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

A survey on transfusion reactions in thalassemia was carried out within the COOLEYCARE Programme, a cooperative enterprise aimed at improving quantity and quality of life in thalassemia through a program of quality assurance of treatment delivered to patients. Reactions were reported in 1,225 of 111,590 red cell transfusions (1.1%) given during 40 months (September 1985-December 1987) to 3,755 thalassemics in Italy and Greece. About 90% of red cell units were leukocyte-poor. Filtration was the most commonly used technique for leukocyte removal. Chills, fever, urticaria, headache and chest pain accounted for more than 80% of symptoms reported. Reactions were reported during transfusion in two thirds of cases. Although reactions were reported from 16% of patients, three quarters of reacting patients had no more than 2 reactions in 40 months.
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PMID:Transfusion reactions in thalassemia. A survey from the Cooleycare programme. The Cooleycare Cooperative Group. 208 72

Post-transfusion hypertension, convulsion and cerebral haemorrhage is a serious complication that may occur in the thalassaemias. In this study we evaluated the effect of blood transfusion on blood pressure, plasma renin activity (PRA), blood viscosity, and urinary vanillylmandelic acid (VMA) and catecholamines in 11 beta-thalassaemia/haemoglobin E patients. The results showed that after each unit of blood transfusion the blood viscosity was increased and correlated with the increased in haematocrit level. At the same time the PRA level was significantly decreased and tended to return to the normal level in a few days after the transfusion. There was no alteration in the urinary VMA and catecholamine levels. During the study two patients developed hypertension and headache. Their PRA were still lower than the pre-transfusion levels and the blood pressure returned to the normal pre-transfusion levels within 30-90 minutes after the intravenous injection of furosemide.
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PMID:Study of mechanisms of post-transfusion hypertension in thalassaemic patients. 269 89

From March to August 1984, 26 patients with hereditary hemolytic anemia in northeastern Ohio developed acute, profound red cell aplasia. The patients included 14 males and 12 females 2 to 23 years old, with sickle cell anemia (20 cases), hemoglobin SC-disease (4 cases), sickle-beta-thalassemia (1 case), or hereditary spherocytosis (1 case). All had an acute onset of severe reticulocytopenia and anemia and prodromal symptoms of illness including fever, abdominal symptoms, headache, and arthralgias. Twenty-two received transfusions. Reticulocytosis occurred spontaneously within 2 to 14 days of presentation. In five acute-phase sera, 10(8) to 10(12) viral particles/mL were detected by electron microscopy. Human parvovirus B19 DNA was demonstrated in high concentration by hybridization in the same five acute-phase sera and in low concentration in sera of eight additional patients. The five highly viremic sera inhibited erythroid colony formation in vitro. B19-specific IgM was detected in sera of 24/26 patients, and B19-specific IgG in 21 of 22 patients tested. Our results indicate that human parvovirus B19 was the etiologic agent in this large epidemic of life-threatening acute red cell aplasia in patients with hereditary hemolytic anemia.
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PMID:Human parvovirus B19-induced epidemic acute red cell aplasia in patients with hereditary hemolytic anemia. 300 91

Spontaneous intracranial haemorrhage is one of the cerebrovascular complications in beta-thalassaemia major patients. This is a report of 2 cases of fatal intracranial haemorrhage. Their ages were 12 and 7 years respectively, and they had been receiving regular blood transfusion for the past 3 and 2 years respectively. They developed acute onset of headache, loss of consciousness and convulsions at 5 and 2 days respectively after their last blood transfusion. C-T scan of the brain showed massive intracranial haemorrhage with extension into the ventricles. The spontaneous intracranial haemorrhages in these two cases was probably multi-factorial in origin. The predisposing factors included recent blood transfusion, prolonged prothrombin time and partial thromboplastin time as well as reduced platelet count.
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PMID:Fatal intra-cranial haemorrhage in 2 cases of beta-thalassaemia major. 775 64

Four children with beta-thalassemia intermedia ages 7 to 11 years developed a clinical picture characterized by headache, hypertension, convulsion, and cerebral hemorrhage after blood transfusion. Successive transfusions did not result in a similar picture. Factors responsible for this syndrome are discussed.
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PMID:Convulsion after blood transfusion in four beta-thalassemia intermedia patients. 782 50

Cyclosporin A (CsA) has been shown to be useful in the prophylaxis of acute graft-versus-host-disease (GVHD). However, this immunosuppressive agent produces multiple side-effects including nephrotoxicity, hypertension, hypertricosis, gum hyperplasia, infections, and neurotoxicity. We report a retrospective analysis of neurotoxicity in 625 recipients transplanted for thalassemia and given CsA as part of GVHD prophylaxis. Neurotoxicity consisted in mental status changes, tremor, headache (grade 1), visual disturbance and cortical blindness (grade 2) and seizures and coma (grade 3). The overall toxicity was 28.8% and the incidence of convulsions was 10.1%. Neurological findings were reversible after temporary reduction or discontinuation of CsA. Class 3 patients, when prepared with protocol 6 (Bu 14 + Cy 200 and CsA for GVHD) or when they developed acute GVHD, had the highest risk of convulsions. Age, sex, different conditioning regimens, different anticonvulsive prophylaxis, liver damage due to iron-overload and/or to chronic inflammation did not influence the occurrence of CsA-related CNS toxicity. The occurrence of acute GVHD with concomitant use of high-dose corticosteroids is the single significant predisposing factor in the occurrence of convulsions. Grades 1 and 2 of neurotoxicity occurred earlier and were not influenced even by acute GVHD.
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PMID:CsA-associated neurotoxicity and ineffective prophylaxis with clonazepam in patients transplanted for thalassemia major: analysis of risk factors. 883 9

Thromboembolic (TE) events have been frequently reported in beta-thalassemic patients in association with known risk factors such as diabetes, complex cardiopulmonary abnormalities, hypothyroidism, liver function anomalies, and postsplenectomy thrombocytosis. In a recent survey involving 9 Italian thalassemic centers, we identified 32 patients with TE episodes in a total of 735 subjects, of whom 683 had thalassemia major and 52 thalassemia intermedia, corresponding to 3.95 and 9.61%, respectively. There was a great variation in localization: the main one (16/32) was CNS, with a clinical picture of headache, seizures and hemiparesis. Other localizations were the pulmonary (3 patients), mesenteric (1 patient) and portal (2 patients) sites. There were 6 cases of deep venous thrombosis (2 in the upper limbs, 4 in the lower ones). Intracardiac thrombosis was found in 2 subjects and clinical and laboratory signs of DIC were observed in 2 others during pregnancy. Since our patients with TE events present a statistically significantly higher incidence of associated dysfunction (cardiomyopathy, diabetes, liver function anomalies, hypothyroidism) than those without TE events (50 vs. 13.8%), we suggest close monitoring of those patients who are at higher risk of developing TE events because of the presence of one or more of these predisposing factors.
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PMID:Thromboembolic events in beta thalassemia major: an Italian multicenter study. 985 99

We report marked intracerebral calcification in eight thalassemic patients with hypoparathyroidism, followed regularly at the Haematology Research Center, Department of Pediatrics, Shiraz, Iran. Their mean age was 16.8 years (range 12-21 years). Six of the eight patients with thalassemia were females. The daily dose of calcitriol was between 0.01 and 0.1 microg/kg b. wt. Calcium-phosphate metabolic control was good or satisfactory in all patients. Three patients had at least one episode of generalized convulsions caused by hypocalcemia, before and during treatment. One patient complained of chronic headache and another patient had a low intelligence quotient. All were on treatment with calcitriol and oral calcium supplementation. The mean serum ferritin concentration was 3225 microg/l (range 2000-6000 microg/l). Calcification was present in the cerebral hemispheres, thalamic nuclei, basal ganglia, the internal capsule, part of the caudate nuclei and the posterior fossa. There was no history of birth asphyxia, head trauma, infections or metabolic diseases in any of the patients. No relationship was observed between the degree of cerebral calcification and the severity of hypoparathyroidism at diagnosis. Our observations stress the importance of a periodic assessment of calcium metabolism, prompt treatment of the endocrinopathy and strict control of calcium metabolism.
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PMID:Hypoparathyroidism with extensive intracerebral calcification in patients with beta-thalassemia major. 1294 1

Neuroimaging and management advances require review of indications for excluding cerebral venous sinus (sinovenous) thrombosis (CSVT) in children. Our goals were to examine (i) clinical presentations of CSVT, (ii) prothrombotic risk factors and other predisposing events, (iii) clinical and radiological features of brain lesions in CSVT compared with arterial stroke, and (iv) predictors of outcome. We studied 42 children with CSVT from five European paediatric neurology stroke registries. Patients aged from 3 weeks to 13 (median 5.75) years (27 boys; 64%) presented with lethargy, anorexia, headache, vomiting, seizures, focal signs or coma and with CSVT on neuroimaging. Seventeen had prior chronic conditions; of the 25 previously well patients, 23 had recent infections, eight became dehydrated and six had both. Two children had a history compatible with prior CSVT. Anaemia and/or microcytosis (21 probable iron deficiency, five haemolytic, including two with sickle cell disease and one with beta-thalassaemia) was as common (62%) as prothrombotic disorder (13/21 screened). High factor VIII and homozygosity for the thermolabile methylene tetrahydrofolate reductase polymorphism were the commonest prothrombotic disorders. The superficial venous system was involved in 32 patients, the deep in six, and both in four. Data on the 13 children with bland infarction and the 12 with haemorrhage in the context of CSVT were compared with those from 88 children with ischaemic (AIS) and 24 with haemorrhagic (AHS) arterial stroke. In multiple logistic regression, iron deficiency, parietal infarction and lack of caudate involvement independently predicted CSVT rather than arterial disease. Five patients died, three acutely, one after recurrence and one after 6 months being quadriparetic and blind. Follow-up ranged from 0.5 to 10 (median 1) years. Twenty-six patients (62%) had sequelae: pseudotumour cerebri in 12 and cognitive and/or behavioural disabilities in 14, associated with epilepsy in three, hemiparesis in two and visual problems in two. Eighteen patients, including six with haemorrhage, were anticoagulated. Older age [odds ratio (OR) 1.54, 95% confidence limits (CI) 1.12, 2.13, P = 0.008], lack of parenchymal abnormality (OR 0.17, 95% CI 0.02, 1.56, P = 0.1), anticoagulation (OR 24.2, 95% CI 1.96, 299) and lateral and/or sigmoid sinus involvement (OR 16.2, 95% CI 1.62, 161, P = 0.02) were independent predictors of good cognitive outcome, although the last predicted pseudotumour cerebri. Death was associated with coma at presentation. Of 19 patients with follow-up magnetic resonance (MR) venography, three had persistent occlusion, associated with anaemia and longer prodrome. A low threshold for CT or MR venography in children with acute neurological symptoms is essential. Nutritional deficiencies may be modifiable risk factors. A paediatric anticoagulation trial may be required, after the natural history has been further established from registries of cases with and without treatment.
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PMID:Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome. 1569 61

Erythema infectiosum (fifth disease) is the most common clinical presentation of acute parvovirus B19 infection in infancy. In healthy adults, most cases of infection are asymptomatic or accompanied by a flu-like syndrome like headaches and myalgia. Haematological manifestations are dominated by transient aplasia of erythroid progenitor cells which remains asymptomatic in most of non immunocompromised patients. Patients with sickle cell disease, thalassemia or other disorders associated with shortened red blood cell survival are at particular risk for marked anemia or red blood cell aplasia. In immunosuppressed patients, anemia may be chronic because of persistent viral load. Neutropenia, lymphopenia or thrombocytopenia have also been reported in acute parvovirus B19 infection. Mechanisms of these cytopenias are not yet elucidated. We present two patients with thrombopenia and/or neutropenia but without anemia due to acute parvovirus B19 infection.
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PMID:[Neutropenia and/or thrombocytopenia due to acute parvovirus B19 infection]. 1941 Dec 38


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