What is the pathophysiology of thrombocytopenia in pediatric hemolytic uremic syndrome (HUS)?

Updated: Nov 12, 2018
  • Author: Robert S Gillespie, MD, MPH; Chief Editor: Craig B Langman, MD  more...
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Thrombocytopenia is believed to result from a combination of platelet destruction, increased consumption, sequestration in the liver and spleen, and intrarenal aggregation. Platelets are damaged as they pass through the affected glomerular capillaries. Remaining platelets circulate in a degranulated form and show impaired aggregation. Stx also binds to activated platelets.

Abnormalities of anti–platelet-aggregating agents (eg, prostaglandin I2 [PGI2]), platelet-aggregating agents (thromboxane A2 [TXA2]) and von Willebrand factor (vWF) multimers are also important factors that contribute to thrombocytopenia. A decrease in PGI2 during the early stages of hemolytic-uremic syndrome has been noted. Defective PGI2 production is believed to play a role in STEC-HUS; abnormal PGI2 synthesis is believed to play a role in aHUS.

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