1/12. Flow cytometric determination of RBC survival in autoimmune hemolytic anemia.BACKGROUND: In cases of warm autoimmune hemolytic anemia (WAIHA), crossmatch incompatible RBCs are most often used for transfusion. The determination of the in vivo survival of transfused and autologous RBCs in WAIHA is helpful in the assessment of the efficacy of transfusion and other therapeutic interventions. CASE REPORT: A 38-year-old man presented with acute WAIHA, thrombocytopenia, and neutropenia. steroids and IVIG therapy were ineffective, and the patient received RBCS: Because of increasing hemolysis and persisting thrombocytopenia, splenectomy was performed, resulting in partial remission. Further improvement was achieved by immunosuppressive therapy. MATERIALS AND methods AND RESULTS: Survival of transfused and autologous RBCs was determined, using a flow cytometric method based on the determination of different blood group antigens of patient and donor RBCS: The survival of autologous and transfused RBCs before splenectomy was determined on two consecutive days. The life span of autologous RBCs remained rather stable at 69 and 64 hours on Days 10 and 11, respectively, whereas the life span of transfused RBCs decreased from 186 hours to 25 hours. After splenectomy, the life span of transfused RBCs almost normalized: 43 days at postsplenectomy Day 3 and 87 days at postsplenectomy Day 69. CONCLUSION: flow cytometry was successfully used to determine changing hemolytic activity during the clinical course of WAIHA. Additionally, the survival of transfused RBCs could be measured, which may be helpful to judge for the compatibility of allogeneic RBCS: Thus, we were able to show the therapeutic inefficacy of steroids and immunoglobulins, and quick improvement after splenectomy.- - - - - - - - - - ranking = 1keywords = survival (Clic here for more details about this article) |
2/12. High-dose chlorambucil for the treatment of chronic lymphocytic leukaemia and low-grade non-Hodgkin's lymphoma.chlorambucil has been used for many years for the treatment of low-grade B-cell lymphoproliferative disorders, including chronic lymphocytic leukaemia and low-grade non-Hodgkin's lymphoma. There is evidence in the literature that increasing the dose of chlorambucil produces better results than 'standard' doses in terms of response rates and overall survival. There is also evidence that this approach may be at least as effective as the use of fludarabine, as well as being very much less expensive. We describe a high-dose chlorambucil (HDC) regimen, which involves a sustained but intermittent dose of chlorambucil, i.e. 30 mg/d for 4 d per week for 4 weeks, followed by a further four courses at fortnightly intervals for 8 weeks (a total of eight 4-d courses) given as a single drug over an initial 12-week period. The outcome of treatment in previously treated and untreated patients was excellent, with a median time to treatment failure of 33 months for the patient cohort overall and for previously treated and chemotherapy-naive patients of 13 and 104 months respectively. In patients previously treated with fludarabine, 78% had a response. Autoimmune haemolytic anaemia was reversed in one patient. Toxicity, both haematological and other, was minimal. We propose that escalated-dose chlorambucil regimens should be compared with fludarabine in randomized controlled trials, rather than 'standard' lower dose protocols.- - - - - - - - - - ranking = 0.14285714285714keywords = survival (Clic here for more details about this article) |
3/12. Successful management of severe chronic autoimmune hemolytic anemia with low dose cyclosporine and prednisone in an infant.Autoimmune hemolytic anemia (AIHA) is characterized by shortened red cell survival due to the presence of autoantibodies directed against antigens on the red blood cell membrane. Corticosteroids and rarely intravenous immunoglobulin g are used in the treatment of AIHA. We report a six-month-old boy with severe AIHA who initially responded to high dose methylprednisolone (HDMP) and intravenous immunoglobulin g (IVIG) therapies but eventually became refractory. He was then treated with low dose cyclosporine and prednisone successfully. In conclusion low dose cyclosporine and prednisone should be kept in mind in severe IHA.- - - - - - - - - - ranking = 0.14285714285714keywords = survival (Clic here for more details about this article) |
4/12. Severe immune haemolysis after standard doses of Penicillin.Summary Penicillin causes immune haemolytic anaemia by the 'drug-adsorption' mechanism and typically occurs after prolonged exposure to large doses of the drug. Withdrawal of the drug is associated with improved red cell survival and gradual cessation of haemolysis. Although this complication is uncommon, it can be potentially serious. An unusual case is described herein. The patient was exposed to a short course (9 days) of standard dose penicillin but suffered acute severe haemolysis about 1 week after cessation of therapy. A high titre anti-penicillin antibody (1 : 512) not cross-reacting with cephalosporins, was demonstrated. The delay in the development of immune haemolysis vis-a-vis penicillin therapy may be due to the patient being immunologically naive to the drug. Penicillin may persist for weeks in circulation, coating red cells and providing continued antigenic stimulation for the development of anti-penicillin antibody.- - - - - - - - - - ranking = 0.14285714285714keywords = survival (Clic here for more details about this article) |
5/12. Activity of rituximab monotherapy in refractory splenic marginal zone lymphoma complicated with autoimmune hemolytic anemia.We describe the case of a 61-year-old patient with refractory splenic marginal zone lymphoma and secondary autoimmune hemolytic anemia, both successfully treated with rituximab. This case demonstrates that rituximab monotherapy might also be a valid therapeutic approach in marginal zone lymphoma and autoimmune hemolytic anemia after failure of first-line treatment. maintenance therapy, although expensive, could be useful to improve event-free survival in patients with unfavorable clinical behavior.- - - - - - - - - - ranking = 0.14285714285714keywords = survival (Clic here for more details about this article) |
6/12. Clinically significant allo-anti-I in an I-negative patient with massive hemorrhage.A 37-year-old white man who had never been transfused was admitted as an emergency patient with a ruptured spleen and a falling hematocrit (19% on admission). All crossmatches were incompatible. His serum contained anti-I, and his red cells (RBCs) were I-negative and strongly i-positive. Only 4 units of crossmatch-compatible I-negative frozen RBCs were available immediately. Because of the likelihood that more than 4 units would be required, chromium survival studies were performed using I-positive cells. Samples obtained at 15 and 30 minutes after injection revealed less than 1 percent survival of the donor RBCs. He received the 4 units of I-negative RBCs during the operation in addition to reinfusion of RBCs harvested from 1800 ml of blood aspirated from the abdominal cavity. The postoperative hematocrit remained greater than 30 percent and the bilirubin less than 1.5 mg per dl. Before recommending frozen storage of autologous RBCs, 51Cr labeled I-positive RBCs from the patient's daughter (obligate li heterozygote) were infused. Survival was 100 percent at 15 and 30 minutes, 90 percent at 3 hours, 85 percent at 26 hours; the remaining RBCs disappeared at a normal rate (T 1/2 27 days) over the succeeding 2 weeks. A repeat 51Cr-labeled RBC study with the original I-positive donor confirmed greater than 92 percent destruction in 90 minutes. The clinical significance of this allo-anti-l (apparently primarily against RBCs from homozygous I-positive donors) is in marked contrast to reported findings with auto-anti-l antibodies.- - - - - - - - - - ranking = 0.28571428571429keywords = survival (Clic here for more details about this article) |
7/12. Haemolytic anaemia in previously healthy adult patients with CMV infections: report of two cases and an evaluation of subclinical haemolysis in CMV mononucleosis.Whereas haemolytic anaemia is commonly encountered in infants and young children with cytomegalovirus (CMV) infections, it is an infrequent complication of CMV-induced infections in previously healthy adults. The data from 2 such patients are presented. One patient's Hb fell to a level of 36 g/l, and she required prednisone and blood transfusions. Her direct antihuman globulin test (DAT) was positive (IgG), and her red blood cell survival (51Cr) revealed a T 1/2 of 5 d. Both saline-agglutinating and low-molecular-weight cold agglutinins (CA) (4 degrees C) that reacted against both cord and adult cells were identified. In the second case, a moderate haemolytic anaemia (lowest Hb 87 g/l) was accompanied by negative DAT and CA studies. 20 other patients with CMV-mononucleosis were evaluated for evidence of subclinical haemolysis. Reticulocyte counts greater than 3.0% were noted in 9 of these patients. Haptoglobin values were below 0.5 g/l in 13 patients, and a positive DAT was recorded in 3/10 cases. This study documents haemolysis in many non-immunosuppressed adult patients with CMV infections. The mechanism responsible remains obscure.- - - - - - - - - - ranking = 0.14285714285714keywords = survival (Clic here for more details about this article) |
8/12. plasma exchange in autoimmune hemolytic anemia (AIHA).plasma exchange therapy in autoimmune hemolytic anemia (AIHA) was used in four patients (two with warm hemolytic anemia and two with cold hemolytic anemia). The size of each plasma exchange approximated 1 plasma volume; three consecutive daily exchanges removed 80-90% of the immunoglobulins--immunoglobulin g (IgG) and immunoglobulin m (IgM)-, complement (C3, C4), and reduced antibody titers. Transfusion requirements dramatically decreased after plasma exchange in each case. In two patients, red blood cell (RBC) survival studies were performed to more accurately assess the effect of plasma exchange therapy, since steroid and/or immunosuppressive therapy was given concomitantly. In one case of cold AIHA, homologous 51Cr-RBC were injected 4 days prior to plasma exchange and repeat injection (same donor) following completion of plasma exchange. The survival curve prior to plasma exchange therapy had a T 1/2 = 7.8 days (r = -0.988) and after plasma exchange therapy had a T 1/2 = 20.4 days (r = 0.925). RBC survival studies using homologous 51Cr-RBC were also performed in a patient with warm AIHA. The survival curve before plasma exchange had a T 1/2 = 2 days (r = -0.95), and postplasma exchange a T 1/2 = 1.8 days (r = -0.91). plasma exchange therapy seems to have a beneficial effect in cold rather than warm autoimmune hemolytic anemia.- - - - - - - - - - ranking = 0.57142857142857keywords = survival (Clic here for more details about this article) |
9/12. beta 0-Thalassemia complicated by autoimmune hemolytic anemia. Globin synthesis during immunosuppressive therapy.The unusual occurrence of both beta o-thalassemia and autoimmune hemolytic anemia (AIHA) in a 28-month-old child is reported to illustrate special diagnostic and therapeutic studies. The diagnosis of beta o-thalassemia was established by clinical, family and globin synthesis studies. The diagnosis of AIHA was confirmed by the shortened survival of transfused autologous and homologous red blood cells and by positive direct antiglobulin tests. During treatment of the AIHA were corticosteroids and various immunosuppressive drugs, globin synthesis studies were performed to evaluate the coincidental effects on gamma/alpha-globin chain synthetic ratios. A 50% increase in the gamma/alpha synthetic ratio during cyclophosphamide treatment suggests that further studies of the possible benefits of cytotoxic drug therapy in beta-thalassemia may be indicated.- - - - - - - - - - ranking = 0.14285714285714keywords = survival (Clic here for more details about this article) |
10/12. Treatment of autoimmune hemolytic anemia with Vinca-loaded platelets.We worked with a new strategy for the treatment of autoimmune hemolytic anemia (AIHA) of the warm antibody type. Platelets were loaded with vinca alkaloids and reacted with antiplatelet antibodies to facilitate their phagocytosis by macrophages, resulting in a measure of selective delivery of the drugs to the cells of the mononuclear phagocyte system, which destroy RBCs in AIHA. Four patients were studied. Three with AIHA refractory to splenectomy and the use of moderate doses of glucocorticoids achieved hematologic and clinical remissions. Administration of steroids was either discontinued or reduced to minimal doses after treatment. Remissions lasted three years in two patients and one year in the other. Responses were characterized by prompt prolongation of RBC survival, correction of anemia, and gradual decrease in cell-bound antibody. In one nonsplenectomized patient, the disorder did not respond. Platelet-Vinca alkaloid complex is useful in the treatment of AIHA refractory to splenectomy and use of steroids.- - - - - - - - - - ranking = 0.14285714285714keywords = survival (Clic here for more details about this article) |
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