Thrombotic microangiopathies

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在2018年3月13日 (二) 17:44所做的修訂版本 (編輯) Guhjy (對話 | 貢獻) ←上一個		當前修訂版本 (2018年3月27日 (二) 12:37) (編輯) (撤銷) Guhjy (對話 | 貢獻)
(4個中途的修訂版本沒有顯示。)
第1行：		第1行：
-	*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	+

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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)