胸腺瘤合并单纯红细胞再生障碍性贫血四例治疗体会

我科2001年至2006年间共收治4例胸腺瘤合并单纯红细胞再生障碍性贫血(PRCA)患者,现结合文献分析报告如下. 临床资料 1.一般资料:本组4例,男3例,女1例,年龄38～55岁,平均46.5岁.     

外科治疗胸腺瘤合并单纯红细胞再生障碍性贫血(附5例报告及国内文献复习)

目的：探讨国人胸腺瘤合并单纯红细胞再生障碍性贫血（PRCA）的临床特征及其外科治疗效果。方法：总结1980年至1997年间5例胸腺瘤合并PRCA病人接受胸腺瘤手术治疗的资料，并通过中国生物医学文献数据库光盘进行文献检索，对中国大陆已报道的这类病例进行文献复习和讨论。结果：本组胸腺瘤合并PRCA者占同期胸腺瘤病人的8．3％（5／60例）。全部均经胸骨正中切口切除胸腺瘤及胸腺组织和前纵隔脂肪，术后45d死亡1例，PRCA复发2例，长期生存2例，迄今为止，国内共检索出此类病人21例，术后早期PRCA情况明显改善，按术后随访满2年，且无PRCA复发征象作为手术有效标准，有效率为38．5％，结论：胸腺瘤合并PRCA是一种少见疾病，其预后取决于PRCA的缓解程度，胸腺瘤切除术对治疗PRCA是有效的，外科治疗是本病的首选治疗手段，也是其他综合性治疗方法的前提和基础。     

胸腺瘤合并单纯红细胞再生障碍性贫血临床特点(附3例报告)

目的探讨胸腺瘤合并纯红再障的诊断和治疗。方法3例胸腺瘤合并纯红再障患者,均手术切除胸腺瘤,并于术后常规胸腺区放疗,观察术后纯红再障的缓解情况。对不能缓解者,给予皮质激素及免疫抑制剂治疗。结果2例术后达临床治愈,1例术后给予激素及免疫抑制剂治疗取得缓解或明显进步。结论胸腺瘤切除-术后胸腺区放疗-皮质激素及免疫抑制剂的运用是胸腺瘤合并纯红再障患者较理想的治疗模式。     

胸腺瘤合并重症肌无力及单纯红细胞再生障碍性贫血二例

例1女,63岁。主诉全身乏力,眼睑下垂3年,发现贫血4个月。入院前已诊断为重症肌无力,在院外口服吡啶斯的明治疗,曾因严重呼吸困难行气管切开2次。4个月前发现贫血,血红蛋白(Hb)最低为28g/L。经外周血象及骨髓象检查诊断为单纯红细胞再生障碍性贫血,经内科系统治疗,并给予输血,效果不明显。胸部CT示:前上纵隔有占位病变,5cm×5cm×4cm大小,边缘清晰,略呈分叶状,向左侧胸腔内突出,密度均匀。白细胞4.8×109/L,红细胞1.2×1012/L,Hb 34g/L,血小板118×109/L。临床诊断:胸腺瘤合并重症肌无力及单纯红细胞再生障碍性贫血,收住我科行手术治疗。…     

获得性纯红细胞再生障碍性贫血20例报告

报告获得性纯红细胞再生障碍性贫血２０例，男９例，女１１例，年龄１－５９岁（中位数２５岁）。继发性１５例，其中有感染史７例，有药物史７例，合并胸腺瘤１例。原发性５例。所有病例血象，骨髓象及临床表现均符合本病特点。经综合治疗１３例获得缓解，５例无效，２例死亡。     

肾移植患者术后应用硫唑嘌呤致单纯红细胞再生障碍性贫血

１３例肾移植受者术后因用硫唑嘌呤（Ａｚａ）致单纯红细胞再生障碍性贫血（简称纯红再障），７便发生于开始口服Ａｚａ后２－６周，６例发生于停用环孢素Ａ（ＣｓＡ），Ａｚａ加量后３－５周。其中１１例经停用Ａｚａ并辅以对症治疗而治愈，２例仅Ａｚａ减量，加用环磷酰胺无效，后导致骨髓衰竭，因败血症死。认为Ａｚａ所致的纯红再障的停用Ａｚａ后一般均以，积极改善骨髓造血功能、延长ＣｓＡ的用药时间以及避免Ａｚａ突然增量等     

肺癌合并再生障碍性贫血3例

肺癌合并再生障碍性贫血３例李建业，崔若恒，沈尔兢我们从１９８６年以来共收治３例肺癌合并再生障碍性贫血（再障）病人，均经手术和骨髓穿刺检查证实，现报告如下：例１男，５５岁。乏力、牙龈出血２个月。查体：除明显贫血貌外无其它异常发现。Ｘ线胸片示右肺下叶３ｃ...     

妊娠合并再生障碍性贫血的治疗（附3例病例分析）

目的 探讨妊娠合并再生障碍性贫血的治疗。方法 总结自1986年以来确诊的3例妊娠合并再生障碍性贫血患者的处理。结果 第1例患者孕38^ 2周因骨盆出口狭窄行剖宫产术，术后持续发热，抗炎治疗后好转；第2例患者引产失败后于孕41^ 1周行剖宫产术，术后宫腔积血并发感染，予抗炎、双侧子宫动脉栓塞及蓉生静丙治疗后好转；第3例患者因重度妊高征孕36周在全麻下行剖宫产术及次全子宫切除术，术后恢复顺利。结论 大剂量蓉生静丙预防感染和剖宫产同时子宫切除预防产后出血是妊娠合并再障患者处理的有效手段。     

造血干细胞移植治疗再生障碍性贫血(附视频)

再生障碍性贫血(简称再障)是一种获得性骨髓造血功能衰竭症。免疫抑制治疗或异基因造血干细胞移植是治愈重型再障的必要手段,对于年龄≤40岁的重型再障患者应首选HLA相合的同胞供者骨髓移植。30年来,随着移植技术的发展,重型再障患者的预后获得明显改善。本文就造血干细胞移植治疗再障的适应证及相关进展做一简要评述。     

Anterior mediastinal exenteration for thymoma associated with pure red cell aplasia

A 46-year-old female bad thymoma associated with pure red cell aplasia, a relatively uncommon entity. Anterolateral thoracotomy in supine position offered excellent exposure for exenteration of anterior mediastinum. Steroids and cyclophosphamide were administered postoperatively. She remains stable one year after surgery. The incidence, clinical features, pathology, pathogenesis, management and prognosis of thymoma with pure red cell aplasia are reviewed.     

Invasive thymoma associated with pure red cell aplasia and liver metastasis: a case report]

A case of invasive thymoma associated with pure red cell aplasia and liver metastasis was reported. A 57-year-old male was admitted to our hospital because of hepatic abnormal shadow on computed tomography. Malignant tumor was suspected by imaging procedures. Left lateral segmental resection of liver was performed and histo-pathological examination proved the tumor to be liver metastasis of thymoma. He was received 50 Gy irradiation after incomplete resection of thymoma. In the course of time he contracted pure red cell aplasia. But he is well controlled medically and alive 7 years after the surgery.     

A case of thymoma with pure red cell aplasia]

A 71-year-old man was admitted to the hospital because of general fatigue. There were few reticulocytes in the peripheral blood and no erythroblasts in the bone marrow. Chest CT revealed an anterior mediastinal tumor. Under a diagnosis of thymoma with PRCA, extended thymothymectomy was performed. Histological diagnosis was mixed type thymoma with no invasive growth beyond the capsule. Administration of predonisolone following surgery was not effective for PRCA. Otherwise, peripheral blood counts were significantly improved following occasional onset of acute bronchitis.     

Thymothymectomy for the thymoma with pure red cell aplasia; report of a case

We report a case of thymothymectomy for the thymoma with pure red cell aplasia (PRCA). A 31-year-old male with a general fatigue had a severe anemia (hemoglobin 3.1 g/dl) since November 1997. By the bone marrow examination, PRCA was diagnosed and treated with blood transfusion and immunosuppressive drug (cyclosporin: CYA) administration but anemia had not been improved. The chest computed tomography displayed a 3 cm in a diameter of thymoma located in the anterior mediastinum. The extended thymothymectomy had been performed in February 1998, pathological detection disclosed Masaoka classification stage I, type AB was diagnosed due to the World Health Organization (WHO) classification. PRCA had not obtained an immediate remission during the postoperative-early term, while, adjuvant therapy (CYA 300 mg/day) has been continued and it brought a complete remission of PRCA in August 2001 (after the postoperative 3 years and 6 months later). Conclusions: Even though only thymothymectomy for thymoma with PRCA showed no effectiveness for the postoperative-early remission of PRCA, however, the combination of thymectomy and the postoperative adjuvant therapy (CYA) should bring a better outcome, and the continuous follow-up would be required for a long postoperative term.     

Diphenylhydantoin-induced pure red cell aplasia after neurological surgery: report of two cases

Pure red cell aplasia (PRCA) is characterized by severe anemia, reticulocytopenia, marked selective hypoplasia of the erythroid precursor cells in the bone marrow and normal peripheral leukocyte and platelet counts. We reported two cases of diphenylhydantoin (DPH)-induced PRCA after neurological surgery. They recovered completely with discontinuance of DPH therapy. Neurosurgeons should be aware of this side effect because most reported cases of PRCA have occurred about a few months after neurological surgery under general anaesthesia and DPH therapy.