CAJ | 학술논문

目的：通过回顾性分析12年中在我院住院治疗的19例血栓性血小板减少性紫癜（ TTP）患者的临床特点，评估其临床表现、治疗和预后的相关性。方法统计19例TTP患者的临床表现，治疗方案和治疗期间的血红蛋白（ HGB）、血小板（ PLT）、乳酸脱氢酶（ LDH）和间接胆红素（ IBIL），了解其对预后的影响。结果19例患者均存在不同程度的PLT降低和神经精神症状，随着病程的进展，HGB逐渐下降，而发热和肾功能损害的表现程度不一。根据患者的转归将其分为好转组和死亡组，统计发现死亡组在治疗期间LDH和IBIL明显高于好转组。15例患者在治疗过程中接受血浆置换治疗，治愈率为80％，而是否使用大剂量激素冲击治疗对患者的转归没有影响。结论①程度不一的PLT减少和神经精神症状是TTP中最常见的临床表现；②外周血破碎红细胞的监测能为早期诊断提供证据；③在治疗过程中，LDH和IBIL水平的变化对转归的判断具有指导意义；④血浆置换是治疗TTP的有效手段，但大剂量激素治疗对预后并无影响。
목적：통과회고성분석12년중재아원주원치료적19례혈전성혈소판감소성자전（ TTP）환자적림상특점，평고기림상표현、치료화예후적상관성。방법통계19례TTP환자적림상표현，치료방안화치료기간적혈홍단백（ HGB）、혈소판（ PLT）、유산탈경매（ LDH）화간접담홍소（ IBIL），료해기대예후적영향。결과19례환자균존재불동정도적PLT강저화신경정신증상，수착병정적진전，HGB축점하강，이발열화신공능손해적표현정도불일。근거환자적전귀장기분위호전조화사망조，통계발현사망조재치료기간LDH화IBIL명현고우호전조。15례환자재치료과정중접수혈장치환치료，치유솔위80％，이시부사용대제량격소충격치료대환자적전귀몰유영향。결론①정도불일적PLT감소화신경정신증상시TTP중최상견적림상표현；②외주혈파쇄홍세포적감측능위조기진단제공증거；③재치료과정중，LDH화IBIL수평적변화대전귀적판단구유지도의의；④혈장치환시치료TTP적유효수단，단대제량격소치료대예후병무영향。
Objective To retrospectively analyze 19 cases with thrombotic thrombocytopenic purpura ( TTP) during the past 12 years, and evaluate the relationship between the clinical features , therapy methods and prognosis . Methods In order to understand the influencing factors on the prognosis of the TTP patients , clinical data of 19 patients were analyzed , including the clinical features , therapy methods, hemoglobin, platelet, lactate dehydrogenase (LDH) and indirect bilirubin (IBIL). Results There were different degree of platelet reducing and neurological manifestations .As the disease developing , the hemoglobin decreased .The degrees of fever and renal injure were inconformity .The patients were divided into two groups according the prognosis; the LDH and IBIL of dead group were higher than favourable group in the course of therapy .Fifteen cases received plasma exchange , and survival ratio was 80%.And the glucocorticoid impact treatment had no influence on the prognosis . Conclusion ①The most common features of TTP are platelet reducing and neurological manifestations ,②fractured red blood cells can provide the clue for diagnosis , ③in the therapy course , the changes of LDH and IBIL can indicate prognosis ,④plasma exchange is a effective treatment for TTP , and the high dose glucocorticoid therapy has no influence on prognosis .