley and Sons
Year: 1997
Tongue: English
Weight: 503 KB
Volume: 20
Category: Article
ISSN: 0148-639X
DOI: 10.1002/(sici)1097-4598(199704)20:4<486::aid-mus12>3.0.co;2-x

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✦ Synopsis

Coagulation and vascular abnormalities were studied in 4 patients with Crow-Fukase syndrome (CFS or POEMS) to understand the pathophysiology. Fibrinogen, fibrinopeptide A, and thrombin-antithrombin complexes (TAT) increased in sera during active phase of CFS. In nerves of 2 untreated cases, the endothelium of small vessels was immunohistochemically stained with antithrombin III antibody, which indicates the existence of TAT. HLA-DR ϩ inflammatory cell infiltrate surrounded these vessels. Blood-nerve barrier opening was suggested by strong immunoglobulin staining in the endoneurium. More than 50% of endoneurial blood vessels had narrowed or closed lumina with thick basement membranes. Endothelial cell abnormality and chronic intravascular coagulation may play an important role in the pathogenesis of CFS, in addition to a still unknown demyelinating factor. Refractory cases responded to combined treatment of prednisolone, human leukocyte interferon, and antithrombin drug.

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