Coagulation and vascular abnormalities in Crow-Fukase syndrome.
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.
Saida
K
K
Department of Neurology, Utano National Hospital, Kyoto, Japan.
Kawakami
H
H
Ohta
M
M
Iwamura
K
K
D000933:Antifibrinolytic Agents / Q000032:analysis; D000990:Antithrombin III / Q000032:analysis; D001369:Axons / Q000473:pathology; D001706:Biopsy; D001779:Blood Coagulation Factors / Q000032:analysis*; D002196:Capillaries / Q000473:pathology; D004730:Endothelium, Vascular / Q000473:pathology; D005260:Female; D005340:Fibrinogen / Q000032:analysis; D005341:Fibrinolysin / Q000032:analysis; D005344:Fibrinopeptide A / Q000032:analysis; D006684:HLA-DR Antigens / Q000032:analysis; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009000:Monocytes / Q000473:pathology; D009410:Nerve Degeneration; D009412:Nerve Fibers / Q000473:pathology; D009504:Neutrophils / Q000473:pathology; D016878:POEMS Syndrome / Q000097:blood* / Q000473:pathology* / Q000628:therapy; D010447:Peptide Hydrolases / Q000032:analysis; D010958:Plasminogen / Q000032:analysis; D011239:Prednisolone / Q000627:therapeutic use; D012583:Schwann Cells / Q000473:pathology; D013497:Sural Nerve / Q000098:blood supply / Q000473:pathology*; D000979:alpha-2-Antiplasmin
D000933:Antifibrinolytic Agents; D001779:Blood Coagulation Factors; D006684:HLA-DR Antigens; D016898:Interferon-alpha; D000979:alpha-2-Antiplasmin; C046193:antithrombin III-protease complex; C037742:plasmin-plasmin inhibitor complex; D005344:Fibrinopeptide A; D000990:Antithrombin III; D005340:Fibrinogen; D010958:Plasminogen; D011239:Prednisolone; D010447:Peptide Hydrolases; D005341:Fibrinolysin