Bianchi Valentina, Robles Rodolfo, Alberio Lorenzo, Furlan Miha, Lämmle Bernhard
Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland.
Blood. 2002 Jul 15;100(2):710-3. doi: 10.1182/blood-2002-02-0344.
A severe deficiency in von Willebrand factor-cleaving protease (ADAMTS13) activity (< 5% that in normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) but not in those with a diagnosis of hemolytic uremic syndrome. However, ADAMTS13 deficiency has been claimed not to be specific for TTP, since it was observed in various thrombocytopenic and other conditions. We studied 68 patients with thrombocytopenia due to severe sepsis or septic shock (n = 17), heparin-induced thrombocytopenia (n = 16), idiopathic thrombocytopenic purpura (n = 10), or other hematologic (n = 15) or miscellaneous conditions (n = 10). Twelve of the 68 patients had subnormal levels of ADAMTS13 activity (<or= 30%), but none had less than 10%. Thus, the study showed that ADAMTS13 activity is decreased in a substantial proportion of patients with thrombocytopenia of various causes. A severe deficiency of ADAMTS13 (< 5%), identified in more than 120 patients during 1996 to 2001 in our laboratory, is specific for a thrombotic microangiopathy commonly labeled TTP.
在大多数诊断为血栓性血小板减少性紫癜（TTP）的患者中观察到血管性血友病因子裂解蛋白酶（ADAMTS13）活性严重缺乏（<正常血浆活性的5%），而诊断为溶血尿毒综合征的患者则未观察到这种情况。然而，有人认为ADAMTS13缺乏并非TTP所特有，因为在各种血小板减少症及其他病症中也观察到了该情况。我们研究了68例因严重脓毒症或脓毒性休克（n = 17）、肝素诱导的血小板减少症（n = 16）、特发性血小板减少性紫癜（n = 10）或其他血液学疾病（n = 15）或杂症（n = 10）导致血小板减少的患者。68例患者中有12例ADAMTS13活性水平低于正常（≤30%），但无一例低于10%。因此，该研究表明，在各种原因导致的血小板减少症患者中，相当一部分患者的ADAMTS13活性降低。1996年至2001年期间，在我们实验室确诊的120多例患者中发现的严重ADAMTS13缺乏（<5%）是一种通常称为TTP的血栓性微血管病所特有的。
