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Red Blood Cell Transfusions (red + blood_cell_transfusion)
Selected AbstractsSustained and full fetal hemoglobin production after failure of bone marrow transplant in a patient homozygous for beta 0-thalassemia: A clinical remission despite genetic disease and transplant rejection,AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2009Katia Paciaroni An adult patient affected by ,0 -thalassemia major underwent allogeneic bone marrow transplant (BMT) from a matched related donor. Forty days after transplant, allogeneic engraftment failure and autologous ,0 -thalassemic bone marrow recovery were documented. Red blood cell transfusions were required until 118 days post-transplant. Thereafter, the haemoglobin (Hb) levels stabilized over 11.8 gr/dl throughout the ongoing 34-month follow-up, abolishing the need for transfusion support. The Hb electrophoresis showed 100% Hb Fetal (HbF). This unexplained case suggests full HbF production may occur in an adult patient with ,0 -thalassemia major. Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc. [source] Red blood cell transfusions and iron overload in the treatment of patients with myelodysplastic syndromesCANCER, Issue 5 2008Elias Jabbour MD Abstract BACKGROUND Approximately 15,000 new cases of myelodysplastic syndromes (MDS) are expected in the United States each year. METHODS The mainstay for the management of myelodysplastic syndromes (MDS) is supportive therapy with red blood cell (RBC) transfusions to improve the patient's quality of life. RBC transfusions enable adequate tissue oxygenation and increase hemoglobin levels, improve fatigue, and improve the physical and intellectual activity of patients. Up to 90% of patients with MDS will receive RBC transfusions during the course of their disease, and many will become chronically dependent on transfusions to manage their anemia. These transfusions lead to an accumulation of excess iron that, in turn, can develop into a condition known as iron overload, causing clinical consequences like hypertransaminasemia and cirrhosis, dilated cardiomyopathy, and progressive dysfunction of the endocrine glands. RESULTS Studies in patients with MDS have indicated that iron overload because of RBC transfusions was an independent, adverse prognostic factor for overall survival (OS) and leukemia-free survival (LFS): OS and LFS were significantly shorter in transfusion-dependent patients with MDS than in those who were not transfusion dependent. CONCLUSIONS Although the National Comprehensive Cancer Network guidelines for the treatment of patients with MDS recommend the use of RBC transfusions as supportive care, they further recommend that the iron burden of transfused patients be monitored regularly and that iron chelation therapy be considered to maintain serum ferritin levels of <1000 ng/mL. Cancer 2008. © 2008 American Cancer Society. [source] Outcome of intensive care of homozygous alpha-thalassaemia without prior intra-uterine therapyJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 7-8 2007Shing YR Lee Aim: To review the outcome of homozygous alpha-thalassaemia without prior intra-uterine therapy treated in neonatal intensive care unit and identify the factors associated with survival. Methods: The hospital records of all patients with homozygous alpha-thalassaemia treated in our neonatal intensive care unit in the last 15 years were reviewed. A literature search beginning in the year 1980 was done to identify homozygous alpha-thalassaemia actively treated in neonatal intensive care units. Those receiving prior intra-uterine therapy were excluded. The following information was collected: the severity of hydrops, sizes of liver and spleen, haemoglobin level, Apgar score at 5 min, ventilator settings, timing and forms of red blood cell transfusion and presence of persistent hypoxaemia. The survivors and the non-survivors were compared. Results: In our centre, in the last 15 years there were six infants born with homozygous alpha-thalassaemia who did not receive intra-uterine therapy; one survived and five succumbed despite aggressive respiratory therapy. In our literature search there were more reports of survivors (10) than non-survivors (six) for these infants, suggesting a reporting bias towards selection of rare cases of survival. Apgar score of four or above occurred in seven of the eight survivors with data available in the reports, whereas this occurred in four of the 11 non-survivors (P = 0.035, Fisher Exact test). Five of the 11 survivors had abnormal neurological outcome including developmental delay and spastic quadriplegia. Conclusion: Without prior intra-uterine therapy, homozygous alpha-thalassaemia has grave outlook in terms of mortality and morbidity despite aggressive respiratory therapy. [source] Transfusion in premature infants impairs production and/or release of red blood cells, white blood cells and plateletsJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 3 2002B Frey Objective: To examine whether red blood cell transfusion in infants with anaemia of prematurity alters peripheral counts of red blood cell precursors, total white blood cells and white cell differential and platelets. Methodology: In 18 consecutive stable premature infants with anaemia of prematurity, peripheral cell counts were prospectively recorded immediately before transfusion of 20 mL/kg packed red blood cells (given over 6 h), and at 48 h after completion of the transfusion. Results: The median (interquartile range) haematocrit increased from 22.0% (21.3,24.0%) pre-transfusion to 37.0% (36.0,38.0%) post-transfusion (P < 0.001). Red-cell precursors decreased: median (interquartile range) reticulocytes from 3.7% (3.0,7.7%) to 3.7% (2.6,4.1%) (P = 0.03); and median (interquartile range) nucleated red blood cells from 0 G/L (0,0.2 G/L) to 0 G/L (0,0 G/L) (P = 0.03). The mean (SD) platelet count decreased from 420 G/L (154 G/L) to 313 G/L (101 G/L) (P = 0.001). The total white blood cell count and neutrophils did not change significantly; however, median (interquartile range) immature neutrophils decreased from 0.12 G/L (0.06,0.74 G/L) to 0.08 G/L (0.01,0.24 G/L) (P = 0.03). Lymphocytes, eosinophils, basophils and plasma cells remained unchanged. Monocytes increased (P = 0.01). Conclusions: Forty-eight hours after red blood cell transfusion to premature infants, there is an absolute decrease in red blood cell precursors, immature white blood cells and platelets, probably due to erythropoietin-suppression. [source] Bone marrow transplantation for ,-thalassaemia major by an HLA-mismatched parentJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 3 2002CF Li Abstract: A six-year-old boy was diagnosed with ,-thalassaemia major during infancy. Since then, he required monthly blood transfusion and irregular iron chelation therapy. He had hepatosplenomegaly and elevated liver enzymes; the serum ferritin was up to 3800 ng/mL. An echocardiogram showed left-ventricular enlargement. His one-antigen-mismatched mother was chosen as a bone marrow donor. He was pretreated with intensive red blood cell transfusion and hydroxyurea for 6 weeks prior to conditioning. The conditioning included total body irradiation (300 cGy), busulfan (14 mg/kg), cyclophosphamide (160 mg/kg) and anti-thymocyte globulin (rabbit; 90 mg/kg). Marrow cell dose was 5.4 × 108/kg. Graft versus host disease (GVHD) prophylaxis included cyclosporine A (CSA) and methylprednisolone. Neutrophil engraftment occurred on day 23. Grade II acute GVHD occurred on day 45. The patient developed complications including septicaemia, haemorrhagic cystitis, intracranial haemorrhage and heart failure. He subsequently recovered from the complications without sequelae. The patient remained transfusion-independent at a follow-up examination after 18 months. This case suggested that a mismatched family member may be considered as a bone marrow donor for ,-thalassaemia major. In places where conventional treatment is not feasible, for example, in China, this approach may be an alternative option. A more intensive immunosuppressive regimen and a higher marrow cell dose may be important for successful engraftment. High-dose anti-thymocyte globulin may also prevent severe GVHD. [source] Outcomes following early red blood cell transfusion in acute upper gastrointestinal bleedingALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 2 2010S. A. Hearnshaw Aliment Pharmacol Ther 2010; 32: 215,224 Summary Background, Acute upper gastrointestinal bleeding (AUGIB) accounts for 14% of RBC units transfused in the UK. In exsanguinating AUGIB the value of RBC transfusion is self evident, but in less severe bleeding its value is less obvious. Aim, To examine the relationship between early RBC transfusion, re-bleeding and mortality following AUGIB, which is one of the most common indications for red blood cell (RBC) transfusion. Method, Data were collected on 4441 AUGIB patients presenting to UK hospitals. The relationship between early RBC transfusion, re-bleeding and death was examined using logistic regression. Results, 44% were transfused RBCs within 12 hours of admission. In patients transfused with an initial haemoglobin of <8 g/dl, re-bleeding occurred in 23% and mortality was 13% compared with a re-bleeding rate of 15%, and mortality of 13% in those not transfused. In patients transfused with haemoglobin >8 g/dl, re-bleeding occurred in 24% and mortality was 11% compared with a re-bleeding rate of 6.7%, and mortality of 4.3% in those not transfused. After adjusting for Rockall score and initial haemoglobin, early transfusion was associated with a two-fold increased risk of re-bleeding (Odds ratio 2.26, 95% CI 1.76,2.90) and a 28% increase in mortality (Odds ratio 1.28, 95% CI 0.94,1.74). Conclusions, Early RBC transfusion in AUGIB was associated with a two-fold increased risk of re-bleeding and an increase in mortality, although the latter was not statistically significant. Although these findings could be due to residual confounding, they indicate that a randomized comparison of restrictive and liberal transfusion policies in AUGIB is urgently required. [source] Controversies related to red blood cell transfusion in critically ill patientsJOURNAL OF VETERINARY EMERGENCY AND CRITICAL CARE, Issue 2 2010DACVECC, DACVIM, Jennifer E. Prittie DVM Abstract Objective , To review the evolution of and controversies associated with allogenic blood transfusion in critically ill patients. Data sources , Veterinary and human literature review. Human Data Synthesis , RBC transfusion practices for ICU patients have come under scrutiny in the last 2 decades. Human trials have demonstrated relative tolerance to severe, euvolemic anemia and a significant outcome advantage following implementation of more restricted transfusion therapy. Investigators question the ability of RBCs stored longer than 2 weeks to improve tissue oxygenation, and theorize that both age and proinflammatory or immunomodulating effects of transfused cells may limit efficacy and contribute to increased patient morbidity and mortality. Also controversial is the ability of pre- and post-storage leukoreduction of RBCs to mitigate adverse transfusion-related events. Veterinary Data Synthesis , While there are several studies evaluating the transfusion trigger, the RBC storage lesion and transfusion-related immunomodulation in experimental animal models, there is little research pertaining to clinical veterinary patients. Conclusions , RBC transfusion is unequivocally indicated for treatment of anemic hypoxia. However, critical hemoglobin or Hct below which all critically ill patients require transfusion has not been established and there are inherent risks associated with allogenic blood transfusion. Clinical trials designed to evaluate the effects of RBC age and leukoreduction on veterinary patient outcome are warranted. Implementation of evidence-based transfusion guidelines and consideration of alternatives to allogenic blood transfusion are advisable. [source] Human prion diseases: biology and transmission by bloodISBT SCIENCE SERIES: THE INTERNATIONAL JOURNAL OF INTRACELLULAR TRANSPORT, Issue 1 2006J. W. Ironside Human prion diseases are a group of rare fatal transmissible neurodegenerative disorders that occur in sporadic, acquired and familial forms. In 1996, a new type of human prion disease, variant Creutzfeldt-Jakob disease (vCJD), was first identified and has subsequently been identified in 10 additional countries. vCJD results from human exposure to the bovine spongiform encephalopathy (BSE) agent, most likely through the consumption of BSE-contaminated meat products. Unlike other human prion diseases, both infectivity and the disease-associated form of the prion protein are readily detected in lymphoid tissues in vCJD, suggesting that infectivity may also be present in blood. Three recent cases of apparent iatrogenic vCJD infection by blood transfusion have occurred in the UK, following red blood cell transfusions from asymptomatic donors who subsequently died from vCJD. The first and third cases resulted in a clinical illness identical to vCJD, while the second case was an asymptomatic infection only detected at autopsy. There are no current means of detecting vCJD infection in asymptomatic donors, so continuing surveillance is required in the UK and other countries to monitor the incidence of vCJD and further cases of secondary transmission by blood transfusion. [source] Association between central venous pressure and blood loss during hepatic resection in 984 living donorsACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 5 2009Y. K. KIM Background: Although low central venous pressure (CVP) anesthesia has been used to minimize blood loss during hepatectomy, the efficacy of this technique remains controversial. We therefore assessed the association between blood loss and CVP during hepatic resection, and examined significant determinants associated with intraoperative hemorrhage during hepatectomy in living donors. Methods: Between April 2004 and April 2008, 984 living donors who underwent a hepatic resection were assessed retrospectively. Univariate and multivariate analyses were performed to explore the relationships between intraoperative blood loss and several variables including CVP. Results: The mean intraoperative blood loss was 691.3 ± 365.5 ml. Only four donors required packed red blood cell transfusions (mean, 1.5 U). The mean duration of hepatic resection was 92.1 ± 26.3 min. The mean, maximum, and minimum values of CVP measured during hepatectomy were 4.6 ± 1.7, 5.3 ± 1.8, and 4.0 ± 1.8 mmHg, respectively, and were not significantly correlated with intraoperative blood loss. On multivariate analysis, predictors of hemorrhage were liver fatty change, gender, and body weight, but none of the mean CVP, surgeons, anesthesiologists, anesthesia duration, resected liver volume, hepatectomy type, systolic blood pressure, heart rate, or body temperature were significant. Conclusions: CVP during hepatic resection was not associated with intraoperative blood loss in living liver donors, suggesting that CVP may not be an important factor in predicting blood loss during hepatectomy in healthy subjects. [source] Microarray analysis of liver gene expression in iron overloaded patients with sickle cell anemia and beta-thalassemia,AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2009Jonathan M. Flanagan Chronic transfusion therapy is used clinically to supply healthy erythrocytes for patients with sickle cell anemia (SCA) or beta-thalassemia major (TM). Despite the benefits of red blood cell transfusions, chronic transfusions lead to iron accumulation in key tissues such as the heart, liver, and endocrine glands. Transfusion-acquired iron overload is recognized as a cause of morbidity and mortality among patients receiving chronic transfusions. At present, there is little understanding of molecular events that occur during transfusional iron loading and the reasons for the large inter-individual variation observed clinically in transfusion-acquired iron accumulation. To address these issues, we examined whether any liver-expressed genes in SCA or TM patients with transfusional iron overload were associated with the degree of iron accumulation. Specifically, we performed microarray analysis on liver biopsy specimens comparing SCA patients with mild or severe iron overload and also compared SCA with TM patients. Fifteen candidate genes were identified with significantly differential expression between the high and low liver iron concentrations. SCA patients and 20 candidate genes were detected between the SCA and TM patient comparison. Subsequent quantitative PCR experiments validated 12 candidate genes; with GSTM1, eIF5a, SULF2, NTS, and HO-1 being particularly good prospects as genes that might affect the degree of iron accumulation. Future work will determine the baseline expression of these genes prior to transfusional iron overload and elucidate the full impact of these genes on the inter-individual variation observed clinically in transfusion-acquired iron accumulation. Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc. [source] Successful treatment of pure red cell aplasia with autologous stem cell transplantationAMERICAN JOURNAL OF HEMATOLOGY, Issue 9 2007Jae H. Park We report a case of 64-year-old patient with pure red cell aplasia (PRCA) who was intolerant of conventional immunosuppressive therapies but achieved a complete long-term remission following autologous hematologic stem cell transplant (HSCT). The patient was initially treated with high-dose prednisone, cyclophosphamide, cyclosporine, antithymocyte globulin, and then rituximab. With the exception of rituximab, all of the above regimens achieved a transient response. However, because of the persistent requirement for red blood cell transfusions and intolerance to the multiple immunosuppressive therapies, autologous HSCT was eventually performed. The patient remains in complete remission and on no other therapy for 36 months following the autologous HSCT. Am. J. Hematol., 2007. © 2007 Wiley-Liss, Inc. [source] Extracorporeal membrane oxygenation as a rescue therapy for leukaemic children with pulmonary failureBRITISH JOURNAL OF HAEMATOLOGY, Issue 1 2010Bernhard Meister Summary In patients with leukaemia, acute respiratory distress syndrome (ARDS) secondary to intensified chemotherapy-induced immunosuppression is a devastating disorder resulting in high morbidity and mortality. Compared to standard indications for extracorporeal membrane oxygenation (ECMO), cytopenia further increases the risks of infection and bleeding. We describe the use of ECMO in four children with ARDS and leukaemia. Two patients (50%) survived, pulmonary function recovered and they are in prolonged first remission. The two other patients died from ARDS and pulmonary leukaemic infiltration. Although ECMO support is a high-risk setup for nosocomial infection we observed no additional septic episodes. All patients had a highly increased demand for packed platelet and red blood cell transfusions. This increased demand and unmanageable chronic bleeding into both lungs in one patient were probably caused by a combination of coagulopathy from the primary illness, the use of anticoagulants, chemotherapy-induced cytopenia, and a reduced survival rate of platelets and red cells due to permanent contact to foreign surface. We concluded that ECMO is a supportive tool to reduce the incidence of early death, treatment-related mortality and, ultimately, to improve overall survival in childhood leukaemia. [source] Matched-related donor transplantation for sickle cell disease: report from the Center for International Blood and Transplant ResearchBRITISH JOURNAL OF HAEMATOLOGY, Issue 5 2007Julie A. Panepinto Summary We report outcomes after myeloablative haematopoietic cell transplantation (HCT) from human leucocyte antigen (HLA)-matched sibling donors in 67 patients with sickle cell disease transplanted between 1989 and 2002. The most common indications for transplantation were stroke and recurrent vaso-occlusive crisis in 38% and 37% of patients respectively. The median age at transplantation was 10 years and 67% of patients had received >10 red blood cell transfusions before HCT. Twenty-seven percent of patients had a poor performance score at transplantation. Ninety-four percent received busulfan and cyclophosphamide-containing conditioning regimens and bone marrow was the predominant source of donor cells. Most patients achieved haematopoietic recovery and no deaths occurred during the early post-transplant period. Rates of acute and chronic graft-versus-host disease were 10% and 22% respectively. Sixty-four of 67 patients are alive with 5-year probabilities of disease-free and overall survival of 85% and 97% respectively. Nine patients had graft failure with recovery of sickle erythropoiesis, eight of who had recurrent sickle-related events. This report confirms and extends earlier reports that HCT from HLA-matched related donors offers a very high survival rate, with few transplant-related complications and the elimination of sickle-related complications in the majority of patients who undergo this therapy. [source] Clinical manifestations of infants with nutritional vitamin B12 deficiency due to maternal dietary deficiencyACTA PAEDIATRICA, Issue 1 2009E Zengin Abstract Aim: In developing countries, nutritional vitamin B12 deficiency in infants due to maternal diet without adequate protein of animal origin has some characteristic clinical features. In this study, haematological, neurological and gastrointestinal characteristics of nutritional vitamin B12 deficiency are presented. Methods: Hospital records of 27 infants diagnosed in a paediatric haematology unit between 2000 and 2008 were evaluated retrospectively. Results: The median age at diagnosis was 10.5 months (3,24 months). All the infants were exclusively breast fed and they presented with severe nonspecific manifestations, such as weakness, failure to thrive, refusal to wean, vomiting, developmental delay, irritability and tremor in addition to megaloblastic anaemia. The diagnosis was confirmed by complete blood counts, blood and marrow smears and serum vitamin B12 and folic acid levels. The median haemoglobin level was 6.4 g/dL (3.1,10.6) and mean corpuscular volume (MCV) was 96.8 fL (73,112.3). Some patients also had thrombocytopaenia and neutropaenia. All the infants showed clinical and haematological improvement with vitamin B12 administration. Patients with severe anaemia causing heart failure received packed red blood cell transfusions as the initial therapy. Conclusion: Paediatricians must consider nutritional vitamin B12 deficiency due to maternal dietary deficiency in the differential diagnosis of some gastrointestinal, haematological, developmental and neurological disorders of infants with poor socioeconomic status. Delay in diagnosis may cause irreversible neurological damage. [source] | ||||||||||||||||||||||