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Journal of Medical Case Reports

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Open Access 01-12-2023 | Thalassemia | Case report

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

Authors: Ali Hossein Samadi Takaldani, Nima Javanshir, Helia Honardoost, Mohammad Negaresh

Published in: Journal of Medical Case Reports | Issue 1/2023

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.

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Title: Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report
Authors: Ali Hossein Samadi Takaldani
Nima Javanshir
Helia Honardoost
Mohammad Negaresh
Publication date: 01-12-2023
Publisher: BioMed Central
Keywords: Thalassemia
Pulmonary Hypertension
Pulmonary Hypertension
Pulmonary Hypertension
Pulmonary Hypertension
Published in: Journal of Medical Case Reports / Issue 1/2023
Electronic ISSN: 1752-1947
DOI: https://doi.org/10.1186/s13256-023-04257-6

At a glance: The STEP trials

Springer Medicine

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

Abstract

Background

Case report

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Case report

Conclusion

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