What is the pathophysiology of pneumococcal-associated hemolytic-uremic syndrome?

Updated: Nov 12, 2018
  • Author: Robert S Gillespie, MD, MPH; Chief Editor: Craig B Langman, MD  more...
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Answer

Pneumococcal-associated hemolytic-uremic syndrome constitutes a distinct subgroup of aHUS. This variant occurs with infections caused by S pneumoniae, usually pneumonia. [4] It is actually a distinct entity that has little relation to the aHUS associated with complement factor mutations. The bacterial toxin neuraminidase damages endothelial cells and initiates hemolytic-uremic syndrome in this setting.

As a toxin-mediated disease, pneumococcal-associated hemolytic-uremic syndrome has much in common with STEC-HUS mediated by Stx. Bacteria with neuraminidase remove N- acetylneuraminic acid from cell-surface glycoproteins and expose the normally hidden T antigen (Thomsen-Friedenreich antigen) on erythrocytes, platelets, and glomeruli. Serum contains anti-T immunoglobulin M (IgM), which can react with the antigen and cause damage to RBCs and the kidneys.


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