Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report

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. In this report,...

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Published in:Journal of medical case reports 2023-12, Vol.17 (1), p.503-503, Article 503
Main Authors: Takaldani, Ali Hossein Samadi, Javanshir, Nima, Honardoost, Helia, Negaresh, Mohammad
Format: Article
Language:English
Subjects:
Adult
Anemia
beta-Thalassemia - complications
Blood diseases
Blood transfusion
Blood transfusions
Bone marrow
Case Report
Classification
COVID-19
Disease management
Extramedullary hematopoiesis
Female
Genetic disorders
Hematopoiesis, Extramedullary
Hemoglobin
Humans
Hypertension, Pulmonary - etiology
Intermediate beta thalassemia
Iron
Lungs
Medical research
Mortality
Osteoporosis
Patients
Polymerase chain reaction
Pulmonary arteries
Pulmonary embolisms
Pulmonary hypertension
Pulmonology
Ribs
Spinal cord
Thalassemia
Thalassemia - complications
Tomography
Vein & artery diseases
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description 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. 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. 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.
doi_str_mv 10.1186/s13256-023-04257-6
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subjects Adult
Anemia
beta-Thalassemia - complications
Blood diseases
Blood transfusion
Blood transfusions
Bone marrow
Case Report
Classification
COVID-19
Disease management
Extramedullary hematopoiesis
Female
Genetic disorders
Hematopoiesis, Extramedullary
Hemoglobin
Humans
Hypertension, Pulmonary - etiology
Intermediate beta thalassemia
Iron
Lungs
Medical research
Mortality
Osteoporosis
Patients
Polymerase chain reaction
Pulmonary arteries
Pulmonary embolisms
Pulmonary hypertension
Pulmonology
Ribs
Spinal cord
Thalassemia
Thalassemia - complications
Tomography
Vein & artery diseases
title Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report
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