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Background: WBC reduction of all blood components is being introduced in many countries. Prevention of immunologic side effects of transfusions is part of the motivation. To compare the immunogenicity of before- or after-storage WBC-reduced RBCs with RBCs without buffy coat, a randomized clinical trial was performed.Study Design and Methods: Cardiac surgery patients were randomly assigned to receive either RBCs without buffy coat (PCs), WBC-reduced RBCs that were filtered before storage (FFs), or WBC-reduced RBCs that were filtered after storage (SFs). Serum samples for antibody analyses were collected before and after surgery.Results: Sera of 404 patients were tested. Of the 317 patients with negative preoperative screening, 12.6 percent developed anti-WBC antibodies (PC, 14.5%; FF, 9.6%; SF, 13.3%). Of the 87 patients with preoperative anti-WBC antibodies, 28.7 percent showed a marked increase in panel reactivity (PC, 31.3%; FF, 29.0%; SF, 25.0%). ELISA showed the newly formed antibodies to be of IgG class and directed against HLA class I in more than 90 percent of the samples tested. Newly formed anti-RBC antibodies appeared in 5.3 percent (PC, 7.1%; FF, 3.4%; SF 5.4%). Alloimmunization against WBCs and RBCs was strongly correlated (p < 0.01). The differences in newly formed anti-WBC antibodies and anti-RBC antibodies between the trial arms did not show significance.Conclusion: Buffy coat removal, and additional WBC reduction by filtration, either before or after storage, result in similar posttransfusion alloimmunization frequencies after a single transfusion event with multiple RBCs. [ABSTRACT FROM AUTHOR]

Summary. A survey was carried out among the European Group for Blood and Marrow Transplantation (EBMT) centres to determine the incidence, risk factors, treatment and outcome of thrombotic thrombocytopenic purpura (TTP) following allogeneic haematopoietic stem cell transplantation. TTP was defined as the simultaneous occurrence of red cell fragmentation, laboratory findings of haemolysis, red cell transfusion requirement and de novo or persistant thrombocytopenia caused by consumption, in the absence of disseminated intravascular coagulation. Forty-five centres reported all patients (n = 406) transplanted between July and December 1996. Twenty-three patients developed TTP; the risk of developing TTP was 6·7% at 2 years (95% CI: 4·1% to 9·3%). The median time of onset was 44 d (range 13–319) post transplantation. Significant risk factors for the development of TTP were female gender (P = 0·005) and an unrelated donor (P = 0·046). To treat TTP, cyclosporin administration was discontinued in 10 cases, plasma exchanges were performed in five cases and 12 patients received plasma infusions without plasma exchange. TTP resolved in 13 of the 23 patients (57%). The only factor predictive of resolution of TTP was the absence of nephropathy. Seven patients (30%) were alive at follow-up of 38–45 months from the onset of TTP. Sixteen patients died; the causes were multiple, only three patients had TTP as a central factor. The median time to death was 41 d (range 1–762 d) from the onset of TTP. TTP is a relatively frequent complication of allogeneic stem cell transplantation and it is associated with high mortality, though death is usually caused by multiple factors. [ABSTRACT FROM AUTHOR]

Frequencies of human leucocyte antigens (HLA)-A, -B and -DR were determined in 751 patients with chronic myelogenous leukaemia (CML) reported to the European Group for Blood and Marrow Transplantation after bone marrow transplantation from HLA-identical family donors and related to the occurrence of graft-versus-host disease (GVHD). HLA-A3 and DR1 were significantly associated with acute GVHD, the first with a higher risk (44% in HLA-A3+ versus 34% in HLA-A3- patients) and the latter with a lower risk (28% in HLA-DR1+ versus 38% in HLA-DR1- patients) for developing acute GVHD grade II–IV. Both factors were independent of known variables for GVHD as shown in a multivariate analysis. The results show that MHC alleles independently influence the incidence of GVHD in bone marrow transplantation from an HLA-identical donor for first chronic-phase CML. Possible mechanisms might include an HLA antigen-specific allele-associated effect, and/or non-specific allele-associated immune hypo- or hyper-responsiveness. [ABSTRACT FROM AUTHOR]

Allogeneic stem cell transplantation from an HLA-identical sibling donor is a curative treatment option for a young patient with myelodysplastic syndrome, limited by age and lack of sibling donors. Alternative stem cell sources have been used more recently, such as unrelated donors, non-identical family members or autologous transplants. This analysis of 1378 transplants reported to the European Group for Blood and Marrow Transplantation (EBMT) addresses the outcome of the varying procedures according to the known risk factors. The estimated disease-free survival (DFS) and estimated relapse risk at 3 years were both 36% for 885 patients transplanted with stem cells from matched siblings. In the multivariate analysis, age and stage of disease had independent prognostic significance for DFS, survival and treatment-related mortality. Patients transplanted at an early stage of disease had a significantly lower risk of relapse than patients transplanted at more advanced stages. The estimated DFS at 3 years was 25% for the 198 patients with voluntary unrelated donors, 28% for the 91 patients with alternative family donors and 33% for the 126 patients autografted in first complete remission. The non-relapse mortality was 58% for patients with unrelated donors, 66% for patients with non-identical family donors and 25% for autografted patients. The relapse rate of 18% was relatively low for patients with non-identical family donors, 41% for patients with unrelated donors and 55% for patients treated with autologous stem cell transplantation. Both allogeneic and autologous stem cell transplantation have emerged as treatment options for patients with myelodysplastic syndromes. Transplantation with an HLA-identical sibling donor is the preferred treatment option. Patients without an HLA-identical sibling donor may be treated with either autologous stem cell transplantation or an alternative donor transplantation. Patients younger than 20 years may be treated with an unrelated donor transplantation. Patients older than 40 years, and probably also patients between 20 and 40 years, may benefit most from an autologous stem cell transplantation. [ABSTRACT FROM AUTHOR]

The objective of this study was to present the long-term results of total adrenalectomy for Cushing's disease. Forty-four patients undergoing total adrenalectomy for Cushing's disease between 1953 and 1989 at Leiden University Medical Center, The Netherlands, were studied retrospectively. Remission was achieved in 42 patients (95%), with a mean duration of 19 years. Adrenal remnants were observed in 12 patients (27%), and were without clinical consequence in the majority of patients, but caused early recurrent disease in 2 patients. Nine patients (20%) experienced Addisonian crises up to 30 years following treatment. Nelson's syndrome developed in 10 patients (23%) 7–24 years following total adrenalectomy. Prior pituitary irradiation was a protective factor against Nelson's syndrome as it delayed its onset ( p= 0.025). On the other hand, subnormal dose or noncontinuous glucocorticoid replacement therapy was associated with increased risk of development of Nelson's syndrome ( p= 0.047). The incidence of Nelson's syndrome increased with prolonged follow-up, and female patients seemed to be at increased risk. Quality-of-life assessment showed less favorable scores on mental health and health perception scales, for which no explanation can be found except the long-lasting metabolic effects of Cushing's disease, even when successfully treated. In conclusion, total adrenalectomy remains the final treatment for Cushing's disease. The presence of adrenal remnants which can cause recurrent disease and the development of Nelson's syndrome during prolonged follow-up enhance the need for continued regular follow-up. Pituitary irradiation prior to total adrenalectomy delays the onset of Nelson's syndrome. [ABSTRACT FROM AUTHOR]

Examines the effects of atracurium on anemic fetus during and after intravascular intrauterine transfusion (IUT). Manifestation of fetal movements; Absence of reduction in fetal heart rate variability; Importance of atracurium in fetal paralysis during IUT.

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