Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with beta-thalassemia intermedia with massive extramedullary hematopoiesis in the mediastinum is presented. These masses, which simulate tumors, are usually asymptomatic only rarely causing spinal cord compression. The histologic examination showed that they consist of hematopoietic tissue. The importance of correct diagnosis without surgery is stressed.
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PMID:Extramedullary hematopoiesis with multiple tumor-simulating mediastinal masses in a patient with beta-thalassemia intermedia. 59 99

Two cases of spinal cord compression resulting from extramedullary haemopoiesis in patients with thalassaemia of intermediate clinical severity are reported. The association between mild thalassaemia with long survival and the risk of spinal cord compression from extramedullary haemopoietic deposits is emphasised.
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PMID:Spinal cord compression in thalassaemia. 59 59

We report a case of homozygous beta thalassaemia who developed chronic paraparesis due to spinal cord compression by paravertebral extramedullary masses. Our patient was successfully treated with hypertransfusion and hydroxyurea. This drug in addition to its well-known cytostatic effects, may be a good alternative in conditions analogous to our case. This action of hydroxyurea can also be attributed to its favourable effect on foetal haemoglobin production.
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PMID:A case of spinal cord compression by extramedullary haemopoiesis in a thalassaemic patient: a putative role for hydroxyurea? 138 78

A 27-year-old-man with beta-thalassaemia/HbE presented with signs and symptoms of spinal cord compression due to epidural extramedullary haematopoiesis. Magnetic resonance images demonstrated spinal cord constriction and atrophy suggesting that the disease process was long-standing. On transverse T2-weighted images, high signal intensity was observed within the spinal cord suggestive of post-compressive spinal cord myelomalacia or gliosis.
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PMID:Case report: chronic spinal cord compression from extramedullary haematopoiesis in thalassaemia--MRI findings. 142 54

Extramedullary haematopoiesis associated with thalassaemia leading to spinal cord compression is an extremely rare event in the course of the disease. The efficacy of radiation therapy is advocated in the management of such a complication. Two patients with thalassaemia, who had presented with spinal cord compression, were successfully treated by a modest dose of local radiotherapy. In one of the patients, however, radiotherapy was resorted to after an initial decompressive laminectomy and partial removal of the intraspinal haematopoietic mass proved unsuccessful. The other patient was managed solely by radiation therapy.
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PMID:The role of radiation therapy in the management of spinal cord compression due to extramedullary haematopoiesis in thalassaemia. 158 17

Two cases of spinal cord compression due to extramedullary haemopoiesis in haemoglobine E thalassaemia are described. Possible mechanisms and treatment modes are discussed.
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PMID:Spinal cord compression in HbE-thalassaemia. 181 27

Spinal cord compression as a consequence of mass lesions due to extramedullary hematopoiesis is a well described but rare syndrome occurring in thalassemia and some other hematologic conditions. The authors report two cases of patients with a genetic variant of beta-thalassemia, who suffered from a progressive compression of the spinal cord in one case, of the cauda equina in the other caused by epidural hematopoietic tissue. The first patient recovered after partial surgical removal of this tissue and subsequent radiotherapy. The second one recovered after only radiotherapy.
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PMID:[Spinal cord and cauda equina compression in 2 patients with beta-thalassemia intermedia]. 201 95

Five cases of tumour-like extramedullary haemopoiesis (EH) causing spinal cord compression or back pain are reported. Three patients were suffering from thalassaemia major, one from sickle cell anaemia and one from thalassaemia intermedia. CT findings included soft tissue masses in the epidural space of the spinal canal, spinal cord displacement and involvement of the underlying bone. MRI studies showed masses compressing the spinal cord. On T1-weighted images there was a signal of slightly higher intensity compared to that of the adjacent marrow. CT suggested the diagnosis in four cases (in the fifth it was not performed), while MRI was positive in all five. There was good correlation between the MRI findings and the CT appearance of the EH masses. Our results suggest that MRI may eliminate the need for other diagnostic examinations in the investigation of patients with EH.
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PMID:CT and MRI of symptomatic spinal involvement by extramedullary haemopoiesis. 214 24

The records of 30 patients presenting radiological evidence of masses of extramedullary myeloadipose (hemopoietic) tissue, 25 patients suffering from thalassemia, one from sickle-cell anemia, and one from myelofibrosis were studied. Three patients presented with primary myelolipomas. Most of the patients with thalassemia presented with masses of hemopoiesis in the costovertebral angle and five patients presented with spinal cord compression. Magnetic resonance imaging is the best method for visualization and assessment of the extent of the masses in the thorax and spinal canal. Radiotherapy is the treatment of choice for spinal cord compression. One patient with thalassemia and one patient with myelofibrosis presented with masses of extramedullary hemopoiesis in the adrenals. All five patients with masses of myeloadipose tissue in the adrenals were treated surgically.
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PMID:Masses of myeloadipose tissue: radiological and clinical considerations. 221 Dec 67

Extramedullary hematopoiesis (EMH) is observed in people suffering from severe anemia of prolonged duration and appears to be a compensatory mechanism for disturbed medullary hematopoiesis. The hemoglobinopathies (such as thalassemia, spherocytosis, and sickle cell disease), neoplastic diseases such as leukemia and lymphoma, and others, including myelofibrosis and osteitis fibrosa cystica, are associated with EMH. These diseases and their resultant anemia have in common the ability to stimulate erythropoietin production, which in turn may stimulate hematopoiesis in organs of mesenchymal origin. The liver and spleen are the most common sites of EMH; however, other sites, including the falx cerebri, thoracic cavity, retroperitoneal area and pelvis have been reported. When present, intrathoracic EMH is most frequently associated with thalassemia. Spinal cord compression and hemothorax have also been reported as complications of intrathoracic EMH.
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PMID:Asymptomatic intrathoracic extramedullary hematopoiesis: a report of three cases. 262 Nov 1


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