Recurrence of spinal cord compression from extramedullary hematopoiesis in thalassemia intermedia treated with low doses of radiotherapy

Dore, Fausto; Pardini, Simonetta; Gaviano, E.; Longinotti, M; Bonfigli, Silvana; Rovasio, S; Tomiselli, A; Cossu, F

doi:10.1002/ajh.2830440216

Cited by 24 publications

(13 citation statements)

References 7 publications

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“…In most cases, these masses are incidentally detected on imaging studies of the chest or abdomen [8,9]. In other cases, the masses may produce symptoms compressing neighboring structures [10,11,12,13,14,15,16,17,18,19,20,21,22]. Magnetic resonance imaging (MRI) is the method of choice for diagnosing EMH [7].…”

Section: Introductionmentioning

confidence: 99%

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

et al. 2017

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Extramedullary hematopoiesis (EMH) results from the extension of hematopoietic tissue beyond the confines of the bones. Since the initiation of regular transfusion programs from an early age for all thalassemia major (ΤΜ) patients, EMH has not been considered a clinical issue anymore. The present study aims to record the prevalence of EMH in chronically transfused ΤΜ patients followed at our institution and to investigate possible risk factors associated with its occurrence. The project was designed as a retrospective, nonexperimental, descriptive, exploratory study. In total, the study enrolled 104 patients. EMH was revealed in 15/104 (14%) patients. The presence of intravening sequence (IVS)-I-6 was significantly related with the development of EMH (p < 0.05). No other demographic or biological factor studied was found to be related with the presence of EMH. The study stresses a profound incidence of asymptomatic EMH in a solid group of well-transfused ΤΜ patients. Given the high incidence of the IVS-I-6 allele in the Mediterranean and Middle Eastern region, high-quality, prospective, multicenter studies could confirm the association of EMH occurrence with the presence of the IVS-I-6 mutation and further evaluate the exact role of this mutation in the EMH process.

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Section: Introductionmentioning

confidence: 99%

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

et al. 2017

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“…However, it is understood that >80% of cases do not have signs and symptoms related directly to the disorder, and the lesions are typically discovered incidentally by radiologic techniques (15–17). The development of neurologic symptoms is suggested to depend on the chronicity of the disease, with neurologic symptoms most frequently being reported during the third and fourth decades of life (18), although few reports described presentation as early as the first decade of life (19–21).…”

Section: Discussionmentioning

confidence: 99%

Extramedullary hematopoiesis: A report of two cases

Zhang

Liu

et al. 2016

Experimental and Therapeutic Medicine

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Extramedullary hematopoiesis (EMH) is defined as hematopoiesis occurring in organs outside of the bone marrow. The present report describes two cases of thalassemic patients with paraspinal medullary hematopoiesis and analyzes the clinical manifestations, imaging, pathology, diagnosis and treatment of EMH. In addition, a supplementary review of previously published cases is provided along with a review of the related literature. Computed tomography (CT) of the first case revealed multiple paraspinal masses, and the largest was 6.2×8.0 cm in diameter. Likewise, CT of the second patient revealed multiple paraspinal masses in the bottom of the left thoracic cavity, and the largest was measured 10.1×10.5 cm. The two cases underwent surgical biopsy and the findings were compatible with a diagnosis of EMH. In conclusion, EMH is a compatible and rare disease, and should be distinguished from other neoplasms. EMH must considered when masses with characteristic radiologic appearance are detected in patients with thalassemia intermedia.

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“…[16] The incidence of EMH was reported to be 15% in patients with homozygous beta-thalassemia who received regular blood transfusions. [461723] In the literature review of Martina et al .,[17] male to female ratio was 2.5:1 and most of the affected patients were within 15-45 years of age. The site of compression was the thoracic region in all cases in their series, as seen in our patient.…”

Section: Discussionmentioning

confidence: 99%

Long segment spinal epidural extramedullary hematopoiesis

Garg

Singh

et al. 2013

Surg Neurol Int

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Background:Extramedullary hematopoiesis is defined as the formation of blood cells outside the bone marrow. It is a common manifestation of many chronic hemolytic anemias, and typically involves the liver, spleen, and lymph nodes. Only rarely is the spinal epidural space involved.Methods:We describe a 25-year-old male, known to have thalassemia intermedia, who presented with a 1-month history of stiffness and weakness in both lower extremities. On physical examination, he had palpable splenomegaly accompanied by spinal tenderness at the D5 level, weakness in both lower extremities, hyperactive bilateral Patellar and Achilles reflexes with bilateral Babinski responses, and a graded sensory loss to pin appreciation below D5.Results:The magnetic resonance (MR) study revealed a posterior, isointense and soft tissue epidural mass extending from D2 to D12 on both the T1- and T2-weighted images. These findings were consistent with the diagnosis of “red marrow,” and long-segment spinal epidural extramedullary hematopoiesis.Conclusions:Although extramedullary hematopoiesis is rarely encountered within the spinal canal, it should be considered among the differential diagnoses when a posterior compressive thoracic lesion contributes to myelopathy in a patient with a history of thalassemia intermedia and the accompanying chronic hemolytic anemia.

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Recurrence of spinal cord compression from extramedullary hematopoiesis in thalassemia intermedia treated with low doses of radiotherapy

Cited by 24 publications

References 7 publications

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

Extramedullary hematopoiesis: A report of two cases

Long segment spinal epidural extramedullary hematopoiesis

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