Coagulation and vascular abnormalities in Crow-Fukase syndrome
Corresponding Author
Kyoko Saida MD
Department of Neurology and Clinical Research Center, Utano National Hospital Kyoto, Japan
Department of Neurology and Clinical Research Center, Utano National Hospital Kyoto, JapanSearch for more papers by this authorHideji Kawakami MD
Department of Neurology, Faculty of Medicine, Hiroshima University, Hiroshima Japan
Search for more papers by this authorMitsuhiro Ohta PhD
Department of Neurology and Clinical Research Center, Utano National Hospital Kyoto, Japan
Search for more papers by this authorKyoko Iwamura MA
Department of Neurology and Clinical Research Center, Utano National Hospital Kyoto, Japan
Search for more papers by this authorCorresponding Author
Kyoko Saida MD
Department of Neurology and Clinical Research Center, Utano National Hospital Kyoto, Japan
Department of Neurology and Clinical Research Center, Utano National Hospital Kyoto, JapanSearch for more papers by this authorHideji Kawakami MD
Department of Neurology, Faculty of Medicine, Hiroshima University, Hiroshima Japan
Search for more papers by this authorMitsuhiro Ohta PhD
Department of Neurology and Clinical Research Center, Utano National Hospital Kyoto, Japan
Search for more papers by this authorKyoko Iwamura MA
Department of Neurology and Clinical Research Center, Utano National Hospital Kyoto, Japan
Search for more papers by this authorAbstract
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+ 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. © 1997 John Wiley & Sons, Inc. Muscle Nerve, 20, 486–492, 1997
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