| Crystalloidal paraprotein deposits in the cornea: an ultrastructural study of two new cases with tubular crystalloids that contain IgG kappa light chains and IgG gamma heavy chains. | |
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MedLine Citation:
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PMID: 9205998 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The fine structure and immunoprotein content of the crystalloids are described in two cases of paraproteinemic crystalloidal keretopathy, both of which had clinical features thought by the referring ophthalmologists to be those of atypical lattice-type corneal dystrophy (presumably because of lattice-like lines). Most keratocytes in one case were surrounded by a mantle of densely packed tubular crystalloids. Individual tubules were annular in cross section with mean dimensions as follows: overall diameter, 29.32 nm (SD 1.26); internal diameter (core), 8.53 nm (SD 1.12); wall thickness, 10.39 nm (SD 0.85) (n = 10). Crystalloids were extracellular and found only in the corneal stroma, with none in Bowman's layer or Descemet's membrane. In the second case, the tubules had a similar distribution but formed geometric arrays with no clear relationship to, or envelopment of the keratocytes. The tubules were thin-walled, with mean dimensions as follows: overall diameter, 26.12 nm (SD 1.12); internal diameter (core), 15.46 nm (SD 1.12); wall thickness, 5.33 nm (SD 0) (n = 10). In both cases the tubules were kappa-light chain- and gamma-chain-positive. Laboratory investigations revealed the presence of two IgM-kappa paraproteins and an IgG-kappa paraprotein in the serum of the first patient. The second patient had an IgG-kappa paraproteinemia and bone marrow changes consistent with low-grade non-Hodgkin's lymphoma. These cases emphasize and extend the morphological range of corneal IgG crystalloids; the second case also demonstrates that corneal IgG crystalloids may be an early indicator of un underlying immunoproliferative disease. |
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Authors:
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J W Stirling; D W Henderson; M A Rozenbilds; J M Skinner; M Filipic |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Ultrastructural pathology Volume: 21 ISSN: 0191-3123 ISO Abbreviation: Ultrastruct Pathol Publication Date: 1997 Jul-Aug |
Date Detail:
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Created Date: 1997-08-21 Completed Date: 1997-08-21 Revised Date: 2009-06-26 |
Medline Journal Info:
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Nlm Unique ID: 8002867 Medline TA: Ultrastruct Pathol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 337-44 Citation Subset: IM |
Affiliation:
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Department of Pathology, Flinders Medical Centre, Bedford Park, Australia. john.stirling@flinders.edu.au |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Cornea / metabolism* Humans Immunoglobulin gamma-Chains / immunology* Immunoglobulin kappa-Chains / immunology* Inclusion Bodies / immunology, ultrastructure* Male Microscopy, Electron Paraproteins / metabolism* |
| Chemical | |
Reg. No./Substance:
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0/Immunoglobulin gamma-Chains; 0/Immunoglobulin kappa-Chains; 0/Paraproteins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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