Thrombotic microangiopathies

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在2018年3月27日 (二) 09:58所做的修訂版本 (編輯)
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當前修訂版本 (2018年3月27日 (二) 12:37) (編輯) (撤銷)
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-*Risk factors+*HUS/TTP, antiphospholipid syndrome, malignant HT., systemic sclerosis (renal crisis), HIV, pregnancy (preeclampsia, HELLP: hemolysis, elevated liver enzymes, low platelet count), hematopoietic stem cell transplantation
-**Pregnancy, pills, quinine, RT p'ts given OKT3 for rejection, calcineurin inhibitors (cyclosporine, tacrolimus), antiplatelets (ticlopidine and clopidogrel), HIV+*Pentad: thrombocytopenic purpura, intravascular hemolytic anemia (fragmented RBCs), fever, + AKI (HUS), + neurol. dis. (TTP)
-*Clinical features+*Drugs: mitomycin, gemcitabine, VEGF inh., anti-rejection (calcineurin inhibitors, OKT3), pills, antiplatelets (ticlopidine, clopidogrel, prasugrel), quinine
-**Thrombocytopenic purpura, hemolytic anemia, fever, renal failure (esp. HUS), neurologic disturbances (esp. TTP)+*PT, aPTT: normal
-*Pathology+*Interlobular artery, arterioles, glomerular capillaries: microthrombi, AKI
-**Endotheliosis with fibrin thrombi+*DD: DIC, SLE, cancer
-*1st classification+*TTP and HUS: ADAMTS13 metalloprotease (which cleaves von Willebrand's factor) deficiency, hypocomplementemia
-**Thrombotic thrombocytopenic purpura (TTP)+*TTP (thrombotic thrombocytopenic purpura)
-**[[Hemolytic-uremic syndrome (HUS)]]+**Familial, acquired, sporadic
-*2nd classification+**Rx: Plasmapheresis, Rituximab (CD20 Ab to deplete B cell), steroids
-**Adult HUS/TTP+*HUS (hemolytic-uremic syndrome):
-***Familial: ADAMTS13 metalloprotease (that cleaves large multimers of von Willebrand's factor) deficiency+**(esp. children) Shiga toxin [verotoxin] with enterohemorrhagic diarrhea (STEC: E. coli O157:H7, rarely Shigella), pneumococcus (rare), supportive Rx (no antibiotics, no anti-diarrhea Rx), dialysis prn.
-***Sporadic+**(children or adults) Atypical HUS (complement activation): very rare, genetic or acquired, no diarrhea, normal or partial def. ADAMTS13
-****Some p'ts have antibody to ADAMTS13 or thrombospondin receptor on endothelial cells+**Rx: Plasmapheresis (not for typical STEC HUS), eculizumab (C5 Ab) for atypical HUS, (splenectomy)
-****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.+

當前修訂版本

  • HUS/TTP, antiphospholipid syndrome, malignant HT., systemic sclerosis (renal crisis), HIV, pregnancy (preeclampsia, HELLP: hemolysis, elevated liver enzymes, low platelet count), hematopoietic stem cell transplantation
  • Pentad: thrombocytopenic purpura, intravascular hemolytic anemia (fragmented RBCs), fever, + AKI (HUS), + neurol. dis. (TTP)
  • Drugs: mitomycin, gemcitabine, VEGF inh., anti-rejection (calcineurin inhibitors, OKT3), pills, antiplatelets (ticlopidine, clopidogrel, prasugrel), quinine
  • PT, aPTT: normal
  • Interlobular artery, arterioles, glomerular capillaries: microthrombi, AKI
  • DD: DIC, SLE, cancer
  • TTP and HUS: ADAMTS13 metalloprotease (which cleaves von Willebrand's factor) deficiency, hypocomplementemia
  • TTP (thrombotic thrombocytopenic purpura)
    • Familial, acquired, sporadic
    • Rx: Plasmapheresis, Rituximab (CD20 Ab to deplete B cell), steroids
  • HUS (hemolytic-uremic syndrome):
    • (esp. children) Shiga toxin [verotoxin] with enterohemorrhagic diarrhea (STEC: E. coli O157:H7, rarely Shigella), pneumococcus (rare), supportive Rx (no antibiotics, no anti-diarrhea Rx), dialysis prn.
    • (children or adults) Atypical HUS (complement activation): very rare, genetic or acquired, no diarrhea, normal or partial def. ADAMTS13
    • Rx: Plasmapheresis (not for typical STEC HUS), eculizumab (C5 Ab) for atypical HUS, (splenectomy)