胸腺瘤合并PRCA的诊治（附4例报告）
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摘要
目的 探讨胸腺瘤合并单纯红细胞再生障碍性贫血的发病机理、临床表现、诊断方法和有效的治疗方法。方法 总结从1998年到2002年间胸腺瘤合并单纯红细胞再生障碍性贫血(pure red cell aplasia PRCA)病例4例的临床资料，并检索近年中英文文献进行回顾。结果 本组病人全部接受胸骨正中切口行胸腺瘤扩大切除术，无手术死亡，早期效果很明显。术后随访以无PRCA复发视为有效，结果2年内均无PRCA复发。结论 胸腺瘤合并PRCA是一种自身免疫性疾病，其诊断可以通过胸片，胸部CT和骨髓穿刺活检而确立，胸腺瘤扩大切除是治疗本病的主要方法，术后应辅以激素治疗。由于T淋巴细胞膜表面的CD34抗原和生长因子受体有关，因此基于生长抑制素类似物(octreotide)的药物治疗可能对顽固性的病例有效。
Objective: to understand the pathogenesis , clinical features, methodes of diagnosis of thymoma associated with pure red cell aplasia (PRCA) and to evaluate the outcome of the therapy. Methods: The clinical data of 4 cases of thymoma associated with PRCA from 1993 to 2002 and English , Chinese literature of recent
years were reviewed. Result: all of the 4 cases underwent extended thymusectomy and there was no postoperative death.The early results of extended thymusectomy was excellent. The standard of effective therapy: no recurrence of PRCA was observed in 2 years after operation.Conclusion: thymoma associated with PRCA is an autoimmune disease (AID).Bone marrow examination, radiological including CT scan examination is necessary to establish the diagnosis and extended thymusectomy after preparation with repeated blood transfusion remains the mainstay of treatment. Prolonged corticosteroid therapy leads to remission for upto 2 years after operative intervention.Suspected that it is
connected with the CD34 antigen expression on the membrane of T-cell, for CD34 antigen support the function of growth factor receptor, somatostatin analogue-based therapy ( octreotide ) may be an effective therapy in unresponsive thymomas.
Objective: to understand the pathogenesis , clinical features, methodes of diagnosis of thymoma associated with pure red cell aplasia (PRCA) and to evaluate the outcome of the therapy. Methods: The clinical data of 4 cases of thymoma associated with PRCA from 1993 to 2002 and English , Chinese literature of recent
years were reviewed. Result: all of the 4 cases underwent extended thymusectomy and there was no postoperative death.The early results of extended thymusectomy was excellent. The standard of effective therapy: no recurrence of PRCA was observed in 2 years after operation.Conclusion: thymoma associated with PRCA is an autoimmune disease (AID).Bone marrow examination, radiological including CT scan examination is necessary to establish the diagnosis and extended thymusectomy after preparation with repeated blood transfusion remains the mainstay of treatment. Prolonged corticosteroid therapy leads to remission for upto 2 years after operative intervention.Suspected that it is
connected with the CD34 antigen expression on the membrane of T-cell, for CD34 antigen support the function of growth factor receptor, somatostatin analogue-based therapy ( octreotide ) may be an effective therapy in unresponsive thymomas.
引文
1. Homan EC, Jensen DE, Sando JJ. Protein ldnase C isozyme expression in phorbol ester-sensitive and -resistant EL4 thymoma cells. J Biol Chem. 1991 Mar 25; 266(9): 5676-81.
2. Fackler MJ, Civin CI, Sutherland DR, Baker MA, May WS. Activated protein kinase C directly phosphorylates the CD34 antigen on hematopoietic cells. J Biol Chem. 1990 Jul 5; 265(19): 11056-61
3. Varley CL, Royds JA, Brown BL, Dobson PR Interleukin-1 beta induced synthesis of protein kinase C-delta and protein kinase Cepsilon in EL4 thymoma cells: possible involvement of phosphatidylinositol 3-1dnase. Exp Clin Immunogenet. 2001; 18(3): 135-42.
4． 傅晋翔 再生障碍性贫血免疫机制的研究进展 国外医学输血及血液学分册，1999，22(5)：306
5. Lastoria S, Vergara E, Palmieri G, Acampa W, Varrella P, Caraco C, Bianco RA, Muto P, Salvatore M. In vivo detection of malignant thymic masses by indium-111-DTPA-D-Phel-octreotide scintigraphy. J Nucl Med. 1998 Apr; 39(4): 634-9.
6. Lin K, Nguyen BD, Ettinger DS, Chin BB. Somatostatin receptor scintigraphy and somatostatin therapy in the evaluation and treatment of malignant thymoma. Clin Nucl Med. 1999 Jan; 24(1): 24-8.
7. Cancer Medicine e.5 Section 28. Neoplasms of the Thorax -> 90. Thymomas and Thymic -> Associated Paraneoplastic Syndromes
8. Murakawa T, Nakajima J, Sato H, Tanaka M, Takamoto S, Fukayama M. Thymoma associated with pure red-cell aplasia: clinical features and prognosis. Asian Cardiovasc Thorac Ann. 2002 Jun; 10(2): 150-4.
9. Suzuki S, Nogawa S, Tanaka K, Koto A, Fukuuchi Y, Kuwana M. Initial predictors of development of pure red cell aplasia in myasthenia gravis after thymectomy. Clin Neurol Neurosurg. 2003 Dec; 106(1): 16-8.
10． 罗辉遇 陈梓宏 余忠华 放射治疗胸腺瘤伴单纯红细胞再生障碍性贫血一例报告 中华放射肿瘤学杂志 1999年9月第7卷第3期
11. Palmieri G, Lastoria S, Montella L, Martignetti A, Lombardi G, Salvatore M, Bianco AR. Role of somatostatin analogue-based therapy in unresponsive malignant thymomas. Ann Med. 1999 Oct; 31 Suppl 2: 80-5
2. Fackler MJ, Civin CI, Sutherland DR, Baker MA, May WS. Activated protein kinase C directly phosphorylates the CD34 antigen on hematopoietic cells. J Biol Chem. 1990 Jul 5; 265(19): 11056-61
3. Varley CL, Royds JA, Brown BL, Dobson PR Interleukin-1 beta induced synthesis of protein kinase C-delta and protein kinase Cepsilon in EL4 thymoma cells: possible involvement of phosphatidylinositol 3-1dnase. Exp Clin Immunogenet. 2001; 18(3): 135-42.
4． 傅晋翔 再生障碍性贫血免疫机制的研究进展 国外医学输血及血液学分册，1999，22(5)：306
5. Lastoria S, Vergara E, Palmieri G, Acampa W, Varrella P, Caraco C, Bianco RA, Muto P, Salvatore M. In vivo detection of malignant thymic masses by indium-111-DTPA-D-Phel-octreotide scintigraphy. J Nucl Med. 1998 Apr; 39(4): 634-9.
6. Lin K, Nguyen BD, Ettinger DS, Chin BB. Somatostatin receptor scintigraphy and somatostatin therapy in the evaluation and treatment of malignant thymoma. Clin Nucl Med. 1999 Jan; 24(1): 24-8.
7. Cancer Medicine e.5 Section 28. Neoplasms of the Thorax -> 90. Thymomas and Thymic -> Associated Paraneoplastic Syndromes
8. Murakawa T, Nakajima J, Sato H, Tanaka M, Takamoto S, Fukayama M. Thymoma associated with pure red-cell aplasia: clinical features and prognosis. Asian Cardiovasc Thorac Ann. 2002 Jun; 10(2): 150-4.
9. Suzuki S, Nogawa S, Tanaka K, Koto A, Fukuuchi Y, Kuwana M. Initial predictors of development of pure red cell aplasia in myasthenia gravis after thymectomy. Clin Neurol Neurosurg. 2003 Dec; 106(1): 16-8.
10． 罗辉遇 陈梓宏 余忠华 放射治疗胸腺瘤伴单纯红细胞再生障碍性贫血一例报告 中华放射肿瘤学杂志 1999年9月第7卷第3期
11. Palmieri G, Lastoria S, Montella L, Martignetti A, Lombardi G, Salvatore M, Bianco AR. Role of somatostatin analogue-based therapy in unresponsive malignant thymomas. Ann Med. 1999 Oct; 31 Suppl 2: 80-5