1 / 18

Heavy chain deposition disease in kidney biopsies

Heavy chain deposition disease in kidney biopsies. Alenka Vizjak , Jerica Mraz, Jelka Lindič, Dušan Ferluga Institute of Pathology, Faculty of Medicine University of Ljubljana , Slovenia Disclosures: no conflicts of interest. Heavy chain deposition disease (HCDD).

shing
Télécharger la présentation

Heavy chain deposition disease in kidney biopsies

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Heavy chain deposition disease in kidney biopsies Alenka Vizjak, Jerica Mraz, Jelka Lindič, Dušan Ferluga Institute of Pathology, Faculty of Medicine University of Ljubljana, Slovenia Disclosures: no conflicts of interest

  2. Heavy chain deposition disease (HCDD) • HCDD - a rare monoclonal immunoglobulin-related disorder of not yet fully explored pathogenesis • Characterized by production and systemic deposition of structurally abnormal immunoglobulin heavy chains, while light chains absent in the deposits • First described by Aucouturier et al(N Engl J Med 1993; 329: 1389-93)

  3. Monoclonalimmunoglobulin deposits in the kidney

  4. Patients and methods • 4 biopsy cases of HCDD (5 kidney biopsies; 1 autopsy), representing 0.09% prevalence among 5481 native kidney biopsies • All 4 female patients, age range 62 – 79 yrs, mean age 73.0 yrs • Standard light microscopy • Immunofluorescence microscopy IgA, IgG, IgM, κ, λ, C3, C1q, fibrin/fibrinogen, albuminIgG1, IgG2, IgG3, IgG4, γCH1, γCH2, γCH3 • Electron microscopy

  5. Clinical presentation / diagnosis in 4 patients with HCDD CKD – chronic kidney disease, NS – nephrotic syndrome

  6. Immunofluorescence microscopy in 4 cases of HCDD

  7. Light and electron microscopy in 4 cases with HCDD Light microscopy • Diffuse nodular glomerulosclerosis 4/4 • Glomerular capillary aneurysms 4/4 • Mesangial proliferation, 4/4with segm endo-, membranoprol pattern 3/4 • Extracapillary crescents (few) 2/4 Electron microscopy • Punctate and powdery electron dense deposits 3/4

  8. IgG (γ heavy chain) κ, λ light chains

  9. IgG (γ heavy chain)

  10. IgG1 IgG2

  11. C3 C1q

  12. γCH1 γCH2

  13. SMA+CD31 CD68

  14. Conclusions of our study • Immunofluorescence examination of kidney biopsy, including testing for Igs heavy and light chains, as well as IgG subclasses, is crucial for the diagnosis of HCDD. • Our study showed that HCDD is peculiar among MIDD because of uniform pattern of nodular glomerulosclerosis with pronounced capillary aneurysms and significant proliferation due to complement activation. • Constant deletion of the gamma heavy chain CH1 domain and its significance in the pathogenesis of HCDD was confirmed.

More Related