Renal crescentic alpha heavy chain deposition disease

A report of 3 cases and review of the literature

Mariam P Alexander, Samih H. Nasr, Didie C. Watson, Gonzalo P. Méndez, Helmut G. Rennke

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Heavy chain deposition disease (HCDD) is a comparatively recently described entity characterized by glomerular and tubular basement membrane deposition of monoclonal heavy chains without associated light chains. To our knowledge, review of the literature shows only 24 previously reported cases of HCDD with unequivocal evidence of monoclonal heavy chain deposition in the kidney using immunofluorescence microscopic and electron microscopic studies. The predominant heavy chain subtype was γ. There has been a single case of μ HCDD and 2 previously reported cases of α HCDD. In this report, we describe 3 additional cases of α HCDD, all with a crescentic pattern of injury and one of which was associated with cutis laxa. We compare their clinicopathologic features with all previously reported cases of HCDD.

Original languageEnglish (US)
Pages (from-to)621-625
Number of pages5
JournalAmerican Journal of Kidney Diseases
Volume58
Issue number4
DOIs
StatePublished - Oct 2011

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Heavy Chain Disease
Kidney
Cutis Laxa
Glomerular Basement Membrane
Fluorescent Antibody Technique
Electrons
Light
Wounds and Injuries

Keywords

  • alpha
  • Cresents
  • heavy chain deposition disease

ASJC Scopus subject areas

  • Nephrology

Cite this

Renal crescentic alpha heavy chain deposition disease : A report of 3 cases and review of the literature. / Alexander, Mariam P; Nasr, Samih H.; Watson, Didie C.; Méndez, Gonzalo P.; Rennke, Helmut G.

In: American Journal of Kidney Diseases, Vol. 58, No. 4, 10.2011, p. 621-625.

Research output: Contribution to journalArticle

Alexander, Mariam P ; Nasr, Samih H. ; Watson, Didie C. ; Méndez, Gonzalo P. ; Rennke, Helmut G. / Renal crescentic alpha heavy chain deposition disease : A report of 3 cases and review of the literature. In: American Journal of Kidney Diseases. 2011 ; Vol. 58, No. 4. pp. 621-625.
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