Massive extramedullary bone ‐ marrow formation in a case of pernicious anæmia

Lyall, Alexander

doi:10.1002/path.1700410310

Cited by 48 publications

(8 citation statements)

References 2 publications

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“…Although not clear, there are two main hypotheses trying to explain such a predilection. Knoblich [36] proposed that local embryonic rests in the epidural space can be transformed into marrow under conducive circumstances, whereas Lyall [37] advocated the direct extension of the process from the marrow into the epidural space [35,38]. …”

Section: Discussionmentioning

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: Discussionmentioning

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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“…It occurs due to expansion of the haemopoietic tissue in order to supplement marrow production. Knoblich [3] proposed that multipotential cells in the epidural space can be transformed into marrow under conducive circumstances, and Lyall [4] advocated the possibility of direct extension from marrow into the epidural space. Occasionally it appears as a tumor-like mass causing compression of spinal cord [5,10,11,12,13].…”

Section: Discussionmentioning

confidence: 99%

MRI findings of extramedullary haemopoiesis

et al. 2001

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Extramedullary haemopoiesis (EH) is a compensatory process associated with chronic haemolytic anaemia. It is rare, however, for such an abnormality to cause spinal cord compression. We present two patients with known beta-thalassaemia intermedia who developed spinal cord compression due to masses of extramedullary haematopoietic tissue in the epidural space of the thoracic spine. The EH masses were diagnosed by MRI as an isointense epidural lesion on both T1- and T2-weighted images, compressing severely the spinal cord. After administration of a paramagnetic agent, an intermediate enhancement of the masses was evident. All the vertebral bodies had low to intermediate signal intensity as a result of displacement of fatty marrow by haematopoietic marrow. Expansion of thoracic ribs with bilateral paravertebral masses were characteristic. A small dose of radiotherapy was given and marked improvement in neurological symptoms was evident. An MRI examination established shrinkage of the mass and decompression of spinal cord. The role of MRI in diagnosis of EH masses is essential and radiation therapy is a very effective treatment for this rare complication.

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“…Various hypotheses exist to explain the development of hemopoietic tissue to supplement marrow production. Lyall (8) proposed the possibility of direct extension from marrow into epidural space. Knoblich (4) proposed that multipotential cells in the epidural space can be transformed into marrow under some circumstances.…”

Section: Discussionmentioning

confidence: 99%

37‐year‐old Woman With Multiple Intracranial Masses

2011

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Extramedullary hematopoiesis (EMH), defined as the presence of hematopoiesis outside bone marrow and peripheral blood, occurs asa compensatory phenomenon in several hematologic disorders and bone marrow dysfunction. EMH predominantly affects reticuloendothelial system including the spleen, liver and lymph nodes. Here,we report a rare case of multiple intracranial meningeal EMH. A37-year-old woman was anemic with gradually worsening vision for 8 months. Multiple extra-axial masses were found on imaging and the patient underwent the biopsy for the left frontotemporal lesion.Final diagnosis was multiple intracranial meningeal EMH. Treatment of fractionated external beam radiotherapy resulted in marked symptomatic improvement. This case indicates that although the diagnosis of meningeal EMH is difficult, there is a need to consider EMHin the differential diagnosis of anemic patients with tumor-like mass lesions in extramedullary sites.

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Massive extramedullary bone ‐ marrow formation in a case of pernicious anæmia

Cited by 48 publications

References 2 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

MRI findings of extramedullary haemopoiesis

37‐year‐old Woman With Multiple Intracranial Masses

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