Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report
Abstract Background Thalassemia is a type of congenital hemoglobinopathy that falls into the category of hemolytic anemias. Extramedullary hematopoiesis is a complication of this disease, which is a mechanism to compensate for chronic anemia in these patients, and imaging is the best diagnostic meth...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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BMC
2023-12-01
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| Series: | Journal of Medical Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13256-023-04257-6 |
| _version_ | 1827590541579124736 |
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| author | Ali Hossein Samadi Takaldani Nima Javanshir Helia Honardoost Mohammad Negaresh |
| author_facet | Ali Hossein Samadi Takaldani Nima Javanshir Helia Honardoost Mohammad Negaresh |
| author_sort | Ali Hossein Samadi Takaldani |
| collection | DOAJ |
| description | Abstract Background Thalassemia is a type of congenital hemoglobinopathy that falls into the category of hemolytic anemias. Extramedullary hematopoiesis is a complication of this disease, which is a mechanism to compensate for chronic anemia in these patients, and imaging is the best diagnostic method. Case report In this report, a 36-year-old Caucasian female patient with intermediate beta thalassemia is presented who, at the time of referral, complained of exacerbated shortness of breath. Imaging showed diffuse expansion masses with soft tissue components in the ribs of both hemithoraxes, leading to the diagnosis of extramedullary hematopoiesis. Conclusion Extramedullary hematopoiesis in the ribs is an uncommon finding in patients with thalassemia and is a sign of the severity of the disease and a poor prognostic factor that might be preventable if blood transfusion begins at younger ages. |
| first_indexed | 2024-03-09T01:18:18Z |
| format | Article |
| id | doaj.art-1fb36960d81641fe91d8a6b0cb531a28 |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-03-09T01:18:18Z |
| publishDate | 2023-12-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-1fb36960d81641fe91d8a6b0cb531a282023-12-10T12:20:09ZengBMCJournal of Medical Case Reports1752-19472023-12-011711510.1186/s13256-023-04257-6Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case reportAli Hossein Samadi Takaldani0Nima Javanshir1Helia Honardoost2Mohammad Negaresh3Department of Internal Medicine (Pulmonology Division), School of Medicine, Emam Khomeini Hospital, Ardabil University of Medical SciencesFaculty of Medicine, School of Medicine, Ardabil University of Medical SciencesDepartment of Internal Medicine, School of Medicine, Ardabil University of Medical SciencesDepartment of Internal Medicine, School of Medicine, Ardabil University of Medical SciencesAbstract Background Thalassemia is a type of congenital hemoglobinopathy that falls into the category of hemolytic anemias. Extramedullary hematopoiesis is a complication of this disease, which is a mechanism to compensate for chronic anemia in these patients, and imaging is the best diagnostic method. Case report In this report, a 36-year-old Caucasian female patient with intermediate beta thalassemia is presented who, at the time of referral, complained of exacerbated shortness of breath. Imaging showed diffuse expansion masses with soft tissue components in the ribs of both hemithoraxes, leading to the diagnosis of extramedullary hematopoiesis. Conclusion Extramedullary hematopoiesis in the ribs is an uncommon finding in patients with thalassemia and is a sign of the severity of the disease and a poor prognostic factor that might be preventable if blood transfusion begins at younger ages.https://doi.org/10.1186/s13256-023-04257-6Extramedullary hematopoiesisIntermediate beta thalassemiaRibs |
| spellingShingle | Ali Hossein Samadi Takaldani Nima Javanshir Helia Honardoost Mohammad Negaresh Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report Journal of Medical Case Reports Extramedullary hematopoiesis Intermediate beta thalassemia Ribs |
| title | Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report |
| title_full | Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report |
| title_fullStr | Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report |
| title_full_unstemmed | Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report |
| title_short | Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report |
| title_sort | extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta thalassemia a case report |
| topic | Extramedullary hematopoiesis Intermediate beta thalassemia Ribs |
| url | https://doi.org/10.1186/s13256-023-04257-6 |
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