1/8. Intraoperative blood salvage during cesarean delivery in a patient with beta thalassemia intermedia.In this case report, we report a patient with a placenta accreta and thalassemia intermedia undergoing cesarean delivery. There are no data regarding the use of cell salvage in patients with thalassemia. During the course of her surgery, she lost approximately 9000 mL of blood. Of this blood, 2250 mL of concentrated red cells were collected, washed, and returned to the patient. During processing, increased hemolysis was noted in the effluent line of the cell salvage machine, which resolved by increasing the wash volume. The patient's postoperative course was uneventful. This case would suggest that cell salvage in patients with thalassemia can be performed safely; however, further study is warranted. IMPLICATIONS: This case report details the safe administration of cell salvage in a patient with beta thalassemia undergoing cesarean delivery. Cell salvage is the collection, washing, and re-administration of blood lost during surgery. This process has not been previously reported in a patient with this type of blood disease.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
2/8. Dominant beta-thalassemia with hemoglobin Hradec Kralove: enhanced hemolysis in the spleen.We describe a 6-year-old girl and her mother with dominant beta-thalassemia due to hemoglobin Hradec Kralove (Hb HK). Both patients presented microcytic anemia, jaundice, splenomegaly, cholelithiasis, and recurrent hemolytic bouts. Osmotic resistance tests using saline and coiled planet centrifugation revealed the increased fragility of the red cell membrane. On the other hand, the glycerol lysing time was prolonged, and results of the isopropanol test were weakly positive. Despite mimicking the features of hereditary spherocytosis, the results of the genetic analyses verified the second reported family with Hb HK (codon 115, GCC [Ala] --> GAC [Asp]). splenectomy was effective for the amelioration of hemolysis. Of 7 reported patients with Hb variants at beta-globin codon 115 (Hb Madrid and Hb HK), 5 underwent splenectomy. Because of the variable augmentation of extramedullary hemolysis in dominant beta-thalassemias, genotyping is necessary for determining the clinical indication of splenectomy.- - - - - - - - - - ranking = 0.75keywords = thalassemia (Clic here for more details about this article) |
3/8. Hyperhemolysis syndrome in anemia of chronic disease.BACKGROUND: Occasional cases of delayed hemolytic transfusion reaction (DHTR) demonstrate severe and persistent hemolysis and are referred to as hyperhemolysis syndrome. This syndrome usually occurs in patients with sickle cell disease and possibly thalassemia who receive multiple transfusions. There are few such clinical reports in patients without hemoglobinopathies. CASE REPORT: A 67-year-old woman with anemia and a history of four previous transfusions was admitted with shortness of breath and a hematocrit (Hct) level of 27 percent. The patient was group O with a negative antibody screen. She received 1 unit of electronically cross-matched red blood cells (RBCs) and was discharged. Thirteen days later she returned to hospital with weakness and a Hct level of 23 percent. The antibody screen now demonstrated anti-K alloantibody. The direct antigloblulin test (DAT) was positive with both anti-immunoglobulin g and anti-complement (C3). Anti-K was recovered in the eluate. The previously transfused RBC unit was positive for presence of the K antigen. The patient's RBCs were negative for the presence of K antigen. Other laboratory data confirmed ongoing hemolysis, and a diagnosis of DHTR was made. She continued to display findings of active hemolysis for 9 more weeks requiring 19 units of RBCs. Thirty-four days after the original transfusion, her DAT remained positive and both the plasma sample and a RBC eluate demonstrated anti-K. CONCLUSION: The delayed hemolytic transfusion reaction with hyperhemolysis can occur among patients without hemoglobinopathies.- - - - - - - - - - ranking = 0.125keywords = thalassemia (Clic here for more details about this article) |
4/8. Urinary creatine: biochemical indicator for evaluation of sickle cell crises.In a patient with known sickle cell beta 0-thalassemia we measured serum lactate dehydrogenase (LD) activity and 24-h urinary creatine excretion rate as markers to evaluate sickle cell crises. We believe that a distinction based on biochemical findings can be made between hemolytic and painful vaso-occlusive sickle cell crises with muscular involvement. To assess hemolytic crises by objective biochemical measures, we have used assay of LD activity, and to assess painful crises with muscular involvement objectively, the 24-h urinary creatine excretion rate. We conclude that hemolytic crises are characterized by high serum LD activities. Furthermore, we conclude that--at least in this patient--painful crises are accompanied by high 24-h urinary creatine excretion rates. Our findings suggest that muscle involvement may play an important role in painful vaso-occlusive sickle cell crises.- - - - - - - - - - ranking = 0.125keywords = thalassemia (Clic here for more details about this article) |
5/8. Acute Wilson's disease and thalassemia minor: a case report.The case of a 17-year-old Italian girl with thalassemia minor and fatal acute Wilson's disease together with severe hemolysis is reported. The fortuitous(?) occurrence of both genetically determined conditions in the same patient was the source of delayed diagnosis of the metabolic disorder.- - - - - - - - - - ranking = 0.625keywords = thalassemia (Clic here for more details about this article) |
6/8. Aberrant congenital dyserythropoietic anemia with negative acidified serum tests and features of thalassemia in a Kurdish family.Three siblings of a Kurdish Jewish family with clinical and hematologic findings compatible with congenital dyserythropoietic anemia (CDA) are described. All patients presented with mild anemia, marked hyperbilirubinemia and splenomegaly. The bone marrow morphology and ultrastructure of the normoblasts was typical of CDA type II and there was strong agglutination of the patients' red blood cells by anti-i serum. These patients displayed two features that were not characteristic of CDA type II, namely, the acidified serum lysis test was negative on more than 10 occasions, and high levels of Hb A2 were observed in two siblings. In one of the siblings, abnormal globin-chain synthesis was found and alpha-chain production exceeded beta-chain production, as in beta-thalassemia minor. In the light of the above findings, our patients are perhaps best classified as having aberrant CDA with features of thalassemia.- - - - - - - - - - ranking = 0.75keywords = thalassemia (Clic here for more details about this article) |
7/8. Initiation codon mutation (ATG --> ATA) of the beta-globin gene causing beta-thalassemia in a Swedish family.An initiation codon mutation ATG-->ATA of the beta-globin gene was found in seven members of three generations of a family living in northern sweden. This mutation, which has not previously been described, changes the initiation codon for methionine into a codon for isoleucine and will then result in a beta zero-thalassemic phenotype. The affected family members all present hematological findings typical for beta-thalassemic trait, with slight anemia, marked microcytosis, and increased levels of Hb A2.- - - - - - - - - - ranking = 0.5keywords = thalassemia (Clic here for more details about this article) |
8/8. Acute hemolysis in association with hepatitis B infection in a child with beta-thalassemia trait.Autoimmune hemolytic anemia may occur in the course of some viral diseases such as Coxsackie virus, cytomegalovirus, Epstein Barr virus, Influenza A, herpes simplex virus, and rarely hepatitis b virus infection. The role of being heterozygous for beta-thalassemia in hemolysis during acute viral hepatitis is not known. In this report, we present an eight-year-old boy with jaundice and anemia. The diagnosis of hepatitis b virus infection and hemolytic anemia were made on the basis of physical and laboratory findings. A hemoglobin electrophoresis revealed that the child was heterozygous for beta-thalassemia. No specific etiology could be found for hemolytic anemia. It remained unclear whether hemolytic anemia in this patient was merely a coincidental finding or whether hepatitis b virus infection and beta-thalassemia trait had played a role in causing hemolysis.- - - - - - - - - - ranking = 0.875keywords = thalassemia (Clic here for more details about this article) |