Thrombotic microangiopathies
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第1行: | 第1行: | ||
*Risk factors | *Risk factors | ||
- | **Pregnancy, pills, quinine, RT | + | **Pregnancy, pills, quinine, RT p'ts given ~OKT3 for rejection, calcineurin inhibitors (cyclosporine, tacrolimus), antiplatelets (ticlopidine and clopidogrel), HIV |
*Clinical features | *Clinical features | ||
**Thrombocytopenic purpura, hemolytic anemia, fever, renal failure (esp. HUS), neurologic disturbances (esp. TTP) | **Thrombocytopenic purpura, hemolytic anemia, fever, renal failure (esp. HUS), neurologic disturbances (esp. TTP) | ||
第10行: | 第10行: | ||
*2nd classification | *2nd classification | ||
**Adult HUS/TTP | **Adult HUS/TTP | ||
- | ***Familial: | + | ***Familial: ADAMTS13 metalloprotease (that cleaves large multimers of von Willebrand's factor) deficiency |
***Sporadic | ***Sporadic | ||
- | ****Some p'ts have antibody to | + | ****Some p'ts have antibody to ADAMTS13 or thrombospondin receptor on endothelial cells |
****Some p'ts have plasminogen-activator inhibitor 1 ↑ | ****Some p'ts have plasminogen-activator inhibitor 1 ↑ | ||
**Childhood HUS | **Childhood HUS |
在2018年3月27日 (二) 09:58所做的修訂版本
- 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
- Thrombotic thrombocytopenic purpura (TTP)
- Hemolytic-uremic syndrome (HUS)
- 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
- Adult HUS/TTP
- 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.