• Journal of Chest Surgery
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• 제50권2호
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• pp.119-122
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• 2017

Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.

• Journal of Yeungnam Medical Science
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• 제5권2호
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• pp.239-246
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• 1988

저자들은 diphenylhydantoin의 사용 후 발생하는 것으로 사료되는 용혈성 빈혈과 동반된 순수적혈구형성부전증 1예를 경험하였기에 간단한 문헌고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제13권3호
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• pp.306-311
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• 1980

Pure red cell aplasia is unusual cause of anemia and a selective aplastic disorder that affects the erythroid series of the bone marrow. Fifty percent of all patients with red cell aplasia will have a thymoma. Twenty-five to 30% of those who undergo thymectomy will be cured. A 57-years-old man was admitted to the medical department of Korea University hospital with complaints. Physical examination reveals a sick looking man with a pale lip, anemic conjunctiva and subicteric sclera. On auscultation, coarse breathing sound and moist rale was heard on the right lung field. Neither the liver nor spleen was palpable. A blood count showed the erythrocytes to number 2,640,000/mm3 and hemoglobin to be 7.0gm/dl. A white blood cell count was 5,000/mm3 and a platelet count was 328,000/mm3 Reticulocyte count was 0.7%. Examination of the peripheral blood smear showed the red cell, to be normocytic and normochromic. Urine sugar was three positive and GTT was positive. The anterior-posterior and lateral view of Chest X-ray was suggestive of an anterior mediastinal mass. A bone marrow biopsy reveals absence of red cell precursors and a normal myeloid series and megakaryocytes. At thoracotomy in May 1980 an encapsulated, lobulated, benign thymoma, which measured 5x7x5 cm was removed, microscopic examination showed it was of the spindle cell type. The postoperative course was uneventful, but the patient never had a return of hemoglobin to the blood. The patient was discharged on the postoperative] 3 days. At postoperative 1 month, the patient was readmitted for bone marrow study and had no return of red cells to bone marrow. At now, patient has been treated with steroid and the further follow up study will be needed.

• Tuberculosis and Respiratory Diseases
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• 제63권5호
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• pp.454-457
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• 2007

저자들은 심한 빈혈에 의한 전신무력감과 어지러움 증상을 주소로 내원한 환자에서 전종격동 종양이 관찰되어 조직검사를 시행하였으며, 흉선종에 동반된 PRCA로 진단하였다. 치료를 위해 흉선종 적출을 시행하였으며 수술 후 1년 6개월까지 더 이상의 수혈 없이 혈색소가 11.0g/dL 이상으로 유지되어 수술적 제거를 통한 흉선종에 동반된 PRCA의 완치를 경험하였다.

• Journal of Chest Surgery
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• 제17권4호
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• pp.796-803
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• 1984

Pure Red-Cell Aplasia [P.R.C.A.] is rare disease characterized by absence of erythroid precursors in the bone marrow, normocytic normochromic anemia with profound reticulocytopenia in the peripheral blood, and relatively or completely spared granulopoiesis and thrombopoiesis. The association rates of P.R.C.A. with Thymoma is approximately 50%, but only 5-10% of all patients with a Thymoma have a P.R.C.A.. P.R.C.A. is thought to be a variety of autoimmune disease, and humoral inhibitor, i.e. IgG, has been demonstrated experimentally. Its treatments such as thymectomy, immunosuppressants, steroid, androgenic hormone, and splenectomy have been tried but the result is not satisfactory and the prognosis is poor. We experienced a case of P.R.C.A. with Thymoma treated with thymectomy and postoperative steroid therapy, and which showed good postoperative recovery clinically and hematologically.

• Biomolecules & Therapeutics
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• 제18권2호
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• pp.184-190
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• 2010

Clinical cases of pure red cell aplasia (PRCA) have been reported during the recombinant human erythropoietin (EPO) therapy for the anemia patients. PRCA is a rare hematological disorder leading to a severe anemia due to an almost complete stop of red blood cell production. Antibody (Ab)-associated PRCA is caused by the EPO-neutralizing Abs that eliminate the biological activity of EPO. In order to detect anti-EPO Abs in human sera, we performed conventional ELISA, directly coated bridging ELISA, and streptavidin coated bridging ELISA, and compared their sensitivity and specificity. Some false positive results were obtained in the conventional ELISA. One positive sample was detected successfully by streptavidin coated bridging ELISA, which was not appeared in the directly coated bridging ELISA. In conclusion, streptavidin coated bridging ELISA was substantially sensitive and specific format and one out of sixty-eight serum samples was proved to be anti-EPO positive.

• 대한의생명과학회지
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• 제15권2호
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• pp.161-166
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• 2009

Human erythropoietin(EPO) is a glycoprotein that enhances red blood cell production by stimulating proliferation and differentiation of erythroid progenitor cells in the bone marrow. Patients with chronic kidney disease(CKD) suffer from anemia caused by reduced production of EPO in the kidney. Recombinant human EPO protein has been used successfully for the treatment of anemia associated with CKD. Recently, attention has been paid to the development of side effect of EPO, pure red cell aplasia(PRCA), in some patients with CKD. PRCA is a rare disorder of erythropoiesis that leads to a severe anemia due to an almost complete cessation of red blood cell production. EPO-related PRCA is caused by the production of EPO-neutralizing antibodies(Abs) that eliminate the biological activity of EPO as well as endogenous EPO in patients undergoing therapy. Since 1988, almost 200 cases worldwide have been reported with Ab-positive PRCA after receiving EPO therapeutics. The underlying mechanisms of the breaking of immune tolerance to self-EPO have been investigated. Modification of formulation, organic compounds of container closures, and route of administration has been suggested for the possible mechanism of increased immunogenicity of EPO. A number of assays have been used to detect Abs specific to EPO. These assays are generally grouped into two major categories: binding Ab assays and neutralizing Ab assays(bioassays). There are several types of binding Ab assays, including radioimmunoprecipitation assay, enzyme-linked immunosorbent assay, and the BIAcore biosensor assay. In vitro cell-based bioassays have been utilized for the detection of neutralizing Abs. Finally, the recent experience with anti-EPO Abs may have considerable implications for the future development and approval of EPO preparations. Also, considering that millions of patients are being treated with EPO, clinicians need to be aware of signs and consequences of this rare but severe clinical case.

• 대한세포병리학회지
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• 제5권2호
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• pp.189-193
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• 1994

Malignant thymoma is usually manifested as a mediastinal mass. Occasiomally, these tumors may be associated with a variety of systemic syndromes, such as myasthenia gravis, pure red cell aplasia or hypogammaglobulinemia However, it is generally accepted that thymomas rarely metastasize and the frequency of extrathoracic metastasis has been reported as 1 to 15%. In general, nuclear changes of tumor cells such as enlarged size, irregular shape and altered textures are determining factors in the assessment of the grade of malignancy. We experienced a case of malignant thymoma of anterior mediastinum in a 35-year-old woman. After 3 years, she developed recurrent malignant thymoma in the right supraclavicular area, diagnosed by aspiration biopsy cytology.