Low back pain in beta thalassemia major revealing sacral extramedullay hematopoeisis: A case report

Raza, Ahmad; Okar, Lina; Elhiday, Abdelhaleem; Almasri, Hussam; Ata, Fateen; Mounir, Elsayed Ahmed; Barah, Ali; Abdelrazek, Mohamed; Gamil, Amna; Eldean, Mouhammad Z. Sharaf; Yassin, Mohamed A

doi:10.1002/ccr3.4258

Cited by 3 publications

(7 citation statements)

References 11 publications

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“…EMH was diagnosed as extradural masses on MRI ( Figure 4 ). However, a confirmation was made via biopsy, which revealed erythroid, megakaryocytes, and myeloid precursors ( Figure 5 ) [ 19 ]. The patient eventually underwent surgery with a resolution of her presenting features.…”

Section: Discussionmentioning

confidence: 99%

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Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review

et al. 2022

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Introduction 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. Methods 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. Results 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. Conclusion 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. KEY MESSAGES 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. 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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Section: Discussionmentioning

confidence: 99%

“…Note . Image taken from Clinical Case reports from a previously published article [ 42 ]. The licence of the article (CC-BY) permits unrestricted reuse of the published work.…”

Section: Discussionmentioning

confidence: 99%

Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review

et al. 2022

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“…Both patients were managed by radiotherapy with excellent outcomes. 6,10 Our experience prompted us to explore the available literature on the management and outcomes of EMH in patients with TDT. The prevalence of EMH is quite variable, ranging from 1% to 13%.…”

Section: Discussionmentioning

confidence: 99%

“…There is at least one retrospective study and multiple case reports on EMH in BTM with variable management strategies and outcomes. [6][7][8]10 We have conducted a thorough literature search and identified a total of 254 patients with TDT and EMH. This systematic review will be among the first ones to provide an aggregate evidence on demographics, management, and outcomes of EMH in TDT.…”

Section: Introductionmentioning

confidence: 99%

Protocol for extramedullary hematopoiesis in patients with transfusion‐dependent β‐thalassemia (TDT): A systematic review

Ata

Subahi

Choudry

et al. 2021

Health Science Reports

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Background and aims Thalassemia is one of the most common hemoglobinopathies, with around 5% of the world's population expected to have some degree and type of thalassemia. Beta thalassemia (BT) occurs due to a deficient production of the beta‐globin chain of hemoglobin. Extramedullary hematopoiesis (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‐thalassemia major (BTM). However, there are increasingly reported cases of EMH in BTM. The incidence of EMH in BTM is thought to be <1%. However, it seems that the true incidence is much higher than expected. This review aims to pool the available data and provide cumulative evidence on the reports of EMH in BTM patients. Methods We aim to conduct a systematic review via searching multiple electronic databases (PubMed, Scopus, Google Scholar) to identify eligible articles from any date up to December 2020. Eligible studies should report extramedullary hematopoiesis in BTM. Case reports, case series, observational studies with cross‐sectional or prospective research design, case‐control studies, and experimental studies will be included if found relevant. Two reviewers (FA and ES) will individually analyze the study quality using the statistical methodology and categories guided by the Cochrane Collaboration Handbook, PRISMA guidelines, and Joanna Briggs Institute checklist for case reports and series. Results This study will analyze and incorporate the available evidence on EMH in BTM concerning patient demographics, sites of EMH, management, and clinical outcomes of EMH. Conclusion By summarizing and statistically analyzing the data about EMH in BTM, this study will generate extensive knowledge on the topic for a better understanding of atypical presentations in BTM, a common hemoglobinopathy.

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“…[ 6 ] Less frequently, patients may also develop complications secondary to massive erythroid expansion (known as extramedullary hematopoiesis “EMH”). [ 7 ]…”

Section: Introductionmentioning

confidence: 99%

Recurrent spinal cord compression due to extramedullary hematopoiesis in thalassemia patient

et al. 2022

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Introduction:Spinal cord compression secondary to extramedullary hematopoiesis (EMH) is a rare condition. Variable treatment options have been reported with different efficacy and recurrence rate. Due to its rarity, no clear optimal management guidelines have been established yet.Patient concerns and diagnosis:We report a recurrence of spinal cord compression secondary to EMH in a 19-year-old male, with a background of transfusion-dependent beta-thalassemia on luspatercept, who presented with weakness in both lower limbs.Interventions and outcomes:He was treated successfully both times with radiotherapy.Conclusion:Early diagnosis and management of EMH compressing the spinal cord are essential to prevent permanent neurological damage. Diagnosis should be suspected based on the clinical presentation and magnetic resonance imaging findings in a patient with a history of ineffective hemopoiesis. Treatment option remains controversial. Radiotherapy option seems effective, even in recurrent cases, and valid, particularly for those at high risk of surgery or who do not prefer it.

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Low back pain in beta thalassemia major revealing sacral extramedullay hematopoeisis: A case report

Cited by 3 publications

References 11 publications

Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review

Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review

Protocol for extramedullary hematopoiesis in patients with transfusion‐dependent β‐thalassemia (TDT): A systematic review

Recurrent spinal cord compression due to extramedullary hematopoiesis in thalassemia patient

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