摘要
目的探讨血栓性血小板减少性紫癜的病因、临床特点和治疗效果。方法对我院1993.-2005.6收治的血栓性血小板减少性紫癜(TTP)患者12例进行回顾性分析。结果本组12例患者中,原发性TTP5例,继发性TTP7例;7例继发性TTP中,感染所致2例,自身免疫性疾病伴感染1例,术后1例,宫内死胎1例,肝移植及骨髓移植术后各1例。原发性TTP中1例起病缓、病程达半年呈慢性型,其余4例及继发性均以急性暴发型起病。临床表现为微血管病性溶血性贫血(12/12)、血小板减少(12/12)、神经精神障碍(12/12)、肾脏损害(11/12)、发热(11/12),全部患者均有血清乳酸脱氢酶(LDH)明显升高。7例继发性TTP中4例血浆置换(PE)和病因治疗为主,均治愈,3例未行PE,均死亡;5例原发性TTP中2例行PE,1例治愈,1例死亡,3例未行PE,均死亡。结论本组12例TTP中以继发性TTP多见,多为急性暴发型起病;突出临床表现为微血管病性溶血性贫血、血小板减少、神经精神障碍、肾脏损害、发热,LDH明显升高;死亡率仍高。治疗上应强调PE的重要性;继发性TTP,若病因能有效控制,预后较原发者好。
Objective： To investigate the pathogenisis, clinical characteristic and therapeutic efficacy of TTP. Method： 12 patientswith TFP were analyzed in a retrospective manner. Results： 5 patients were idiopathic TIP and 7 were secondary; The secondary TIP were caused by infection （2 patients）, autoimrnune disease with infection （ 1 patient）, surgery （ 1 patient）, fetal death in utero （ 1 patient）, liver transplantation （ 1 patient） and bone marrow transplantation （ 1 patient）. 1 idiopathic TTP developed slowly with a course of half a year; the rest developed and progressed rapidly. Clinical manifestations were characterized by microangiopathic hemolytic anemia （12/12）, severe thrombocytopenia （12/12）, and neurologic abnormalities （12/12）, renal involvement （ 11/12） and fever （11/12）, and serumal LDH was extremely high in all the patients., 4 of 7 secondary TIP patients were cured by plasma exchange （PE） and etilogical eradication, the other 3 patients died without PE; 2 of 5 idiopathic TTP patients were treated with PE, one was cured and another died, the other 3 patients died without PE. Conclusion： The secondary TTP were more eommon than the idiopathic TIP in our study, most TTP developed and progressed rapidly; the characteristic Clinical manifestations were hemolytic anemia, severe thrombocytopenia, neurologie abnormalities, renal involvement, fever, and impressively elevated LDH in serum; The mortality is still very high. PE is an important treatment manner for TTP. If the etiology of secondary TTP was well eradicated, its prognosis may much better than idiopathic TTP.
出处
《华西医学》
CAS
2006年第2期255-257,共3页
West China Medical Journal
