Thrombotic microangiopathies
出自KMU Wiki
- Risk factors
- Pregnancy, pills, quinine, RT p'ts given OKT3 for rejection, calcineurin inhibitors (cyclosporine, tacrolimus), antiplatelets (ticlopidine and clopidogrel), HIV
- Clinical features
- Thrombocytopenic purpura, hemolytic anemia, fever, renal failure (esp. HUS), neurologic disturbances (esp. TTP)
- Pathology
- Endotheliosis with fibrin thrombi
- 1st classification
- 2nd classification
- Adult HUS/TTP
- Familial: ADAMTS13 metalloprotease (that cleaves large multimers of von Willebrand's factor) deficiency
- Sporadic
- Some p'ts have antibody to ADAMTS13 or thrombospondin receptor on endothelial cells
- Some p'ts have plasminogen-activator inhibitor 1 ↑
- Childhood HUS
- Escherichia coli O157:H7 (occasionally Shigella dysenteriae) causing enterohemorrhagic diarrhea
- Treatment
- Daily plasmapheresis
- Patients with childhood HUS from infectious diarrhea are not given antibiotics, as antibiotics are thought to accelerate the release of the toxin and the diarrhea is usually self-limited.
