The histopathologic spectrum of cryofibrinogenemia in four anatomic sites: Skin, lung, muscle, and kidney Academic Article Article uri icon

Overview

MeSH Major

  • Lymphoma, T-Cell, Cutaneous
  • Precancerous Conditions
  • Skin Neoplasms

abstract

  • Although the histologic characteristics of cryofibrinogenemia have been described in skin lesions, the literature is largely devoid of descriptions of this disorder in other organs. This series is the first to document the histopathologic manifestations of intrapulmonary, intramuscular, and renal cryofibrinogenemia. We describe the histopathologic manifestations of cryofibrinogenemia in 10 cases with manifestations in 4 organ systems: skin in 7 cases, skeletal muscle in 2, lung in 2, and kidney in 1. Irrespective of anatomic site, all lesions showed an occlusive thrombotic diathesis comprising eosinophilic refractile deposits within vessel lumina with extension into the intima, with or without an accompanying characteristic granulomatous vasculitic component. Ultrastructural examination of the renal deposits showed fibrillary material within glomerular capillary lumina with unique morphologic features not previously described. Analysis of plasma from several cases revealed a cold-precipitable protein, which in most cases included a monoclonal paraprotein. The laboratory and histologic distinctions between cryofibrinogenemia and cryoglobulinemia are addressed. We provide guidelines for the proper handling of patient specimens in the workup of cryofibrinogenemia.

publication date

  • January 2003

Research

keywords

  • Academic Article

Identity

Digital Object Identifier (DOI)

  • 10.1309/KB5A-RGWV-L1R2-BPBN

PubMed ID

  • 12520706

Additional Document Info

start page

  • 114

end page

  • 22

volume

  • 119

number

  • 1