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

A number of Saudi children (31) with sickle cell disease and thalassemia underwent splenectomy: 12 for frequent blood transfusions, 15 for chronic hypersplenism (most of whom were also the recipients of periodic blood transfusion) and 4 for splenic abscess. The mean age of splenectomy was 8.8 years (8 months-18 years). Eight patients had sickle cell disease, 14 beta-thalassemia and 9 had sickle cell thalassemia. All patients received prophylaxis against pneumococcal infection. There was one postoperative death most probably due to sepsis. Sixteen of those who required frequent preoperative blood transfusions needed no more transfusions, while in 7 the need for transfusions decreased significantly (p less than 0.05). For those with hypersplenism, there was a significant postoperative increase in total hemoglobin (P less than 0.001), RBC (P less than 0.001) and platelet counts (p less than 0.02); and a substantial decrease in reticulocyte counts (p less than 0.05). The common post splenectomy complications were chest infection and a brief episode of pyrexia, but without undue morbidity. The study establishes a definite place for splenectomy in a selected population of children with sickle cell disease and thalassemia.
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PMID:Splenectomy in children with sickle cell disease and thalassemia. 263 77

Patients afflicted by homozygous beta-thalassemia suffer from severe anemia and hypersplenism and are dependent on blood transfusions. The consequent hypoxia and massive tissue iron deposition lead to concomitant cardiac, hepatic, and endocrine system failure. Hemodynamic changes related to gestation may aggravate the underlying multiorgan damage of the pregnant mother and lead to high fetal wastage. These entanglements may be prevented by performing thorough maternal and fetal surveillance and by maintaining the hemoglobin concentration above 10 g/dL. We describe a successful full-term pregnancy in a patient with transfusion-dependent homozygous beta-thalassemia major.
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PMID:Successful full-term pregnancy in homozygous beta-thalassemia major: case report and review of the literature. 264 25

Since 1973, 419 children (233 boys, 186 girls) with chronic haemolytic anaemia (mean age, 10.2 +/- 3.5 years) have been splenectomised. Fifty-three (12.6%) have also been cholecystectomised because of cholelithiasis. Between 1973 and 1982, cholecystectomy and splenectomy were performed at the same time in symptomatic patients. Later, between 1983 and 1987, cholecystectomy was also performed in clinically asymptomatic patients with positive sonographic findings. In the first period, 11 thalassaemic patients (45.8%) were cholecystectomised a short period after splenectomy (in two cases, only 2 and 3 weeks later). Between 1983 and 1987, when asymptomatic patients with cholelithiasis were screened by sonography, simultaneous cholecystectomy and splenectomy were performed in 13.6% of the patients with thalassaemia, in 41% of the patients with sickle cell or microdrepanocytic disease, and in 22.2% of the patients with spherocytosis. As there is a high incidence of asymptomatic cholelithiasis and hypersplenism in patients with chronic haemolytic anaemia, screening for gallstones should precede splenectomy. Simultaneous cholecystectomy should be performed in patients with cholelithiasis.
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PMID:Experience of splenectomy and cholecystectomy in children with chronic haemolytic anaemia. 273 20

The in vitro immune functions of peripheral blood lymphocytes have been studied in 12 children with beta-thalassaemia major and hypersplenism. The study was performed prior to splenectomy and on the 2nd, 6th, 15th and 30th day after splenectomy. It was found that before splenectomy, patients had low numbers of blood leucocytes, normal rosette and T3 lymphocyte counts, low T4 and normal T8 lymphocyte counts with a T4/T8 ratio below 2, impaired T lymphocyte mitogenic responses induced by PHA, increased numbers of polyvalent and monovalent B lymphocytes and normal immunoglobulin levels of IgG and IgA. After splenectomy, especially on the 2nd day, leucocytosis, a significant decrease of T cells and their subsets and a reduction of the IgM levels were found. These parameters, except the IgM levels, increased until the 30th day after splenectomy and reached presplenectomy values. On the 2nd day after splenectomy, large mononuclear cells behaving like immunocytes appeared in the peripheral blood. They had the phenotype of T3, T4, T8, B lymphocytes and OKM1 monocytes. All the large mononuclear cells increased significantly on the 6th day after splenectomy and remained elevated during the whole study (30 days). The T4/T8 ratio was increased, but no increase was found in the functional responses of T lymphocytes.
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PMID:Immunological profile after splenectomy in children with beta-thalassaemia major. 312 90

Thirty-five infants and children with beta-thalassemia major and 12 with beta-thalassemia trait were studied. Their ages ranged between 6 months and 12 years. Thirty-three were males and 14 females. Spontaneous rosette (E1-RFC), total rosette (E2-RFC), enumeration of T lymphocyte subsets using monoclonal antibodies (OKT3, OKT4, and OKT8), migration inhibition factor (MIF) assay and in vivo delayed hypersensitivity skin reactions were tested. Lower mean T cell population was present in thalassemia major but not the trait. The helper/suppressor ratio was decreased in patients with evidence of hypersplenism. Patients who had suffered from pneumonia or hepatitis manifested lower mean T cell count, depletion of helper cells and decreased helper/suppressor ratio. They also showed depressed delayed cutaneous hypersensitivity and MIF activity. Study of the cell-mediated immunity in patients with thalassemia might be useful to detect those who could be-more susceptible to infections.
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PMID:T cell functions in infants and children with beta-thalassemia. 312 38

Most patients homozygous for beta thalassaemia have beta thalassaemia major, a severe illness requiring regular blood transfusions. However, some homozygotes remain well without regular transfusions and are described by the term thalassaemia intermedia. Three factors have now been identified which may result in beta thalassaemia intermedia: the inheritance of mild beta+ thalassaemia mutations, the co-inheritance of alpha thalassaemia and the inheritance of factors enhancing gamma-globin gene expression. In addition other less common genetic interactions also result in thalassaemia intermedia such as the compound heterozygous state for beta and delta beta thalassaemia. These patients need careful clinical follow up, especially since the complications of hypersplenism and iron overload (even in the absence of blood transfusion) can occur.
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PMID:Thalassaemia intermedia. 333 12

Reports of acute splenic sequestration crises in adults with sickle cell hemoglobin C disease or sickle cell thalassemia are rare, although an enlarged and distensible spleen persists in half of these patients. Seven episodes of acute splenic sequestration crises in four adults, two with sickle C disease and two with sickle thalassemia, are described. The crises were life-threatening and recurrent in all, but there were no fatalities. One patient had mild steady-state thrombocytopenia suggesting hypersplenism. Technetium 99m/sulfur colloid scanning of the spleen during the acute splenic sequestration crises in three patients showed almost total lack of splenic uptake or decreased uptake with intrasplenic filling defects thought to be splenic infarcts or hematomas on follow-up computed tomographic scanning. The scanning abnormalities resolved following recovery from the crises. Acute splenic sequestration crises probably are common in adults with sickle C disease and sickle thalassemia but may be underdiagnosed or misdiagnosed as splenic infarctions. The hematologic and splenic findings during acute splenic sequestration crises resemble those following splenic vein ligation in animals.
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PMID:Acute splenic sequestration crises in adults with sickle cell disease. 370 82

The blood requirements using packed red cells (C) and the mean annual haemoglobin level (Hb ma), corresponding to the mean of all haemoglobin levels before (Hb pre T) and after (Hb post T) transfusion over 1 year, have been measured in 14 splenectomized and regularly transfused thalassaemia major patients. In these non-hypersplenic patients a linear relation was determined between C and Hb ma. The blood consumption C has also been measured in 5 patients with hypersplenism. The comparison of C between these two groups of patients offered the possibility of defining the limit of C, for a given value of Hb ma, above which hypersplenism is indicated. The measurement of Hb ma requires the value of Hb post T, a parameter which is sometimes difficult to obtain in practice. For this reason, a method of calculating Hb post T using Hb pre T, C and the number of transfusions over 1 year (N) is described. The relation between these parameters is: Hb post T = Hb pre T + C/N X 0.23. To use this formula, the conditions must be as follows: the haematocrit of blood units must be between 75% and 85%, and the blood volume of each transfusion in a given patient must be identical throughout the year.
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PMID:[Transfusion requirements and mean annual hemoglobin level in thalassemia major]. 380 40

Partial dearterialization of the spleen is a surgical method aimed at reducing the volume of the spleen whilst preserving its defensive properties against bacterial infections. Four children with thalassaemia major and one with congenital dyserythropoiesis who developed hypersplenism in the course of their disease underwent the operation and were subsequently followed up for periods of 24 to 36 months. The effectiveness of the method was demonstrated by a smaller enlargement of the spleen, a lesser need for transfusions, a longer life of 51 Cr-labelled red cells and more numerous circulating platelets. Surgery was also effective in reducing post-transfusional iron overload, as shown by the decrease and stability of serum ferritin levels in all children subjected to iron chelation. No episode of infection or changes in serum IgM occurred during the follow-up period, and radioisotopic studies of the spleen showed that macrophage function was preserved in the remaining splenic tissue. One child inexplicably developed thrombosis of the splenic vein 1 year after surgery.
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PMID:[Hypersplenism in thalassemia major. Treatment by partial dearterialization of the spleen. Preliminary results apropos of 5 cases]. 623 27

The indications and management of blood transfusion in the haemoglobinopathies have been reviewed. The sickle cell diseases that require transfusion support are sickle cell anaemia, sickle haemoglobin-C and -D diseases and sickle beta-thalassaemia. Homozygous beta-thalassaemia (Cooley's anaemia) is the major problem among the thalassaemias. The pathophysiology of the sickle cell disorders is largely based on the secondary effects of increased blood viscosity, whereas in the thalassaemias the defect is ineffective haematopoiesis. In the former the major problems occur as manifestations of vaso-occlusive crises with disseminated bone and abdominal pain, priapism, stroke and leg ulcers. Bone infarction and aseptic necrosis occur but the widespread bone changes, underdevelopment and haemochromatosis that complicate the thalassaemia are not prominent. Transfusion therapy in the sickle cell diseases is mainly episodic and is guided by the frequency of crises and the severity of vaso-occlusive complications. Partial exchange transfusion and the maintenance of haemoglobin A concentrations at 40 to 50 per cent is frequently indicated. In the thalassaemias, maintenance of haemoglobin levels is essential for normal growth and development. The problem of haemochromatosis is very serious. With hypertransfusion regimens the haemoglobin and haemotocrit are maintained above 12-13 g/dl and 35 per cent. The resulting benefit appears to be reduced blood volume, less iron turnover, and less intestinal iron absorption. The splenomegaly in these disorders is frequently associated with hypersplenism requiring well-timed splenectomy. Chronic and intensive chelation is necessary to prevent the ravages of iron overload. The availability of automated equipment for in vivo and ex vivo blood cell separation has brought new possibilities for improving the management of these haemoglobinopathies. It is feasible, but not as yet practical, to offer transfusions of neocytes (red cells with a mean age of 30 days) which have a 50 per cent longer survival than routine red cell preparations (mean age of 60 days). Neocytes can be prepared ex vivo from fresh routine blood donations using blood cell separator devices. The result is reduced transfusion requirements. A more recent suggestion for using the new technology is to remove the patient's oldest and most abnormal corpuscles on the basis of buoyant density and replacing them with neocytes . Thus the short-lived abnormal red cells would be removed before they could unload their iron. With automation it is possible to perform these procedures on an outpatient basis.
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PMID:Transfusion support for haemoglobinopathies. 637 80


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