{"title":"脾切除成人输血依赖型地中海贫血患者的长期并发症、生存率和死亡率。","authors":"Urmimala Bhattacharjee, Alka Khadwal, Charanpreet Singh, Deepak Bansal, Amita Trehan, Thakur Deen Yadav, Arihant Jain, Gaurav Prakash, Prashant Sharma, Reena Das, Pankaj Malhotra","doi":"10.1016/j.transci.2024.104064","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Splenectomy is frequently performed in transfusion-dependent thalassemia (TDT) patients to lower blood transfusion needs but is associated with significant long-term complications, including sepsis, thrombosis, and pulmonary hypertension. This study examines the long-term complications, survival rates, and causes of mortality among adult patients with TDT who have undergone splenectomy in a low and middle-income country (LMIC).</p><p><strong>Methods: </strong>A retrospective analysis was conducted on 103 adult TDT patients (≥18 years) who underwent splenectomy between July 2013 and March 2024. Data collected included demographic and clinical characteristics, haematological parameters, transfusion requirements before splenectomy and at the last follow-up, survival rates, complications, and mortality causes.</p><p><strong>Results: </strong>The median age at splenectomy was 12 years (range 5-34). The majority (98 %) underwent open splenectomy. The yearly transfusion volume decreased from 276.7 ml/kg/year pre-splenectomy (range 207-433) to 146.2 (range 0-252.9) post-splenectomy at the last follow-up, p < 0.0001. Three patients were completely transfusion-free at the last follow-up. Complications included pulmonary hypertension in 10 (9.7 %), thrombosis in 5 (4.8 %), and overwhelming post-splenectomy infection (OPSI) in 4 (3.9 %). The iron-overload-related complications included cardiomyopathy in 17 (16.5 %), endocrinopathy in 56 (54.3 %), chronic liver disease in 15 (14.5 %) and hepatocellular carcinoma in 2 (0.9 %). The 15-year post-splenectomy overall survival (OS) was 84.7 % (95 % CI- 77.3 % - 92.8 %), with 17 deaths (16.5 %) recorded. Iron-overload-related cardiomyopathy was the leading cause of death in 8 (53.3 %).</p><p><strong>Conclusion: </strong>Splenectomy significantly reduces transfusion requirements in TDT patients but is associated with risks such as thrombosis, pulmonary hypertension, and OPSI. Long-term mortality is primarily driven by iron-overload-related cardiomyopathy.</p>","PeriodicalId":49422,"journal":{"name":"Transfusion and Apheresis Science","volume":"64 1","pages":"104064"},"PeriodicalIF":1.4000,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Long-term complications, survival and mortality in splenectomised adult transfusion-dependent thalassemia patients.\",\"authors\":\"Urmimala Bhattacharjee, Alka Khadwal, Charanpreet Singh, Deepak Bansal, Amita Trehan, Thakur Deen Yadav, Arihant Jain, Gaurav Prakash, Prashant Sharma, Reena Das, Pankaj Malhotra\",\"doi\":\"10.1016/j.transci.2024.104064\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Splenectomy is frequently performed in transfusion-dependent thalassemia (TDT) patients to lower blood transfusion needs but is associated with significant long-term complications, including sepsis, thrombosis, and pulmonary hypertension. This study examines the long-term complications, survival rates, and causes of mortality among adult patients with TDT who have undergone splenectomy in a low and middle-income country (LMIC).</p><p><strong>Methods: </strong>A retrospective analysis was conducted on 103 adult TDT patients (≥18 years) who underwent splenectomy between July 2013 and March 2024. Data collected included demographic and clinical characteristics, haematological parameters, transfusion requirements before splenectomy and at the last follow-up, survival rates, complications, and mortality causes.</p><p><strong>Results: </strong>The median age at splenectomy was 12 years (range 5-34). The majority (98 %) underwent open splenectomy. The yearly transfusion volume decreased from 276.7 ml/kg/year pre-splenectomy (range 207-433) to 146.2 (range 0-252.9) post-splenectomy at the last follow-up, p < 0.0001. Three patients were completely transfusion-free at the last follow-up. Complications included pulmonary hypertension in 10 (9.7 %), thrombosis in 5 (4.8 %), and overwhelming post-splenectomy infection (OPSI) in 4 (3.9 %). The iron-overload-related complications included cardiomyopathy in 17 (16.5 %), endocrinopathy in 56 (54.3 %), chronic liver disease in 15 (14.5 %) and hepatocellular carcinoma in 2 (0.9 %). The 15-year post-splenectomy overall survival (OS) was 84.7 % (95 % CI- 77.3 % - 92.8 %), with 17 deaths (16.5 %) recorded. Iron-overload-related cardiomyopathy was the leading cause of death in 8 (53.3 %).</p><p><strong>Conclusion: </strong>Splenectomy significantly reduces transfusion requirements in TDT patients but is associated with risks such as thrombosis, pulmonary hypertension, and OPSI. Long-term mortality is primarily driven by iron-overload-related cardiomyopathy.</p>\",\"PeriodicalId\":49422,\"journal\":{\"name\":\"Transfusion and Apheresis Science\",\"volume\":\"64 1\",\"pages\":\"104064\"},\"PeriodicalIF\":1.4000,\"publicationDate\":\"2025-01-03\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Transfusion and Apheresis Science\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1016/j.transci.2024.104064\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Transfusion and Apheresis Science","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.transci.2024.104064","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
Long-term complications, survival and mortality in splenectomised adult transfusion-dependent thalassemia patients.
Background: Splenectomy is frequently performed in transfusion-dependent thalassemia (TDT) patients to lower blood transfusion needs but is associated with significant long-term complications, including sepsis, thrombosis, and pulmonary hypertension. This study examines the long-term complications, survival rates, and causes of mortality among adult patients with TDT who have undergone splenectomy in a low and middle-income country (LMIC).
Methods: A retrospective analysis was conducted on 103 adult TDT patients (≥18 years) who underwent splenectomy between July 2013 and March 2024. Data collected included demographic and clinical characteristics, haematological parameters, transfusion requirements before splenectomy and at the last follow-up, survival rates, complications, and mortality causes.
Results: The median age at splenectomy was 12 years (range 5-34). The majority (98 %) underwent open splenectomy. The yearly transfusion volume decreased from 276.7 ml/kg/year pre-splenectomy (range 207-433) to 146.2 (range 0-252.9) post-splenectomy at the last follow-up, p < 0.0001. Three patients were completely transfusion-free at the last follow-up. Complications included pulmonary hypertension in 10 (9.7 %), thrombosis in 5 (4.8 %), and overwhelming post-splenectomy infection (OPSI) in 4 (3.9 %). The iron-overload-related complications included cardiomyopathy in 17 (16.5 %), endocrinopathy in 56 (54.3 %), chronic liver disease in 15 (14.5 %) and hepatocellular carcinoma in 2 (0.9 %). The 15-year post-splenectomy overall survival (OS) was 84.7 % (95 % CI- 77.3 % - 92.8 %), with 17 deaths (16.5 %) recorded. Iron-overload-related cardiomyopathy was the leading cause of death in 8 (53.3 %).
Conclusion: Splenectomy significantly reduces transfusion requirements in TDT patients but is associated with risks such as thrombosis, pulmonary hypertension, and OPSI. Long-term mortality is primarily driven by iron-overload-related cardiomyopathy.
期刊介绍：
Transfusion and Apheresis Science brings comprehensive and up-to-date information to physicians and health care professionals involved in the rapidly changing fields of transfusion medicine, hemostasis and apheresis. The journal presents original articles relating to scientific and clinical studies in the areas of immunohematology, transfusion practice, bleeding and thrombotic disorders and both therapeutic and donor apheresis including hematopoietic stem cells. Topics covered include the collection and processing of blood, compatibility testing and guidelines for the use of blood products, as well as screening for and transmission of blood-borne diseases. All areas of apheresis - therapeutic and collection - are also addressed. We would like to specifically encourage allied health professionals in this area to submit manuscripts that relate to improved patient and donor care, technical aspects and educational issues.
Transfusion and Apheresis Science features a "Theme" section which includes, in each issue, a group of papers designed to review a specific topic of current importance in transfusion and hemostasis for the discussion of topical issues specific to apheresis and focuses on the operators'' viewpoint. Another section is "What''s Happening" which provides informal reporting of activities in the field. In addition, brief case reports and Letters to the Editor, as well as reviews of meetings and events of general interest, and a listing of recent patents make the journal a complete source of information for practitioners of transfusion, hemostasis and apheresis science. Immediate dissemination of important information is ensured by the commitment of Transfusion and Apheresis Science to rapid publication of both symposia and submitted papers.
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