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

In 30 children with homozygous beta-thalassemia the hemostasis screening tests (bleeding time, PT, PTT), platelet count and specific assays of clotting factors were carried out 25 days after their last transfusion. PT, PTT, and bleeding time showed minor variations; considerable thrombocytosis was found in splenectomized patients. Factors IX and XII were decreased in a high proportion of patients, the vitamin K-dependent factors (II, VII, IX, X) were slightly reduced and factors I, V and VIII remained within the normal range in a majority of patients. Hepatic failure resulting in defective protein synthesis does not explain the more marked impairment of factors XI and XII, which might be secondary to activation of the intrinsic coagulation and/or kallikrein systems following intravascular haemolysis and multiple blood transfusions.
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PMID:Screening coagulation tests and clotting factors in homozygous beta-thalassemia. 10 56

Iron deficiency anaemia secondary to menorrhagia was observed in a woman of Greek Cypriot origin. Moderate thrombocytopenia was also present. Treatment with parenteral and oral iron produced a transient thrombocytosis, the platelet count then returning to normal. Subsequent analysis revealed that she also carried the alpha1-thalassaemia trait. Previous reports of thrombocytopenia responsive to iron treatment are reviewed.
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PMID:Thrombocytopenia and iron deficiency anaemia in a patient with alpha1-thalassaemia trait. Response to iron therapy. Case report. 41 76

Thrombocytosis and morphological changes in shape and size of circulating platelets are commonly found in splenectomized thalassemia patients. Functional abnormalities of the platelets are evidently related to their fragile nature. Spontaneous aggregation of platelets can easily occur following application of mild pressure either from a stirring magnetic bar or due to centrifugal force. Platelets are hyper-reactive to chemical stimulation and the release of platelet granule contents (eg ATP) together with other membranous lipid metabolites, thromboxane A2 and malondialdehyde is markedly enhanced. The lipid soluble antioxidant, vitamin E is depleted from various blood compartments. The findings suggest that circulating platelets in splenectomized thalassemia are continuously attacked by yet to be identified blood borne factor(s) and the defective platelets could play a pivotal role in the pathogenesis of hypoxemia.
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PMID:Pathogenesis of hypoxemia. 129 90

Partial splenic embolization is an alternative procedure to total splenectomy in patients with hypersplenism, and was performed in 10 patients with beta-thalassaemia major who were then followed for 5 to 7 years. The results were compared with those of a 7-yr follow-up of 6 splenectomized thalassaemics. The blood consumption decreased and the leucocyte counts increased in both groups of patients. However, after partial splenic embolization, severe thrombocytosis--which is typical of splenectomized patients--did not develop and there were no severe complications from the operation, such as infections or reappearance of hypersplenism. In addition, the minor surgical injury and avoidance of abdominal scars were further advantages of partial splenic embolization over total splenectomy.
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PMID:Effectiveness of partial splenic embolization as treatment for hypersplenism in thalassaemia major: a 7-year follow up. 139 39

The authors report about one case of left branchial arterial thrombosis in a 6-year-old child with thalassemia-sickle cell disease. In their opinion, although this hemoglobinopathy usually causes microthrombosis, its existence at the same time as that of the brachial thrombosis does not seem to be a coincidence. The actual origin of this disease remains to be found, as well as the role that thrombocytosis may play. The authors emphasize the relative rarity of thrombolytic arterial diseases in African Negroes and advise practitioners to recognize them, because their diagnosis, often established late in our climates, has severe consequences.
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PMID:[Acute thrombosis of the brachial artery and beta-thalassemia-sickle cell anemia]. 161 6

A patient, double heterozygous for HbE-beta-thalassaemia, had recurrent thromboembolic complications following splenectomy. In addition to marginally decreased protein C and S plasma levels, laboratory studies revealed a persistent thrombocytosis and markedly elevated plasma concentrations of platelet factor 4 (PF4). PF4 neutralizes the heparin anticoagulant activity. The increased PF4 levels explained the initial heparin resistance observed during anticoagulant treatment in this patient. Subsequent heparin loading tests revealed an abnormal reaction of the PF4 plasma levels, i.e. no increase of PF4. However, upon repeated heparin injections this PF4 response normalized, which may be due to depletion of the endothelial cell associated heparin mobilizable PF4 pool. These observations may be of relevance for the treatment of similar patients.
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PMID:Thromboembolic complications in an asplenic HbE-beta-thalassaemia patient. 260 89

A microcomputer program (BCDE) has been developed to analyze automated blood cell counts and differentials' similarity to normal values or to 36 disease categories. In 50 normal subjects, the analytic program listed the correct diagnosis as the first diagnosis in 49 cases (the only diagnosis in 44) and second of two diagnoses in one case. In 182 subjects with known hematologic disorders, the correct diagnosis was listed first in 134 and second or third in an additional 40. Subjects with iron deficiency, heterozygous thalassemia, immune thrombocytopenia, anemia of chronic disease, reactive thrombocytosis, acute infection, and chronic leukemia had the disorder identified as the most likely one by the analytic program with both sensitivity greater than 80% and specificity greater than 98%. Subjects with acute leukemia, folate deficiency, sickle cell anemia, cytotoxic chemotherapy, and chronic liver disease had the disorder identified as most likely by the program with a sensitivity less than 80%. In a different 11 cases with known hematologic status, a panel of 37 physicians identified the disorder(s) or normality only 72% of the time, whereas the analytic program listed the correct diagnosis first in 10 of 11 (91%). The analytic program appears useful for both triage of normal from abnormal data and for the initial differential analysis of abnormal data.
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PMID:Evaluation of BCDE, a microcomputer program to analyze automated blood counts and differentials. 330 76

Platelet changes in thalassemia included prolongation of bleeding time (30%), thrombocytosis (29%), decreased platelet retention (79%), and normal PF3 release. Platelet aggregation was found to be hyperaggregation in 50%, normal in 22%, and hypoaggregation in 28% of the studied patients. Platelet changes in thalassemia can be hyper-, normal, or hypofunction. The changes are likely to be related to many factors, particularly to the progress of the disease. Platelet hypoaggregation may lead to a bleeding problem. Platelet hyperaggregation and thrombocytosis were prominent in the splenectomized patients and/or the severely anemic group; antiplatelet drugs may be indicated in these patients.
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PMID:Platelet function tests in thalassemic children. 368 23

We determined the platelet count and MPV in 100 normal subjects, in 147 subjects with thrombocytopenia or thrombocytosis due to other than primary hematologic disorders, and in smaller groups with immune or septic thrombocytopenia or iron deficiency. In these groups, the inverse, nonlinear relation between MPV and platelet count was the same as in a previous study of normal subjects. The same relation between platelet volume and count was found in individual patients as platelet counts rose during recovery from immune or septic thrombocytopenia. The concomitant progressive fall in MPV during recovery from thrombocytopenia, at which times rapidly rising platelets counts were necessarily associated with a population of young platelets, suggests that magnitude of stimulation of thrombopoiesis, not platelet age, is the major determinant of platelet volume. In contrast, as compared to normal persons with similar platelet counts, MPV was increased in subjects with heterozygous thalassemia but decreased in patients receiving chemotherapy for malignancy or renal transplantation. The undefined mechanism of regulation of platelet formation from megakaryocytes, reflected by the inverse relation of platelet size and count, thus seems altered in these disorders. Platelet volume is an easily obtained variable that appears to be useful in the evaluation of abnormal platelet production.
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PMID:The inverse relation between platelet volume and platelet number. Abnormalities in hematologic disease and evidence that platelet size does not correlate with platelet age. 682 64

While venous thrombosis and pulmonary embolism are rare among the Chinese, two of nine patients with haemoglobin H disease developed these complications after splenectomy. The clinical data of the two patients were reported and the relevant literature reviewed. It was concluded that the persistent thrombocytosis and an intravascular haemolysis, particularly prominent in this form of thalassaemia, are contributory to the hypercoagulable state.
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PMID:Venous thrombosis in haemoglobin H disease after splenectomy. 696 15


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