Thrombotic microangiopathies

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在2018年3月27日 (二) 12:35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)
第5行：		第5行：
	*Interlobular artery, arterioles, glomerular capillaries: microthrombi, AKI		*Interlobular artery, arterioles, glomerular capillaries: microthrombi, AKI
	*DD: DIC, SLE, cancer		*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)

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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)