scholarly journals Compressive Dorsal Myelopathy Secondary to Extramedullary Hematopoiesis in a Thalassemic Patient

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Ismail Ibrahim Ismail ◽  
Fathi Massoud ◽  
K. J. Alexander ◽  
Jasem Youssef Al-Hashel
Keyword(s):  
Extramedullary Hematopoiesis ◽  
Cord Compression ◽  
Lower Limbs ◽  
Beta Thalassemia ◽  
Sensory Level ◽  
Compensatory Mechanism ◽  
Hematopoietic Tissue ◽  
Thalassemic Patient ◽  
Low Dose Radiotherapy ◽  
Magnetic Resonance Imaging Mri

Background. Extramedullary hematopoiesis (EMH) is one of the rare causes of spinal cord compression (SCC). It results from noncancerous proliferation of hematopoietic tissue outside the bone marrow as a compensatory mechanism for ineffective erythropoiesis. It occurs in the paraspinal area in 11–15% of thalassemic patients in intermediate and severe cases causing a paraspinal compressive mass. We present a rare case of spinal EMH with thoracic cord compression in a 22-year-old female with beta thalassemia who presented with paraparesis and we provide a review of literature. Case Report. A 22-year-old female patient with a known history of beta thalassemia presented with subacute onset of weakness and numbness of both lower limbs with a sensory level at T6. Magnetic resonance imaging (MRI) of the dorsal spine showed cord compression secondary to paraspinal EMH from T2 to T9 with most prominent compression over T5. She was managed with blood transfusion and low-dose radiotherapy. Conclusion. Although rare, EMH should be suspected in thalassemic patients presenting with paraplegia. Treatment with blood transfusions is usually effective. Other options include radiotherapy, surgery, hydroxyurea or a combination of these modalities.

Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia

2020 ◽  
Vol 78 (10) ◽  
pp. 663-664
Author(s):  
Renan Ramon Souza LOPES ◽  
Larissa Soares CARDOSO ◽  
Franz ONISHI
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Compression ◽  
Extramedullary Hematopoiesis ◽  
Cord Compression ◽  
Beta Thalassemia

scholarly journals Spinal cord compression in b-thalassemia: follow-up after radiotherapy

1998 ◽  
Vol 116 (6) ◽  
pp. 1879-1881 ◽  
Author(s):  
Silvana Fahel da Fonseca ◽  
Maria Stella Figueiredo ◽  
Rodolfo Delfini Cançado ◽  
Fernando Nakandakare ◽  
Roberto Segreto ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Case Report ◽  
Spinal Cord Compression ◽  
Low Dose ◽  
Extramedullary Hematopoiesis ◽  
Cord Compression ◽  
Hematologic Disorders ◽  
Rare Syndrome ◽  
Low Dose Radiotherapy

CONTEXT: Spinal cord compression due to extramedullary hematopoiesis is a well-described but rare syndrome encountered in several clinical hematologic disorders, including <FONT FACE="Symbol">b</font>-thalassemia. CASE REPORT: We report the case of a patient with intermediate <FONT FACE="Symbol">b</font>-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. DISCUSSION: Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms.

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

2017 ◽  
Vol 137 (3) ◽  
pp. 175-182 ◽  
Author(s):  
Nikolaos Sousos ◽  
Despoina Adamidou ◽  
Philippos Klonizakis ◽  
Alexandra Agapidou ◽  
Stamatia Theodoridou ◽  
...  
Keyword(s):  
Middle Eastern ◽  
Thalassemia Major ◽  
Extramedullary Hematopoiesis ◽  
Biological Factor ◽  
Beta Thalassemia ◽  
Eastern Region ◽  
Hematopoietic Tissue ◽  
Multicenter Studies ◽  
Clinical Issue

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.

scholarly journals Spinal Cord Compression Secondary to Extramedullary Hematopoiese in a Patient with a Beta-Thalassemia

2019 ◽  
Vol 4 (5) ◽  
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Compression ◽  
Mass Effect ◽  
Myeloproliferative Disorders ◽  
Extramedullary Hematopoiesis ◽  
Cord Compression ◽  
Neurological Deficits ◽  
Beta Thalassemia ◽  
Spleen And Lymph Nodes ◽  
Upper Thoracic

Extramedullary hematopoiesis (EMH) is a rare cause of spinal cord compression (SCC). EMH represents the growth of blood cells outside of the bone marrow and occurs in a variety of hematologic illnesses, including various types of anemia and myeloproliferative disorders. Although EMH usually occurs in the liver, spleen, and lymph nodes, it may also occur within the spinal canal. When this occurs, the mass effect can compress the spinal cord, potentially leading to the development of neurological deficits. We present a case of SCC secondary to EMH. Our patient is a 26-year-old male with beta-thalassemia who presented with both upper thoracic and lower extremity symptoms of spinal cord compression. This report illustrates the importance of considering EMH in the differential diagnosis of SCC, even in the absence of signs of its most common etiologies.

Spinal cord compression due to extramedullary hematopoiesis in homozygous thalassemia

1975 ◽  
Vol 42 (2) ◽  
pp. 212-216 ◽  
Author(s):  
Wlllem Luyendijk ◽  
Lodewijk Went ◽  
Hans D.G. Schaad
Keyword(s):  
Spastic Paraparesis ◽  
Extramedullary Hematopoiesis ◽  
Cord Compression ◽  
Surgical Removal ◽  
Pathological Findings ◽  
Hematopoietic Tissue ◽  
Content Type ◽  
Vertebral Canal ◽  
Direct Extension ◽  
Fine Print

✓ The authors report a case of homozygous thalassemia in which a mass of hematopoietic tissue in the vertebral canal caused spastic paraparesis. Surgical removal of the tissue plus radiotherapy were successful. The pathological findings indicated direct extension of hematopoietic tissue from the adjacent bone marrow into the epidural space of the vertebral canal.

scholarly journals Extramedullary Haematopoiesis Causing Spinal Cord Compression in a Thalassemia Intermedia Patient

2013 ◽  
Vol 2 (2) ◽  
pp. 81-85
Author(s):  
S Wahab ◽  
RA Khan ◽  
K Ahmad ◽  
A Wahab ◽  
I Ahmad
Keyword(s):  
Spinal Canal ◽  
Extramedullary Hematopoiesis ◽  
Cord Compression ◽  
Clinical Severity ◽  
Globin Chain ◽  
Thalassemia Intermedia ◽  
Hematopoietic Tissue ◽  
Extramedullary Haematopoiesis ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

The thalassemias are a heterogenous group of inherited disorders of hemoglobin (Hb) synthesis characterized by a lack of or decreased /defective synthesis of globin chains. The name Mediterranean anemia, which Whipple introduced, is misleading because the condition can be found in any part of the world. In ?-thalassemia, ?-globin chain synthesis is reduced, whereas in ?-thalassemia, ?-globin chain synthesis is defective. Their clinical severity varies widely, ranging from asymptomatic forms to severe or even fatal entities. Like most anemias, erythropoietin production and erythropoiesis is increased in thalassemia causing erythroid marrow hyperplasia as well as extramedullary haematopoiesis which is a common compensatory mechanism for chronic anemia found in patients with hemoglobinopathies such as thalassemia, sickle cell anemia, and hereditary spherocytosis. These patients are usually asymptomatic. Extramedullary hematopoiesis (EMH) usually manifests in the liver, spleen, kidneys and as paravertebral masses. Extramedullary hematopoietic tissue occurring within the spinal canal and causing cord compression is an unusual but well-described entity. Total surgical excision is usually not feasible because of the diffuse spread of extramedullary hematopoietic tissue and the possibility of recurrence, but acute neurological impairment does require emergency surgery. Extramedullary hematopoiesis is radiosensitive so radiation therapy is widely accepted as a very effective mode of therapy. Repeated blood transfusion and cytostatic agents are also recommended for treatment.We report a case of a 35-year-old man with underlying beta thalassemia intermedia who developed pain in lower back and progressive paresthesia and weakness of both lower limbs due to multifocal extramedullary epidural hematopoietic tissue occurring within the spinal canal with compression over the cord. Nepalese Journal of Radiology; Vol. 2; Issue 2; July-Dec. 2012; 81-85 DOI: http://dx.doi.org/10.3126/njr.v2i2.7692

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