Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review
<h3>Introduction</h3><p dir="ltr">Around 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...
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2022
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| author | Eihab A. Subahi (12217761) |
| author2 | Fateen Ata (12217764) Hassan Choudry (12217767) Phool Iqbal (12217770) Mousa A. AlHiyari (12217773) Ashraf T. Soliman (12217776) Vincenzo De Sanctis (12217779) Mohamed A. Yassin (8361183) |
| author2_role | author author author author author author author |
| author_facet | Eihab A. Subahi (12217761) Fateen Ata (12217764) Hassan Choudry (12217767) Phool Iqbal (12217770) Mousa A. AlHiyari (12217773) Ashraf T. Soliman (12217776) Vincenzo De Sanctis (12217779) Mohamed A. Yassin (8361183) |
| author_role | author |
| dc.creator.none.fl_str_mv | Eihab A. Subahi (12217761) Fateen Ata (12217764) Hassan Choudry (12217767) Phool Iqbal (12217770) Mousa A. AlHiyari (12217773) Ashraf T. Soliman (12217776) Vincenzo De Sanctis (12217779) Mohamed A. Yassin (8361183) |
| dc.date.none.fl_str_mv | 2022-03-09T03:00:00Z |
| dc.identifier.none.fl_str_mv | 10.1080/07853890.2022.2048065 |
| dc.relation.none.fl_str_mv | https://figshare.com/articles/journal_contribution/Extramedullary_haematopoiesis_in_patients_with_transfusion_dependent_-thalassaemia_TDT_a_systematic_review/29117018 |
| dc.rights.none.fl_str_mv | CC BY 4.0 info:eu-repo/semantics/openAccess |
| dc.subject.none.fl_str_mv | Biomedical and clinical sciences Cardiovascular medicine and haematology Clinical sciences Thalassaemia transfusion-dependent thalassaemia extramedullary haematopoiesis |
| dc.title.none.fl_str_mv | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review |
| dc.type.none.fl_str_mv | Text Journal contribution info:eu-repo/semantics/publishedVersion text contribution to journal |
| description | <h3>Introduction</h3><p dir="ltr">Around 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. </p><h3>Methods</h3><p dir="ltr">This 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. </p><h3>Results</h3><p dir="ltr">Data 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 (N = 23) (paraspinal EMH). Magnetic resonance imaging (MRI) was the most widely used diagnostic modality (226). Overall, blood transfusion was the commonest reported treatment (30), followed by radiotherapy (20), surgery (15), hydroxyurea (12), steroids (6), and exchange transfusion (2). An outcome was reported in 20% of patients, all recovered, except one who died as a result of nosocomial infection. </p><h3>Conclusion</h3><p dir="ltr">EMH 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.</p><h3>KEY MESSAGES</h3><p dir="ltr">Extramedullary 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. </p><p dir="ltr">There are no clear guidelines on the management of EMH in TDT, with reported patients showing similar outcomes with conservative invasive treatment modalities. </p><p dir="ltr">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.</p><h2>Other Information</h2><p dir="ltr">Published in: Annals of Medicine<br>License: <a href="http://creativecommons.org/licenses/by/4.0/" target="_blank">http://creativecommons.org/licenses/by/4.0/</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1080/07853890.2022.2048065" target="_blank">https://dx.doi.org/10.1080/07853890.2022.2048065</a></p> |
| eu_rights_str_mv | openAccess |
| id | Manara2_98bdbfd5a1400a13bd26b48d41e9853a |
| identifier_str_mv | 10.1080/07853890.2022.2048065 |
| network_acronym_str | Manara2 |
| network_name_str | Manara2 |
| oai_identifier_str | oai:figshare.com:article/29117018 |
| publishDate | 2022 |
| repository.mail.fl_str_mv | |
| repository.name.fl_str_mv | |
| repository_id_str | |
| rights_invalid_str_mv | CC BY 4.0 |
| spelling | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic reviewEihab A. Subahi (12217761)Fateen Ata (12217764)Hassan Choudry (12217767)Phool Iqbal (12217770)Mousa A. AlHiyari (12217773)Ashraf T. Soliman (12217776)Vincenzo De Sanctis (12217779)Mohamed A. Yassin (8361183)Biomedical and clinical sciencesCardiovascular medicine and haematologyClinical sciencesThalassaemiatransfusion-dependent thalassaemiaextramedullary haematopoiesis<h3>Introduction</h3><p dir="ltr">Around 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. </p><h3>Methods</h3><p dir="ltr">This 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. </p><h3>Results</h3><p dir="ltr">Data 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 (N = 23) (paraspinal EMH). Magnetic resonance imaging (MRI) was the most widely used diagnostic modality (226). Overall, blood transfusion was the commonest reported treatment (30), followed by radiotherapy (20), surgery (15), hydroxyurea (12), steroids (6), and exchange transfusion (2). An outcome was reported in 20% of patients, all recovered, except one who died as a result of nosocomial infection. </p><h3>Conclusion</h3><p dir="ltr">EMH 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.</p><h3>KEY MESSAGES</h3><p dir="ltr">Extramedullary 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. </p><p dir="ltr">There are no clear guidelines on the management of EMH in TDT, with reported patients showing similar outcomes with conservative invasive treatment modalities. </p><p dir="ltr">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.</p><h2>Other Information</h2><p dir="ltr">Published in: Annals of Medicine<br>License: <a href="http://creativecommons.org/licenses/by/4.0/" target="_blank">http://creativecommons.org/licenses/by/4.0/</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1080/07853890.2022.2048065" target="_blank">https://dx.doi.org/10.1080/07853890.2022.2048065</a></p>2022-03-09T03:00:00ZTextJournal contributioninfo:eu-repo/semantics/publishedVersiontextcontribution to journal10.1080/07853890.2022.2048065https://figshare.com/articles/journal_contribution/Extramedullary_haematopoiesis_in_patients_with_transfusion_dependent_-thalassaemia_TDT_a_systematic_review/29117018CC BY 4.0info:eu-repo/semantics/openAccessoai:figshare.com:article/291170182022-03-09T03:00:00Z |
| spellingShingle | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review Eihab A. Subahi (12217761) Biomedical and clinical sciences Cardiovascular medicine and haematology Clinical sciences Thalassaemia transfusion-dependent thalassaemia extramedullary haematopoiesis |
| status_str | publishedVersion |
| title | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review |
| title_full | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review |
| title_fullStr | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review |
| title_full_unstemmed | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review |
| title_short | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review |
| title_sort | Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review |
| topic | Biomedical and clinical sciences Cardiovascular medicine and haematology Clinical sciences Thalassaemia transfusion-dependent thalassaemia extramedullary haematopoiesis |