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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

As described in the literature, spinal cord compression by extramedullary hematopoietic tissue rarely occurs in thalassemic patients. Laminectomy and/or radiotherapy are the main approaches. We report on a patient with thalassemia intermedia who developed paralysis of both lower extremities due to the compression of the spinal cord by extramedullary hematopoietic tissue.
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PMID:Spinal cord compression by extramedullary hematopoietic tissue in a thalassemic patient: prompt effect of radiotherapy. 263 14

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. After low-dose radiotherapy alone, a rapid and durable response occurred in a patient with thalassemia. No side effects were encountered and the patient has been stable neurologically for over 7 years since treatment. The results of the current case, and those previously reported in which radiotherapy alone was given, suggest that low-dose radiotherapy should be considered as a primary treatment modality for the management of this syndrome, at least in patients with thalassemia.
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PMID:Spinal cord compression due to extramedullary hematopoiesis in thalassemia: long-term follow-up after radiotherapy. 336 75

The subject of marrow heterotopia has been reviewed on the basis of 15 cases suffering from thalassemia. Other cases reported in the literature were also reviewed. Using conventional radiography, scintigraphy, computerized tomography and myelography, 17% of the cases admitted into the hospital with the diagnosis of Thalassemia, were found to have macroscopic masses of marrow heterotopia. The most common site of development of these masses was the costovertebral gutter, followed by the anterior end of the ribs and the extradural space of the spinal canal. In one case, masses were located in the maxillary antra. The clinical implications, the pathogenesis of the masses and the differential diagnosis from other tumour-like entities are discussed. Three patients presented with symptoms and signs of spinal cord compression. All three patients were treated satisfactorily with small doses of radiotherapy.
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PMID:The marrow heterotopia in thalassemia. 372 Jul 53

A case is reported of spinal cord compression resulting from extramedullary haemopoiesis in a patient with thalassaemia. A 28-year-old woman with beta thalassaemia intermedia presented with a two week history of paraparesis with bladder and bowel incontinence. Recovery followed laminectomy and the removal of haemopoietic tissue in the spinal epidural space. This is a rare complication and only eight cases have been reported in the literature.
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PMID:Paraplegia with thalassaemia. 657 82

Two patients with beta-thalassemia and spinal cord compression due to extradural extramedullary hematopoietic tissue are presented and the related literature is reviewed. This rare complication, once diagnosed, may be treated readily with surgical decompression or radiation. The diagnosis is suggested by evidence of extramedullary hematopoiesis elsewhere and the presence of an extradural lesion, usually in the mid- and lower thoracic region, on myelography and, at times, a paravertebral mass on chest x-ray films. Our findings lend support to the hypothesis that such hematopoietic tissue in the spinal cord vicinity arises from embryonal rests in the extradural areolar tissue of mesodermal origin.
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PMID:Curable paraplegia due to extradural hematopoietic tissue in thalassemia. 716 76

Twelve patients with beta-thalassemia/hemoglobin E disease had spinal cord compression. Ten were made and two female, aged 17 to 40 years. The causes of spinal cord compression presumably were extramedullary hematopoietic masses. This was proved by surgery in two cases. In six cases, myelography demonstrated extradural blockade. In the others, the recurrent nature of the paraparesis and the prompt response to deep x-ray therapy were compatible with cord compression by extramedullary hematopoietic masses. Although spontaneous recovery and disappearance of the neurological signs after blood transfusions were observed, these were slow and uncertain. Deep x-ray therapy led to prompt response with more lasting benefit in all cases and is thus recommended as standard treatment for this complication.
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PMID:Spinal cord compression in thalassemia. Report of 12 cases and recommendations for treatment. 724 89

Spinal cord compression due to extramedullary haemopoietic tissue in a case of beta thalassaemia and G6 PD deficiency is described. Surgical decompression alone had no beneficial effect. However, the addition of radiotherapy proved very useful.
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PMID:Spinal cord compression in homozygous beta thalassaemia. 738

A 22-year-old male with a history of hemoglobin E beta zero-thalassemia presented with thoracic spinal cord compression due to extramedullary hematopoietic tissue in the epidural space. Following decompressive laminectomy, this epidural mass was irradiated to 20.4 Gy in 12 fractions. The patient made a complete recovery from his neurological symptoms following treatment. Follow-up radiographs showed significant reductions in size of the extradural tumor. Results from this case supported by review of the literature suggest that radiation therapy, with or without decompressive surgery, is an effective therapy in treatment of patients with spinal cord compression due to epidural extramedullary hematopoietic tissue.
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PMID:Combined radiotherapeutic and surgical management of a spinal cord compression by extramedullary hematopoiesis in a patient with hemoglobin E beta-thalassemia. 809 37

Symptomatic spinal cord compression caused by an epidural mass of extramedullary hematopoietic tissue in patients with beta-thalassemia is a rare occurrence, that becomes exceptional in childhood and adolescence. The literature is not uniform about the optimal treatment of these patients and different modes of therapy have been proposed so far, including surgical excision or decompressing laminectomy followed by local irradiation and/or hypertransfusional regimens. We report the successful treatment of such a complication in an adolescent with homozygous beta-thalassemia by surgery followed by repeated blood transfusions aimed at maintaining hematocrit at normal levels.
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PMID:Spinal cord compression in homozygous beta-thalassemia intermedia. 820 93

A 19-year-old girl with thalassemia intermedia presented with signs of thoracic spinal cord compression secondary to extramedullary hematopoiesis. She was started on a transfusion regimen to maintain a hemoglobin level of more than 12.5 g/dL. Clinical signs disappeared within the first week and circulating erythroblastemia was completely suppressed by the second week. Magnetic resonance imaging 4 weeks after diagnosis revealed near-complete resolution of the extradural mass, followed by gradual improvement in the posterior tibial somatosensory evoked potentials. Transfusion therapy may be diagnostically and therapeutically useful in spinal cord compression secondary to extramedullary hematopoiesis, obviating the need for surgery or radiotherapy.
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PMID:Hypertransfusion for spinal cord compression secondary to extramedullary hematopoiesis. 871 6


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