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Coagulation and vascular abnormalities in Crow-Fukase syndrome

K Saida1, H Kawakami, M Ohta

  • 1Department of Neurology, Utano National Hospital, Kyoto, Japan.

Muscle & Nerve
|April 1, 1997
PubMed

Insights

Crow-Fukase syndrome (CFS) involves vascular and coagulation issues, with increased thrombin-antithrombin complexes (TAT) and narrowed blood vessels in nerves. Treatment with prednisolone, interferon, and antithrombin drugs helped refractory cases.

Area of Science:

  • Neurology
  • Hematology
  • Pathophysiology

Background:

  • Crow-Fukase syndrome (CFS), also known as POEMS syndrome, is a rare multisystem disorder.
  • The exact pathophysiology of CFS, particularly the role of vascular and coagulation abnormalities, remains incompletely understood.

Purpose of the Study:

  • To investigate the coagulation and vascular abnormalities in patients with Crow-Fukase syndrome.
  • To elucidate the underlying pathophysiology of CFS.

Main Methods:

  • Studied 4 patients with CFS, analyzing serum markers and performing nerve immunohistochemistry.
  • Measured fibrinogen, fibrinopeptide A, and thrombin-antithrombin complexes (TAT).
  • Examined endoneurial blood vessels for endothelial abnormalities, TAT presence, inflammatory infiltrates, and blood-nerve barrier integrity.

Main Results:

  • Elevated levels of fibrinogen, fibrinopeptide A, and TAT were observed during the active phase of CFS.
  • Immunohistochemistry revealed TAT in the endothelium of small nerve vessels and surrounding inflammatory cell infiltrates.
  • Evidence of blood-nerve barrier opening and significant narrowing or closure of endoneurial blood vessels with thickened basement membranes was found.

Conclusions:

  • Endothelial cell abnormalities and chronic intravascular coagulation are likely key contributors to CFS pathogenesis.
  • These vascular changes may occur alongside an unidentified demyelinating factor.
  • Combined treatment with prednisolone, human leukocyte interferon, and an antithrombin drug showed efficacy in refractory CFS cases.

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