Rheumatic heart disease

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Rheumatic heart disease, abbreviated RHD, is a relatively uncommon heart valve pathology that follows rheumatic fever.

General

  • Classically leads to mitral valve stenosis.
    • Rheumatic fever accounts for 99% of mitral stenosis.[1]
      • Caused by Streptococcus pyogenes.[2]
  • Disease less frequent today - as streptococcal pharynigits is treated with antibiotics.

Gross

  • "Fish-mouth appearance".
  • Significant valvular thickening.
  • Thickening and shortening of the cordae tendinae.

DDx:

  • Thickening of the cordae tendinae due to micronodular cirrhosis.[4]

Images

  • RHD - showing valvular thickening and thickening of the cordae tendinae. (WC)

  • RHD - showing valvular thickening - aortic valve. (WC)

Microscopic

Features:[5]

  • Caterpillar cells (AKA Anitschkow cells)
    • Abundant eosinophilic cytoplasm.
    • Moderately-poorly defined cell border.
    • Well-defined central ovoid nucleus with a prominent wavy ribbon-like chromatin -- looks vaguely like a caterpillar with some imagination.
    • Pathognomonic for rheumatic fever.
  • Aschoff bodies - usually in the heart itself:
    • Jumbled collagen, eosinophilic.
    • Surrounded by lymphocytes (T cells) +/- plasma cells.

Notes:

  • Anitschkow cells are thought to be histocytes and Aschoff bodies are thought to be granulomas.[6]
    • This is disputed.[7]

Images

  • RHD - intermed. mag. (WC/Nephron)

  • RHD - high mag. (WC/Nephron)

  • RHD - very high mag. (WC/Nephron)

  • Aschoff body (WC/Uthman)

  • Anitschkow myocytes (WC/Uthman)

IHC

Features (Aschoff bodies & Anitschkow cells):[6]

  • S100 -ve.
  • Muscle specific actin -ve.
  • Desmin -ve.
  • NF -ve.
  • Vimentin +ve.
  • CD45 +ve (weak).

See also

References

  1. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 594. ISBN 0-7216-0187-1.
  2. ↑ Chopra, P.; Gulwani, H. (Oct 2007). "Pathology and pathogenesis of rheumatic heart disease.". Indian J Pathol Microbiol 50 (4): 685-97. PMID 18306530.
  3. ↑ URL: http://en.wikipedia.org/wiki/Ellipse. Accessed on: 13 November 2010.
  4. ↑ Rose, Alan G. (2008). Atlas of Gross Pathology with Histologic Correlation (1st ed.). Cambridge University Press. pp. 25. ISBN 978-0521868792.
  5. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 593. ISBN 0-7216-0187-1.
  6. ↑ 6.0 6.1 Love, GL.; Restrepo, C. (Jul 1988). "Aschoff bodies of rheumatic carditis are granulomatous lesions of histiocytic origin.". Mod Pathol 1 (4): 256-61. PMID 3070554.
  7. ↑ Stehbens, WE.; Zuccollo, JM. (May 1999). "Anitschkow myocytes or cardiac histiocytes in human hearts.". Pathology 31 (2): 98-101. PMID 10399163.
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