1/21. Successful prevention of post-transfusion Rh alloimmunization by intravenous Rho (D) immune globulin (WinRho SD).Alloimmunization to the D blood group antigen following the transfusion of D-positive red blood cells to a D-negative recipient may be prevented in most persons by a prompt and adequate dose of Rho (D) immune globulin (RhIG). Until recently, the only RhIG approved by the US food and Drug Administration (FDA) for this indication required intramuscular injection, an inconvenient and painful route for the relatively large volume that may be required. We describe the successful prevention of Rh alloimmunization following the unintentional transfusion of D-positive red blood cells to a D-negative infant by the intravenous infusion of WinRho SD, a new RhIG that is FDA-approved for prevention of post-transfusion Rh alloimmunization by intravenous administration. We believe that this more convenient and less painful approach should be the treatment of choice for preventing Rh alloimmunization following the transfusion of D-positive red cells to a D-negative recipient.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/21. An acute hemolytic transfusion reaction due to anti-IH in a patient with sickle cell disease.BACKGROUND: A hemolytic transfusion reaction (HTR) due to anti-IH is reported in a patient with sickle cell disease (SCD). CASE REPORT: An 18-year-old woman with SCD and a complete phenotype on file had been identified as group B-positive with negative antibody-screening tests and had received 1 unit of packed RBCs. Ten days later, she was readmitted in painful crisis with a Hb of 4.2 g per dL. Antibody-screening tests and panel cells were positive at all test phases with a negative autocontrol, which suggested alloantibodies. Phenotypically matched group O RBCs were issued emergently. After the transfusion of 100 mL, the patient had an HTR with chills, fever, and tachycardia and laboratory findings of hemoglobinemia, hemoglobinuria, and negative DATs. A high-titer, IgM anti-IH with a high thermal amplitude (reactive with group O, but not group B RBCs at 37 degrees C) was identified. Autologous RBCs appeared to have normal I antigen expression, but less H antigen than pooled group B RBCs. She was given group B RBCs, uneventfully, by use of a blood warmer. CONCLUSIONS: This is a rare case of anti-IH as the cause of a HTR, as a serologic problem that may be seen in SCD, and as an autoantibody that may mimic an alloantibody. Ironically, this HTR resulted from the effort to provide phenotypically matched RBCs, which necessitated the selection of group O RBCs.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/21. Successful cord blood transplantation for sickle cell anemia from a sibling who is human leukocyte antigen-identical: implications for comprehensive care.We report the successful transplantation of umbilical cord blood stem cells from a sibling who is human leukocyte antigen-matched to a child with sickle cell anemia. Conditioning was with busulfan, cyclophosphamide, and antithymocyte globulin. time to neutrophil count >500/microL was 23 days and to platelet count >50,000/microL was 49 days. Full donor engraftment was achieved without graft-versus-host disease. This case demonstrates the potential usefulness of harvesting cord blood from full siblings of patients with sickle cell disease. Routine collection of umbilical cord blood from siblings should be considered for patients with sickle cell disease, and may increase acceptance and use of transplantation by families.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
4/21. hematopoietic stem cell transplantation after reduced-intensity conditioning as treatment of sickle cell disease.OBJECTIVE: Sickle cell disease generates considerable morbidity and mortality. Allogeneic hematopoietic stem cell transplantation (SCT) has the potential of curing the disease and halting end-organ damage. However, in older patients this treatment is associated with a significant risk of toxicity and death. SCT after reduced-intensity conditioning (RIC) might be a safer approach for the treatment of sickle cell disease. MATERIALS AND methods: A 22-year-old male had experienced multiple, life-threatening hemolytic crises. We treated him with G-CSF-mobilized stem cells from his heterozygote, human leukocyte antigen-matched brother after RIC with fludarabine and cyclophosphamide. GVHD prophylaxis consisted of cyclosporine (CyA) and mycophenolate mofetil (MMF). chimerism of peripheral blood mononuclear cells was evaluated using short tandem repeat analysis and hemoglobin analysis was performed by high-performance liquid chromatography. RESULTS: There were no major treatment-related toxicities. At day 30 after transplantation the patient had mixed hematopoietic chimerism, which later converted to full chimerism. Hemoglobin analysis revealed 3.4% HbA(2), 1.0% HbF, and 41.2% HbS, which essentially is the same hemoglobin partition as in his brother's blood. MMF was discontinued on day 35 and CyA on day 120. After discontinuation of CyA the patient developed mild chronic GVHD, which resolved with continued CyA, low-dose steroids, and the retinoid isotretinoin. He is doing well on day 315 without evidence of GVHD. CONCLUSIONS: Allogeneic SCT after RIC is feasible in adult patients with sickle cell disease. Mixed chimerism is sufficient to relieve disease-related symptoms and is possibly correlated with less acute GVHD.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/21. Autologous immune complex nephritis associated with sickle cell trait: diagnosis of the haemoglobinopathy after renal structural and immunological studies.A renal tubular epithelial antigen (RTE)--anti-RTE autologous immune complex nephritis associated with sickle cell anaemia (SS) has been reported, but immune complex nephritis has never been described in patients with sickle cell trait (SA). During investigation of a child with "asymptomatic proteinuria" cryoprecipitable complexes of RTE-anti-RTE were detected in the serum and granular deposits of RTE, immunoglobulins, and complement localised on the glomerular basement membranes. Morphological and ultrastructural studies showed increased mesangial matrix, sickled red blood cells in the glomeruli and vessels, and tubular and interstitial abnormalities. These findings prompted haemoglobin electrophoretic studies, which showed previously undiagnosed haemoglobin SA in this patient and her family. These observations suggest that nephritis mediated by similar immunopathogenic mechanisms may be associated with SS and SA haemoglobinopathy. Under some conditions patients with sickle cell trait may experience haemodynamic and oxygenation abnormalities, which may be aetiological factors in the immune complex nephritis associated with SS disease.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/21. Transfusion-related acute lung injury or acute chest syndrome of sickle cell disease? - A case report.PURPOSE: To describe how to differentiate transfusion-related acute lung injury from acute chest syndrome of sickle cell disease. Clinical features: A neurosurgical patient with sickle cell disease received two units of packed red blood cells postoperatively. Four hours later she developed progressive respiratory distress, diffuse geographical airspace disease and bilateral pulmonary edema. The patient recovered sufficiently to be transferred from the intensive care unit within four days. The temporal relationship to transfusion, features on computerized tomographic scan, and the rapid resolution of severe edema point to a diagnosis of transfusion related acute lung injury. Granulocyte or human leukocyte antigen antibodies in donor plasma may confirm a diagnosis of transfusion injury. CONCLUSION: The clinician should appreciate that erythrocyte transfusion to prevent or treat acute chest syndrome may cause transfusion related acute lung injury, a condition that mimics, exacerbates or possibly triggers the syndrome it was intended to treat.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/21. Delayed haemolytic transfusion reaction and hyperhaemolysis complicating peri-operative blood transfusion in sickle cell disease.We present a case of delayed haemolytic transfusion reaction and hyperhaemolysis syndrome in a patient with sickle cell disease. A 32-year-old woman with a history of sickle cell disease was scheduled for total hip replacement. She was transfused pre-operatively and suffered a delayed haemolytic transfusion reaction. Postoperatively the patient continued to haemolyse, despite the use of antigen compatible blood, suggesting that she had developed hyperhaemolysis syndrome following her delayed haemolytic transfusion reaction. Although rare, both conditions must be borne in mind when dealing with patients who have undergone multiple transfusions.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/21. Bystander immune cytolysis.In addition to alloimmune and autoimmune cell lysis, a third category of immune destruction of blood cells should be recognized. This additional immunologic response occurs when cells or tissues are injured by immunologic reactions in which the cells act as "innocent bystanders." One mechanism by which an immune response to an exogenous antigen leads to the destruction of autologous blood cells is the temporary development of autoantibodies. This is actually an alloimmune reaction which results in a temporary state of "pseudo"-autoimmunity. Although originally described as a type of hemolysis of autologous cells, the concept of bystander immune cytolysis has been extended to include other instances in which immune destruction of cells is caused by antibody that is not developed in response to intrinsic antigens on the cell being lysed. In recent years, compelling data have been presented documenting bystander immune cytolysis in a number of different clinical settings, and efforts have been made to define the mechanisms by which this occurs. physicians must be aware that some examples of immune lysis of autologous cells are, in reality, examples of temporary bystander immune cytolysis rather than true autoimmune disease. Furthermore, some alloimmune hemolytic reactions can result in lysis of bystander cells.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/21. Sickle cell disease and hemochromatosis.A 50 year-old patient with sickle cell anemia was seen who had received only two units of blood during his lifetime. He had marked iron overloading, cirrhosis of the liver, arthralgia, and mild glucose intolerance. We believe the iron overloading was associated with hereditary hemochromatosis rather than sickle cell anemia because he had HLA-A3 and b7 antigens, and hepatic iron deposits were primarily in parenchymal cells rather than Kupfer cells. The coexistence of either homozygous or heterozygous hemochromatosis should be suspected in sickle cell patients with organ damage from iron overloading.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/21. Intravenous desmopressin acetate in children with sickle trait and persistent macroscopic hematuria.Persistent gross hematuria associated with sickle hemoglobinopathy that fails to respond to conventional supportive therapy represents a difficult management dilemma. Two such patients with protracted, often painful, sickle trait macrohematuria are described. Both patients had normal renal anatomy and vasculature and had failed to respond to bed rest, intravenous hydration, and a trial of oral epsilon-aminocaproic acid. Patient 1 had normal coagulation function. Patient 2 had von Willebrand disease (decreased factor viii antigen and quantitative ristocetin cofactor activity). Patient 1 responded to intravenous desmopressin acetate at a dose of 0.3 microgram/kg with a 155% increase in factor viii clotting activity and a 135% increase in ristocetin cofactor and cessation of her macrohematuria within 18 hours after completion of the desmopressin infusion. She remained free of gross hematuria for 5 months with the exception of short-lived trauma-induced hematuria (in three voids) 6 weeks after desmopressin therapy. Patient 2 did not respond to intravenous desmopressin infusion despite a 234% and a 360% increase in factor viii clotting activity and ristocetin cofactor, respectively. Intravenous desmopressin acetate may be helpful in halting protracted significant macrohematuria associated with sickle trait hemoglobinopathy in some patients when conventional management fails.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
| | Next -> |