目的探讨血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)的临床特点及预后因素。方法回顾性分析北京协和医院2012至2017年60例TTP患者的临床表现、实验室检查、诊断、治疗及转归,采用Logistic单因素及多因素回归分...目的探讨血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)的临床特点及预后因素。方法回顾性分析北京协和医院2012至2017年60例TTP患者的临床表现、实验室检查、诊断、治疗及转归,采用Logistic单因素及多因素回归分析危险因素对预后的影响,采用K多个独立样本检验及Mann-Whitney U检验分析不同治疗方案对预后的影响。结果 60例患者中,男性17例(28.3%,17/60),女性43例(71.7%,43/60),平均年龄(41±15)岁。28例患者(46.7%,28/60)表现为发热、微血管病性溶血性贫血、血小板降低三联征,23例(38.3%,23/60)表现为发热、微血管病性溶血性贫血、血小板降低、肾功能不全、神经系统异常五联征,其余9例(15.0%,9/60)无典型三联征/五联征表现。28例患者接受血管性血友病因子裂解蛋白酶13(a disintegrin and metalloproteinase with a thrombospondin type 1motif,member 13,ADAMTS13)活性检测,其中23例ADAMTS13活性<10%,阳性率为82.1%(23/28);20例患者检测了ADAMTS13抑制物,阳性率为90.0%(18/20);20例同时行ADAMTS13活性及其抑制物检测,二者均为阳性者占90.0%(18/20)。根据是否有血源、患者经济条件等不同情况,采用血浆置换、糖皮质激素、利妥昔单抗、免疫抑制剂、注射用免疫球蛋白等不同治疗方案,42例患者(70.0%,42/60)病情缓解,18例(30.0%,18/60)死亡。在血浆置换及激素治疗的基础上加用免疫抑制治疗,可将缓解率由57.1%提高至85.2%(P=0.032)。未发现导致患者死亡率增高的危险因素。结论 TTP多表现为三联征或五联征,ADAMTS13活性降低及抑制物检测阳性率高,免疫抑制治疗可改善预后。展开更多
Thrombotic thrombocytopenic purpura (TTP) is a rare but acute, life-threatening condition which may be precipitated by pregnancy. This disorder that presents with thrombocytopenia, haemolytic anemia, and clinical cons...Thrombotic thrombocytopenic purpura (TTP) is a rare but acute, life-threatening condition which may be precipitated by pregnancy. This disorder that presents with thrombocytopenia, haemolytic anemia, and clinical consequences of microvascular thrombosis such as stroke. The exact cause is not known but it is associated with a deficiency of ADAMTS13 enzymes. Immune mediated TTP is more common and can present in pregnancy. The aim of this case is to bring awareness as many clinicians are unaware of this condition in pregnancy, its diagnosis may be missed or delayed, leading to fetal loss or serious maternal implications. In this case the patient presented at 29 weeks with stroke in Emergency department, referred to delivery suit for Obstetric review, with suspicion of Pre-eclampsia/HELLP. The diagnosis of TTP was achieved by a multidisciplinary team who worked tirelessly together. The patient was transferred to a Specialist Tertiary Care Centre for further management. The pregnancy continued until 33 weeks and 5 days. She underwent an emergency caesarean section for fetal distress. Steroids and Rituximab were continued postnatally. The outcome was favourable due to fast and efficient multidisciplinary care. Awareness of this rare but important condition can lead to recognition of clinical presentation, prompt diagnosis and appropriate management.展开更多
文摘目的探讨血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)的临床特点及预后因素。方法回顾性分析北京协和医院2012至2017年60例TTP患者的临床表现、实验室检查、诊断、治疗及转归,采用Logistic单因素及多因素回归分析危险因素对预后的影响,采用K多个独立样本检验及Mann-Whitney U检验分析不同治疗方案对预后的影响。结果 60例患者中,男性17例(28.3%,17/60),女性43例(71.7%,43/60),平均年龄(41±15)岁。28例患者(46.7%,28/60)表现为发热、微血管病性溶血性贫血、血小板降低三联征,23例(38.3%,23/60)表现为发热、微血管病性溶血性贫血、血小板降低、肾功能不全、神经系统异常五联征,其余9例(15.0%,9/60)无典型三联征/五联征表现。28例患者接受血管性血友病因子裂解蛋白酶13(a disintegrin and metalloproteinase with a thrombospondin type 1motif,member 13,ADAMTS13)活性检测,其中23例ADAMTS13活性<10%,阳性率为82.1%(23/28);20例患者检测了ADAMTS13抑制物,阳性率为90.0%(18/20);20例同时行ADAMTS13活性及其抑制物检测,二者均为阳性者占90.0%(18/20)。根据是否有血源、患者经济条件等不同情况,采用血浆置换、糖皮质激素、利妥昔单抗、免疫抑制剂、注射用免疫球蛋白等不同治疗方案,42例患者(70.0%,42/60)病情缓解,18例(30.0%,18/60)死亡。在血浆置换及激素治疗的基础上加用免疫抑制治疗,可将缓解率由57.1%提高至85.2%(P=0.032)。未发现导致患者死亡率增高的危险因素。结论 TTP多表现为三联征或五联征,ADAMTS13活性降低及抑制物检测阳性率高,免疫抑制治疗可改善预后。
文摘Thrombotic thrombocytopenic purpura (TTP) is a rare but acute, life-threatening condition which may be precipitated by pregnancy. This disorder that presents with thrombocytopenia, haemolytic anemia, and clinical consequences of microvascular thrombosis such as stroke. The exact cause is not known but it is associated with a deficiency of ADAMTS13 enzymes. Immune mediated TTP is more common and can present in pregnancy. The aim of this case is to bring awareness as many clinicians are unaware of this condition in pregnancy, its diagnosis may be missed or delayed, leading to fetal loss or serious maternal implications. In this case the patient presented at 29 weeks with stroke in Emergency department, referred to delivery suit for Obstetric review, with suspicion of Pre-eclampsia/HELLP. The diagnosis of TTP was achieved by a multidisciplinary team who worked tirelessly together. The patient was transferred to a Specialist Tertiary Care Centre for further management. The pregnancy continued until 33 weeks and 5 days. She underwent an emergency caesarean section for fetal distress. Steroids and Rituximab were continued postnatally. The outcome was favourable due to fast and efficient multidisciplinary care. Awareness of this rare but important condition can lead to recognition of clinical presentation, prompt diagnosis and appropriate management.
