G. De Gregorio, S. D’Alessandro, G. Trapani, E. Quartuccio, A. Lo Casto, G. Evangelista
{"title":"IMRT/IGRT螺旋断层治疗:Β-thalassaemia成功治疗髓外造血所致肺实质压迫1例报告","authors":"G. De Gregorio, S. D’Alessandro, G. Trapani, E. Quartuccio, A. Lo Casto, G. Evangelista","doi":"10.33590/emjhematol/21-00205","DOIUrl":null,"url":null,"abstract":"Thalassaemia is a chronic haemolytic anaemia that is endemic in the Mediterranean Basin. Extramedullary haematopoiesis (EMH) is a natural compensatory reaction involving several organs or tissues. This report outlines a case of dyspnoea due to bilateral dorsal paravertebral EMH, which was treated successfully with helical tomotherapy, a technique that combines intensity-modulate radiation therapy with image-guided radiation therapy. By the end of the first week of treatment, an increase in the haemoglobin value (up to 7.8 g/dL) and a remarkable reduction of dyspnoea were obtained, with haemoglobin values maintained at 7.8–7.3 g/dL without further blood transfusions. At 1-year follow-up, the patient was totally asymptomatic, with a complete resolution of dyspnoea and asthenia.\n\nThe therapeutic approach to EMH remains controversial because there are no pre-established protocols, with current treatments including serial blood transfusions, hydroxyurea, radiation therapy, and surgical decompression. This clinical case description reports how helical tomotherapy may be used as a valid and effective treatment for compressive atelectasis due to EMH. In fact, radiation therapy improved the general clinical condition and self-reported symptoms of the patient and reduced the size of EMH masses visible in the chest CT scan.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":"84 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2022-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"IMRT/IGRT Helical Tomotherapy: A Successful Treatment of Lung Parenchyma Compression Due to Extramedullary Haematopoiesis in Β-thalassaemia – a Case Report\",\"authors\":\"G. De Gregorio, S. D’Alessandro, G. Trapani, E. Quartuccio, A. Lo Casto, G. Evangelista\",\"doi\":\"10.33590/emjhematol/21-00205\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Thalassaemia is a chronic haemolytic anaemia that is endemic in the Mediterranean Basin. Extramedullary haematopoiesis (EMH) is a natural compensatory reaction involving several organs or tissues. This report outlines a case of dyspnoea due to bilateral dorsal paravertebral EMH, which was treated successfully with helical tomotherapy, a technique that combines intensity-modulate radiation therapy with image-guided radiation therapy. By the end of the first week of treatment, an increase in the haemoglobin value (up to 7.8 g/dL) and a remarkable reduction of dyspnoea were obtained, with haemoglobin values maintained at 7.8–7.3 g/dL without further blood transfusions. At 1-year follow-up, the patient was totally asymptomatic, with a complete resolution of dyspnoea and asthenia.\\n\\nThe therapeutic approach to EMH remains controversial because there are no pre-established protocols, with current treatments including serial blood transfusions, hydroxyurea, radiation therapy, and surgical decompression. This clinical case description reports how helical tomotherapy may be used as a valid and effective treatment for compressive atelectasis due to EMH. In fact, radiation therapy improved the general clinical condition and self-reported symptoms of the patient and reduced the size of EMH masses visible in the chest CT scan.\",\"PeriodicalId\":326555,\"journal\":{\"name\":\"EMJ Hematology\",\"volume\":\"84 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-03-28\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"EMJ Hematology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.33590/emjhematol/21-00205\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"EMJ Hematology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.33590/emjhematol/21-00205","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
IMRT/IGRT Helical Tomotherapy: A Successful Treatment of Lung Parenchyma Compression Due to Extramedullary Haematopoiesis in Β-thalassaemia – a Case Report
Thalassaemia is a chronic haemolytic anaemia that is endemic in the Mediterranean Basin. Extramedullary haematopoiesis (EMH) is a natural compensatory reaction involving several organs or tissues. This report outlines a case of dyspnoea due to bilateral dorsal paravertebral EMH, which was treated successfully with helical tomotherapy, a technique that combines intensity-modulate radiation therapy with image-guided radiation therapy. By the end of the first week of treatment, an increase in the haemoglobin value (up to 7.8 g/dL) and a remarkable reduction of dyspnoea were obtained, with haemoglobin values maintained at 7.8–7.3 g/dL without further blood transfusions. At 1-year follow-up, the patient was totally asymptomatic, with a complete resolution of dyspnoea and asthenia.
The therapeutic approach to EMH remains controversial because there are no pre-established protocols, with current treatments including serial blood transfusions, hydroxyurea, radiation therapy, and surgical decompression. This clinical case description reports how helical tomotherapy may be used as a valid and effective treatment for compressive atelectasis due to EMH. In fact, radiation therapy improved the general clinical condition and self-reported symptoms of the patient and reduced the size of EMH masses visible in the chest CT scan.