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。     

58例胸腺瘤患者的外科治疗及预后

胸腺瘤常合并重症肌无力(MG)、单纯红细胞再生障碍性贫血(PRCA)等自身免疫性疾病;同时,胸腺瘤又具有局部外侵、复发和远处转移、复杂的显微镜下结构、病理与预后不相一致等特点.结合我院手术治疗的58例胸腺瘤患者的临床资料和随访结果,现就胸腺瘤的外科治疗和预后进行讨论.     

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.     

纵隔、胸腔血管

胸腺瘤合并单纯红细胞再生障碍性贫血（附5例报道）,电视纵隔镜手术128例,外科治疗29例胸部动脉瘤的临床分析,异种胸腺修饰对淋巴细胞功能的影响,应重视胸交感神经切断术治疗手汗症并发症的防治（专家论坛）,胸腔镜胸腺扩大切除治疗重症肌无力的远期疗效分析,[编者按]     

罗沙司他联合免疫抑制剂治疗尿毒症合并纯红细胞再生障碍性贫血一例

重组人促红细胞生成素（rhuEPO）是治疗尿毒症肾性贫血的最常用药物,但rhuEPO可引起纯红细胞再生障碍性贫血（PRCA）。尿毒症合并PRCA临床上较为少见。我们报告1例用罗沙司他联合免疫抑制剂治疗尿毒症合并PRCA病例,患者贫血得到快速纠正,未见明显不良反应,出院后随访期间无复发。     

合并纯红细胞再生障碍性贫血的尿毒症患者肾移植二例

贫血是尿毒症患者的常见并发症，近年来，应用重组人促红细胞生成素使贫血的症状大为缓解。但在2002年，国外研究发现，使用促红细胞生成素的尿毒症患者可发生少见的纯红细胞再生障碍性贫血（PRCA），每年发病率为0．19‰。过去治疗尿毒症合并PRCA的患者主要是停用促红细胞生成素，使用免疫抑制剂抑制抗体生成，但效果欠佳。有报道，肾移植可纠正尿毒症合并PRCA患者的贫血症状。我院2例尿毒症合并PRCA的患者肾移植后贫血被纠正，现报告如下。     

α-红细胞生成素致纯红细胞再生障碍性贫血二例报告及文献复习

终末期肾衰期(ESRD)合并纯红细胞再生障碍性贫血(PRCA)临床少见，因使用α-红细胞生成素(EPO)治疗。肾性贫血而出现PRCA国内罕有报道。现将我院近年发现的2例ESRD伴PRCA病例报道如下，并结合文献进行分析。     

胸腺瘤的外科治疗体会

胸腺瘤约占原发性纵隔肿瘤的9％～17％，大约95％的胸腺瘤发生在纵隔以外的部位，如颈部、肺门和肺实质内。作为最常见的伴发疾病，约30％～65％的患者伴有重症肌无力(MG)，其他伴发疾病有单纯红细胞再生障碍性贫血(PRCA)，低丙种球蛋白血症(HG)，系统性红斑狼疮(SLE)，甲状腺炎等自身免疫性疾病。我科自1998年2月～2004年5月行手术治疗胸腺瘤患者12例，     

а-红细胞生成素致纯红细胞再生障碍性贫血二例报告及文献复习

终末期肾衰期(ESRD)合并纯红细胞再生障碍性贫血(PRCA)临床少见,因使用α-红细胞生成素(EPO)治疗肾性贫血而出现PRCA国内罕有报道。现将我院近年发现的2例ESRD伴PRCA病例报道如下,并结合文献进行分析。 一、临床资料 1.例1:女性,35岁,病案号147367,因反复蛋白尿10年,透析1年半,乏力、气喘半年于2000年6月7日入院。患者于1989年体检发现蛋白尿,     

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。     

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。     

Thymoma with pure red cell aplasia and Good's syndrome

Thymoma patients with pure red cell aplasia (PRCA) or hypogammaglobulinemia (Good's syndrome) are rare, whereas those with both PRCA and Good's syndrome are even rarer. Here we present the case report of a 70-year-old woman with invasive thymoma and simultaneous PRCA and Good's syndrome, who achieved complete PRCA remission after thymectomy.     

Renal and thymic pathology in thymoma-associated nephropathy: report of 21 cases and review of the literature.

BACKGROUND: Acquired thymic disease (malignant thymoma or thymic hyperplasia) is associated with various autoimmune diseases, such as myasthenia gravis (MG), pure red-cell aplasia (PRCA), pemphigus vulgaris or systemic lupus erythematosus (SLE). Renal disease has rarely been observed in association with thymoma. METHODS: This retrospective, multicentric study collected data on patients with thymic disease and biopsy-proven renal involvement. RESULTS: Twenty-one patients were studied (age: 49+/-14 years; male/female ratio: 8/13). Thymic pathology revealed mostly high-grade malignant thymoma (B2 and AB type); two cases were associated with non-malignant thymic hyperplasia. MG was found in nine out of 21 cases, SLE in three, PRCA in three and pemphigus in two. In 47% of these cases, nephropathy occurred after curative treatment of thymoma (108+/-83 months; range: 8-180 months), mainly based on surgical thymectomy associated with radiotherapy. Clinical and laboratory findings included nephrotic syndrome (75%), renal failure (50%), frequent presence of antinuclear antibodies and hypogammaglobulinaemia. Renal pathology showed minimal change disease in 14 patients and focal segmental glomerulosclerosis (FSGS) in one. Membranous nephropathy was observed in four cases, ANCA-associated glomerulonephritis in two and thrombotic microangiopathy in one. Most patients with minimal change disease or FSGS (11/13) were steroid-sensitive. Despite good response to steroids, 38% of patients died from thymoma and 17% developed end-stage renal failure. CONCLUSIONS: Glomerulopathy can be associated with thymoma or thymic hyperplasia. The present series shows that minimal change disease is the most frequent thymoma-associated glomerular lesion and that it may occur several years after thymectomy.     

Thymoma

OBJECTIVE: We evaluated the prognostic factors for thymoma that remain controversial. METHODS: We studied 72 consecutive patients treated for thymoma during the period between 1966 and 1997. Recurrence-free interval rates and overall survival rates calculated by the Kaplan-Meier method were compared using log-rank test by the Masaoka stage, extent of surgical resection, histology, or associated disease(s). Multivariate analysis was performed using Cox's proportional hazards model. RESULTS: Thirty-two thymomas were at Masaoka stage I, 9 at stage II, 15 at stage III, and 16 were at stage IV. There were 56 complete resections, 7 incomplete resections (2 at stage III and 5 at stage IV), and 9 biopsies (1 at stage III and 8 at stage IV). Forty-one thymomas were cortical, 16 medullary, and 15 were mixed form. Association of myasthenia gravis was found in 20 patients, and pure red cell aplasia in 7. After an average follow-up period of 103 months, the recurrence-free 5-, 10-, 15-year interval rate was 89%, 80%, 80%, respectively, and overall 5-, 10-, 15-year survival rate was 86%, 71%, 59%, respectively. Factors influencing the recurrence-free interval and overall survival included the Masaoka stage, extent of surgical resection, and association with pure red cell aplasia. Multivariate analysis revealed stage IV tumor and association with pure red cell aplasia as risk factors for recurrence. Pure red cell aplasia indicated poor prognosis for overall survival. CONCLUSIONS: Masaoka stage, extent of surgical resection, and association with pure red cell aplasia were prognostic factors for thymoma. Multidisciplinary treatment for stage IV tumors and better control of pure red cell aplasia, if associated, should be investigated.     

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.     

Thymoma

Objective: We evaluated the prognostic factors for thymoma that remain controversial.Methods: We studied 72 consecutive patients treated for thymoma during the period between 1966 and 1997. Recurrence-free interval rates and overall survival rates calculated by the Kaplan-Meier method were compared using logrank test by the Masaoka stage, extent of surgical resection, histology, or associated disease(s). Multivariate analysis was performed using Cox's proportional hazards model.Results: Thirty-two thymomas were at Masaoka stage I, 9 at stage II, 15 at stage III, and 16 were at stage IV. There were 56 complete resections, 7 incomplete resections (2 at stage III and 5 at stage IV), and 9 biopsies (1 at stage III and 8 at stage IV). Forty-one thymomas were cortical, 16 medullary, and 15 were mixed form. Association of myasthenia gravis was found in 20 patients, and pure red cell aplasia in 7. After an average follow-up period of 103 months, the recurrence-free 5-, 10-, 15-year interval rate was 89%, 80%, 80%, respectively, and overall 5-, 10-, 15-year survival rate was 86%, 71%, 59%, respectively. Factors influencing the recurrence-free interval and overall survival included the Masaoka stage, extent of surgical resection, and association with pure red cell aplasia. Multivariate analysis revealed stage IV tumor and association with pure red cell aplasia as risk factors for recurrence. Pure red cell aplasia indicated poor prognosis for overall survival.Conclusions: Masaoka stage, extent of surgical resection, and association with pure red cell aplasia were prognostic factors for thymoma. Multidisciplinary treatment for stage IV tumors and better control of pure red cell aplasia, if associated, should be investigated.     

Concomitant thymectomy and cardiac operation in a patient with pure red cell aplasia

Pure red cell aplasia is a rare condition resulting in severe anemia. Medical therapy is indicated, unless a thymoma is present. In patients with concurrent cardiac pathology requiring operation, simultaneous operation should be contemplated to avoid risky resternotomy. We describe an exceptionally rare case of a patient with pure red cell aplasia secondary to a thymoma who underwent concomitant thymectomy and coronary artery grafting with a successful surgical outcome.     

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.     

Thyroid maltoma: report of a case without coexistent Hashimoto's thyroiditis

Maltomas of the thyroid are rare neoplasms that originate from parafollicular B lymphocytes and are often associated with autoimmune thyroiditis (Hashimoto's disease). The authors describe a case of a primary thyroid lymphoma in which no evidence for a previous thyroiditis could be found during clinical evaluation (clinical progression of the tumor, diagnostic studies, preoperative cytology, histology of the surgical specimen). Noteworthy was the development of a severe form of pure red cell aplasia (PRCA) about 6 months after the operation without detectable autoantibodies or signs of previous viral infection. The aplasia resolved completely with corticosteroid therapy.