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.     

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.     

胸腺瘤合并单纯红细胞再生障碍性贫血(附5例报道)

目的 探讨胸腺瘤合并单纯红细胞再生障碍性贫血（PRCA）的临床特征及外科治疗方法.方法 回顾分析1979年至2004年间5例胸腺瘤合并PRCA病人的临床资料.结果 男2例,女3例;年龄43～68岁,平均54.6岁,均获根治性切除.胸腺瘤合并PRCA者占同期胸腺瘤病人的2.7%（5/185例）.术后早期PRCA情况明显改善,术后长期生存4例,1例复发死亡.结论 胸腺瘤合并PRCA是一种少见疾病,外科治疗是本病的首选治疗手段.对单纯红细胞再生障碍性贫血不能缓解者给予激素和免疫抑制剂治疗,可望取得较好疗效.     

Azathioprine-induced pure red cell aplasia: case report and review

The major adverse effect of azathioprine is its myelotoxicity, with leukocytes being affected more commonly than the other bone marrow elements. Although megaloblastic change is frequent, reportedly seen in 16% to 82% of bone marrow aspirates, long-term use of azathioprine rarely causes severe anemia. We report a case of refractory pure red cell aplasia resulting from long-term use of azathioprine in a renal transplant recipient and examine the possible underlying mechanisms. There was no response to dose reduction or to erythropoietin administration. However, there was immediate recovery after complete drug withdrawal. A review of the literature revealed that only ten cases of azathioprine-induced red cell aplasia have so far been described, all in transplant recipients.     

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。     

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。     

Invasive thymoma involving the liver: a case report of transdiaphragmatic extension]

A 38-year-old woman had an invasive thymoma which disseminated into the thoracic cavity and infiltrated the lateral liver surface. Such transdiaphragmatic extension by an invasive thymoma is rare, however, routine abdominal CT should be performed in invasive thymoma cases to avoid underestimating the extent of disease.     

Surgery for the thymoma combined with pure red cell aplasia and myasthenia gravis

Pure red cell aplasia (PRCA) and myasthenia gravis (MG) are respectively combined with thymoma, however, these 3 complications are extremely rare coexisted as a clinical triad. A 73-year-old female with mediastinal tumor found in 2000 was pointed out anemia in June 2002. As PRCA was diagnosed by the bone marrow examination, blood transfusion had been performed. By a chest computed tomography (CT), a thymoma in size of 7 x 5 cm in diameter was recognized in the anterior mediastinum. The serum level of anti-acetylcholine receptor antibody was elevated to be 35 nmol/l. MG was simultaneously diagnosed with a decreased power of neck muscle. The extended thymectomy was performed in August 2002, and pathological diagnosis disclosed a 'type AB' by World Health Organization (WHO) classification. After the operation, the decreased power of neck muscle had been improved, however, PRCA had not been remitted in the early-postoperative term. Blood transfusion had been required (2-4 units/1-2 weeks) for the postoperative 7 months' term. A cyclosporin (250 mg/day) as an adjuvant therapy was administered in April 2003. One month later, the patient's serum level of Hb had been over 10 g/dl without blood transfusion. The patient has been followed up with reducing the dose of cyclosporin. Conclusions: Surgery for a thymoma combined with PRCA and MG was effective for MG but not for PRCA in an early-postoperative term, however, a multimodality therapy with immunosuppressant as a postoperative adjuvant should bring a favorable outcome to patient's clinical data, and the postoperative long-observation must be critical in this case.     

A case of pure red cell aplasia appearing after thymectomy]

We present a case of pure red cell aplasia appearing four months after thymectomy for an invasive thymoma. A 61-year-old female whose chest X-ray demonstrated an anterior mediastinal mass was admitted to our hospital. Preoperative examination revealed neither anemia nor myasthenia gravis. During surgery, as it was found that the thymoma had invaded the upper left lobe of the lung and the left diaphragmatic nerve, these tissues were partially resected along with the thymus and the parathymic adipose tissue. Histologically the epithelial cells of the thymoma had round-oval nuclei with predominant lymphatic infiltration. There were some clusters of epithelial cells in the adjacent involuted thymic tissues. After postoperative radiotherapy of 40 Gy, the patient was discharged. About four months after the operation, she was readmitted because of anemia. Bone marrow aspiration demonstrated few erythroblasts but normal findings of granulopoiesis. Chest CT and radioisotopic examination revealed neither metastases nor recurrence of thymoma. Her anemia showed marked improvement by steroid therapy. After she was discharged, her condition has been uneventful in spite of tapering the steroid dose for seven months.     

A case of pure red cell aplasia with hypogammaglobulinemia appearing after thymo-thymectomy]

We present a case of 83-year-old woman with pure red cell aplasia appearing eight months after thymo-thymectomy for an invasive thymoma. She underwent thymo-thymectomy for an invasive thymoma in July 1996. Preoperative examination revealed neither anemia nor hypogammaglobulinemia. About eight months after the operation, she was readmitted because of anemia and hypogammaglobulinemia. Bone marrow aspiration revealed absence of erythroblasts and chest CT revealed norecurrence of thymoma. Her anemia had responded to ciclosporin.     

Bronchocentric granulomatosis associated with pure red cell aplasia and lymphadenopathy.

Bronchocentric granulomatosis developed in a patient with previously diagnosed pure red cell aplasia and lymphadenopathy. There was an excellent response to corticosteroid treatment. An immunological pathogenesis common to bronchocentric granulomatosis and pure red cell aplasia is suggested.     

Epoetin-induced autoimmune pure red cell aplasia

During the first 10 yr of therapy with recombinant human erythropoietin ([EPO]), only three cases of antibody-associated pure red cell aplasia have been described in patients who were treated with EPO, whereas several millions of patients have received this treatment. Thus, the possibility for epoetin to induce the formation of anti-EPO antibodies was considered extremely low. However, since 1998, a significant increase in the number of cases of EPO-induced pure red cell aplasia has been found in patients with chronic kidney disease with a peak in 2001 and 2002. The incidence rate seems now to be back to the baseline level. The change in formulation of epoetin a sold outside the United States seems to be the cause of these antibodies.     

True thymic hyperplasia causing pure red cell aplasia: a case report

Pure red cell aplasia caused by true thymic hyperplasia is extremely rare. We report the case of a 25-year-old female diagnosed with pure red cell aplasia. Following a thymectomy confirming true thymic hyperplasia and corticosteroid therapy, complete response was achieved. Patients diagnosed with pure red cell aplasia should be investigated with a computerized tomographic scan to assess for thymic pathology and if present, this should be resected. Follow-up is essential to monitor for recurrence.     

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.