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.     

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.     

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.     

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。     

Epoetin-induced pure red cell aplasia successfully treated with androgens

Pure red cell aplasia (PRCA) is a severe, non-regenerative form of anaemia, with selective erythroid aplasia of the bone marrow. It appears as a severe complication of treatment for anaemia of end-stage renal insufficiency with erythropoiesis-stimulating agents. There is no definite treatment. We described for the first time a patient with chronic renal failure and antibody-mediated PRCA successfully treated with androgens.     

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.     

Epoetin-induced pure red cell aplasia: diagnosis and treatment

PURPOSE OF REVIEW: Antibody-mediated pure red cell aplasia is now recognized as a rare complication of erythropoiesis-stimulating agent therapy. The incidence of this adverse effect peaked in 2002, but new cases still appear sporadically. The aim of this review is to discuss the latest opinions regarding the detection and management of this condition. RECENT FINDINGS: The diagnosis of classical erythropoiesis-stimulating agent induced pure red cell aplasia is made by a constellation of clinical features, including severe transfusion-dependent anaemia, reticulocytopenia, low or absent erythroblasts in the bone marrow, and the presence of circulating antierythropoietin antibodies. Recently, some cases have been reported in which the bone marrow findings show red cell hypoplasia rather than aplasia; this may represent earlier presentations of the same condition. SUMMARY: Management of pure red cell aplasia as a complication of erythropoiesis-stimulating agent therapy consists of stopping the drug and implementing an immunosuppressive regimen to reduce or abolish erythropoietin antibody production. A recent animal study suggested that a possible alternative strategy may be to administer a novel peptide-based erythropoietin receptor agonist called Hematide that does not cross react with antierythropoietin antibodies, and will allow ongoing stimulation of erythropoiesis; this is the subject of a current clinical trial.     

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.     

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.     

Antierythropoietin antibody-induced pure red cell aplasia: posttransplant evolution

Pure red cell aplasia is a rare complication of recombinant human erythropoietin (rHuEPO) treatment, which physicians should consider once the more frequent causes of hyporegenerative anemia have been excluded. To our knowledge, no pediatric cases have been described. In our patient, cyclosporin A treatment enabled a reduction in the number of transfusions and the risk of hyperimmunization. After transplantation, our patients hemoglobin level has remained normal and stable.     

Acquired pure red cell aplasia in a hemodialyzed patient

A 68-year-old male had end-stage renal disease secondary to hypertension. He was placed on chronic dialytic therapy and was given recombinant human erythropoietin (epoetin) for renal anemia. One month later, rapidly progressing anemia was noted. The anemia was unresponsive to maximal doses of epoetin and the patient soon became transfusion-dependent. Erythroid hypoplasia was demonstrated by bone marrow biopsy. A detailed search for the cause of the erythroblastopenia revealed nothing. A diagnosis of acquired pure red cell aplasia was made. The use of immunosuppressive agents improved the patient's symptoms and laboratory data. Antibodies for erythropoietin (EPO) were negative after the treatment. It is suggested that patients with EPO-resistant anemia with no obvious etiology should be examined for underlying hematologic disorders.     

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.