血栓性微血管病的診治(存儲(chǔ)版)
【正文】 發(fā)于SLE，進(jìn)行性系統(tǒng)性硬化癥，腫瘤，HIV感染 藥物相關(guān)性〔抵克立得、奎寧等〕 家族性〔罕見(jiàn)〕,第二十九頁(yè)，共五十四頁(yè)。nxu232。) 患者凝血功能檢查多正常，但反復(fù)發(fā)作型患者緩解期的血清中常有巨大vWF多聚體 TTP患者血小板減少較HUS為重，TTP發(fā)作期血小板計(jì)數(shù)可降至20109/L以下 溶血導(dǎo)致LDH水平增高，并可作為疾病活動(dòng)的指標(biāo),第三十五頁(yè)，共五十四頁(yè)。o)及預(yù)后,皮質(zhì)激素推薦劑量靜脈注射甲潑尼龍(1g/d，沖擊治療3天成人劑量), 過(guò)渡到口服潑尼松龍(1mg/kg/d) 免疫抑制劑可以防止自身抗體(k224。o)，預(yù)后良好， 惡性腫瘤相關(guān)性和骨髓移植后相關(guān)性TTP，血漿置換無(wú)效，預(yù)后不佳，可以考慮使用蛋白質(zhì)A柱狀免疫吸附 重組ADAMTS13可作為先天性或無(wú)抑制物的獲得性TTP患者的選擇治療 對(duì)于存在高滴度抑制物的TTP患者那么需加強(qiáng)免疫抑制治療 血小板計(jì)數(shù)和LDH水平雖不作為TTP診斷依據(jù)，但可作為TTP療效判斷和復(fù)發(fā)監(jiān)測(cè)的重要指標(biāo),第三十九頁(yè)，共五十四頁(yè)。jiāng)輸注是復(fù)發(fā)或慢性TTP治療的首選方法 有個(gè)例報(bào)道長(zhǎng)春新堿、環(huán)磷酰胺或硫唑嘌呤可使TTP病情緩解 難治性/復(fù)發(fā)性TTP應(yīng)該給予利妥昔單抗〔美羅華，靶向CD20陽(yáng)性B細(xì)胞的嵌合性單抗〕 血小板減少和溶血嚴(yán)重，藥物治療無(wú)效可考慮行脾切除術(shù),第四十一頁(yè)，共五十四頁(yè)。ow249。n)修訂,首次將ADAMTS13活性及其抑制物水平指標(biāo)納入TTP診斷 遺傳性TTP患者ADAMTS13活性降低(ji224。,PTTM,3 cases of PTTM that presented as the initial manifestation of metastatic gastric adenocarcinoma with TMA and pulmonary infiltrates moderate thrombocytopenia without renal failure, hemolysis with extremely high serum lactate dehydrogenase levels, leukoerythroblastosis in peripheral blood smear, and interstitial pulmonary infiltrates. All patients died within 2 weeks of diagnosis. Two cases were initially misdiagnosed as idiopathic thrombotic thrombocytopenic purpura and treated with plasma exchange with no response. One patient had bone marrow infiltration by malignant cells.,Medicine〔Baltimore).2022 Nov；93〔24〕：35963,第五十頁(yè)，共五十四頁(yè)。腎小動(dòng)脈可見(jiàn)血管腔內(nèi)血栓形成及管腔閉塞，進(jìn)而引起局灶或廣泛的腎內(nèi)質(zhì)壞死。Thank you,第五十四頁(yè)，共五十四頁(yè)。ir243。,PTTM,a man in 60s underwent total gastrectomy for advanced gastric cancer with paraaortic lymph node metastases. pulmonary hypertension and rightsided heart failure developed, died of sudden cardiopulmonary arrest 30 hours after admission autopsy revealed widespread tumor embolism, fibrocellular intimal proliferation, and thrombus formation in the small arteries PTTM should be considered as differential diagnosis for acute dyspnea or pulmonary hypertension in patients with carcinoma,Nihon Shokakibyo Gakkai Zasshi.2022 Jul。ng)的標(biāo)準(zhǔn)必須包
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