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 24-year-old female with haemoglobin E-thalassaemia who presented with paraparesis of 18 months' duration was found to have spinal extradural extramedullary haemopoietic tissue extending from T4 to T9. Complete neurological recovery followed laminectomy. The importance of prompt diagnosis and treatment, of this unusual complication of extramedullary haemopoiesis, before the spinal cord is irreversibly damaged is stressed.
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PMID:A case of paraparesis due to extramedullary haemopoiesis in HbE-thalassaemia. 88 81

Pathomorphology of symmetrical areas of various departments of the central nervous system was studied in 8 children aged 3--8 years who had died of beta-thalassaemia major. In 5 patients transient cerebral vascular disorders occurred against the background of hemolytic crisis, in 2 -- phenomena of polyradiculoneuritis, in 1 -- spinal desorders with lower flaccid paraparesis. Vessles were found to be damaged by the pathological process first. Impairment of permeability of vascular membranes, hemorrhages and edema of the brain substance were observed. As to neurons lesions, along with normal neurons there were nerve cells with reactive and dystrophic changes. In pulpous nerve fibres periaxonal alterations and Wallerian degeneration predominated. The combination of the erythroblastic process with secondary impairment of the metabolism, toxicosis, hypoxaemia contributed to the mixed character of the morphological changes, which accounted for the polymorphism of neurological symptoms.
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PMID:[Pathomorphology of the nervous system in beta-thalassemia major]. 101 94

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.
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PMID:Spinal cord compression due to extramedullary hematopoiesis in homozygous thalassemia. Case report. 111 58

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 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

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

Two patients with massive, tumor-simulating extramedullary hematopoiesis are presented. In 1 patient with homozygous beta-thalassemia, a progressive paraparesis developed because of a mass of ectopic marrow in the spinal epidural space. Surgical removal and radiotherapy were successful. The second patient, who died during surgical biopsy, had posterior mediastinal masses without any apparent cause. A correct preoperative diagnosis may avoid unnecessary surgical intervention. It is suggested that heterotopic marrow should be included in the differential diagnosis of a mass in the lower thoracic region.
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PMID:Tumor-simulating thoracic extramedullary hematopoiesis. 746 78

Extramedullary hematopoiesis associated with thalassemia causing spinal cord compression is an extremely rare event in the course of the disease. Documentation with an imaging technique, such as MRI, is mandatory. A patient with thalassemia intermedia, who developed paraparesis in spite of transfusion, underwent surgical decompression. Rapid neurological improvement was observed postoperatively and this neurological condition was protected with adequate hemoglobin level. Management of these patients remains controversial. Various modes of therapy such as surgical decompression, radiotherapy, and transfusion are discussed and the related literature is reviewed.
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PMID:Spinal cord compression secondary to extramedullary hematopoiesis in thalassemia intermedia. 988 60

Thalassemia is a common hematological disease in Southeast Asia. Extramedullary hematopoiesis is common sequelae in thalassemic patients but extramedullary hematopoiesis in the spinal epidural space that leads to paraparesis in pregnancy is very rare. We managed a thalassemic patient with extramedullary hematopoiesis and spinal cord compression during pregnancy. The diagnosis was made on clinical features and magnetic resonance imaging (MRI) showing a paravertebral mass infiltrating the epidural space. She was treated successfully with repeated blood transfusions until delivery. Fetal growth restriction was found but otherwise the fetus was clinically normal. She had an uneventful recovery when she was seen 6 weeks after delivery.
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PMID:Spinal cord compression: a rareness in pregnant thalassemic woman. 1087 Mar 3

Patients with beta-globin disorders show amelioration of clinical condition by sustained synthesis of fetal haemoglobin in adult life. We report data on a patient with beta(o)-thalassaemia genotype and thalassaemia intermedia clinical phenotype. He received therapy with hydroxyurea (20 mg/kg/d) because of the presence of extramedullary masses causing paraparesis, neurogenic bladder and impotence. During therapy, the patient showed an improved clinical picture and a significant increase in total Hb (from 71.8 to 103.2 g/L) and a gamma/alpha globin synthetic ratio (from 0.39 to 0.68). The myelosuppressive effect of hydroxyurea was revealed by a decrease in CFU-GEMM, BFU-E, and CFU-GM. Therefore hydroxyurea can be effective in the treatment of patients with extramedullary haematopoiesis (EMH) who are not transfusion-dependent and cannot be treated with radiotherapy.
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PMID:Hydroxyurea therapy in paraparesis and cauda equina syndrome due to extramedullary haematopoiesis in thalassaemia: improvement of clinical and haematological parameters. 1090 97


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