SynthesisAnnals of medicine2022
Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review.
Synthesis in Annals of medicine, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 23 citations in OpenAlex.
- Hematological parameters of bronchopulmonary dysplasia in preterm infants: a meta-analysis.Frontiers in pediatrics · 2025Pooled it
- β-Thalassemia Minor and Pregnancy Outcomes: Pathophysiology, Clinical Implications, and Management.Medical sciences (Basel, Switzerland) · 2026Review
- Spleen granulopoiesis in psoriasis immune microenvironment aggravates psoriasis via IL-6/P-STAT3 signaling.Biology direct · 2025Article
- Paraspinal Extramedullary Hematopoiesis in a Transfusion-Dependent Beta-Thalassemia Patient: A Case Report.Cureus · 2025Article
- Acute splenomegaly, haemolysis, and thrombocytopenia following luspatercept initiation in a patient with transfusion-dependent thalassaemia: A case report.British journal of haematology · 2025Article
- Pelvic extramedullary hematopoiesis with life-threatening hemorrhage: a case report.International journal of emergency medicine · 2025Article
- MRI-T2Frontiers in medicine · 2025Article
- Idiopathic multicentric Castleman disease with marrow fibrosis and extramedullary hematopoiesis.European journal of haematology · 2024Article
- Extramedullary haematopoiesis in patients with thalassemia: a cross-sectional description of its prevalence, clinical features and survival.Hematology, transfusion and cell therapy · 2024Article
- Luspatercept's use in a patient with transfusion-dependent beta-thalassemia and intrathoracic extramedullary hematopoiesis (EMH).Clinical case reports · 2024Article
- Beta-Thalassemia Major and Myocardial Iron Overload: A Longitudinal Study with Magnetic Resonance Imaging.Cardiology research and practice · 2024Article
- A case of intrathoracic extramedullary hematopoiesis-associated pleural effusion in a beta-thalassemia intermedia patient and a short literature review.SAGE open medical case reports · 2024Article
- A Rare Case of Cord Compression From Extra-Medullary Hematopoiesis (EMH) in Beta Thalassemia.Cureus · 2023Article
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionAround 5% of the world's population is expected to have some degree and type of thalassaemia. Beta thalassaemia (BT) occurs due to a deficient production of the beta-globin chain of haemoglobin. Extramedullary haematopoiesis (EMH) is one of the complications of BT, mainly observed in minor/intermedia subtypes. EMH is the production of blood cells outside the marrow as a compensatory response to longstanding hypoxia. Due to chronic transfusions, it is not expected in patients with beta-thalassaemia major (BTM). However, there are increasingly reported cases of EMH in BTM. The incidence of EMH in BTM is thought to be <1%. We aim to pool the available data and provide cumulative evidence on the occurrence of EMH in BTM patients.
methodsThis is a systematic review of case reports, series, and retrospective studies that presented data on the occurrence of EMH in BTM patients. Data were recorded and analyzed in Microsoft Excel 2016 and SPSS 26. The protocol has been registered in PROSPERO: CRD42021242943.
resultsData from 253 cases of EMH in BTM patients were extracted with a mean age of 35.3 years. Mean haemoglobin at presentation with EMH was 8.2 mg/dL. Lower limb weakness was the most common presenting feature (
conclusionEMH is rare in BTM and can occur in any organ system with varied clinical features. MRI can effectively diagnose EMH, and conservative management has similar results compared to invasive treatments. Larger studies, focussing on outcomes may enhance guidelines on preventive and therapeutic strategies for managing EMH in BTM.KEY MESSAGESExtramedullary haematopoiesis is a rare complication in beta thalassaemia. Although it is more common in non-transfusion dependent thalassaemia, increasingly reported cases suggest a higher prevalence of EMH in TDT than what is known before.There are no clear guidelines on the management of EMH in TDT, with reported patients showing similar outcomes with conservative invasive treatment modalities.More extensive and preferably prospectively designed studies are required focussing on the management of EMH and its outcomes in patients with TDT to formulate evidence-based guidelines.
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