Post-transfusion Haemoglobin Levels Research Articles

Life Threatening Hyperhemolysis in Two Children with Beta-Thalassemia Major (β-TM) Treated with Stem Cell Transplantation (SCT)

Hyperhaemolysis is a rare, poorly understood complication of red cell transfusion. We report the outcomes of SCT in 2 boys of Middle Eastern origin who developed life-threatening hyperhaemolysis each following their third transfusion for β-TM at the ages of 2.5 and 4 years. The diagnosis of hyperhaemolysis was based on laboratory evidence for haemolysis, post-transfusion haemoglobin (Hb) levels lower than pre-transfusion, positive Coomb’s tests and no allo-antibody able to be identified. Haemolysis was intravascular, extravascular and severe. Precipitous drops in Hb made transfusion unavoidable. The first boy responded to prednisolone, intravenous immunoglobulin (IVIG) and splenectomy. Haemosidderosis and fibrosis were present on liver biopsy, he was therefore regarded as a Class 3 thalassaemia patient and received hydroxyurea, azathioprine, erythropoietin, desferrioxamine, busulfan, cyclophosphamide and fludarabine conditioning prior to an HLA identical sibling SCT. He is alive and well 7 years post BMT with 30% stable donor chimerism and a normal Hb. The second child’s hyperhaemolysis failed to respond to prednisolone, IVIG, rituximab and splenectomy. Provision of the large number of suitably matched red cell units required was problematic. After receiving hydroxyurea, azathioprine, desferrioxamine, busulfan, cyclophosphamide, fludarabine, thiotepa and antithymocyte globulin (ATG) preparation for a CD3/CD19 depleted maternal haplotype peripheral blood SCT, the haemolysis finally stopped. Post-transplant severe veno-occlusive disease and multi-organ failure (MOF) required dialysis and ventilation. The maternal graft was rejected so 28 days after the first transplant he received a 4/6 mismatched unrelated cord blood transplant (UCBT) following further fludarabine and ATG, which fully engrafted. He recovered from MOF and was discharged from hospital 47 days after the UCBT, transfusion independent. On day +86 he contracted Respiratory Syncytial Virus chest infection with acute intravascular haemolysis necessitating transfusions. Fulminant liver failure developed, presumably due to iron toxicity, and death occurred on day +102, having received 112 transfusions in the 12 months since presentation. In conclusion, avoiding red cell transfusion is not always possible in hyperhaemolysis, especially in β-TM. Patients may quickly become classified as Class 3 in terms of predicting BMT outcome. Immune modulation therapy and SCT was effective in 1 case but only temporarily stopped haemolysis in the other, despite full engraftment ultimately being achieved with a mismatched UCBT. SCT should be considered early in cases of hyperhaemolysis in β-TM because it can potentially cure both and result in transfusion independence. DisclosuresOff Label Use: Intravenous immunoglobulin and rituximab for treatment of haemolytic anaemia; hydroxyurea, azathioprine, fludarabine, erythropoeitin, busulphan, cyclophosphamide, thiotepa and antithymocyte globulin for use in stem cell transplantation in children with thalassaemia.

Potentially avoidable blood transfusion during trauma resuscitation

IntroductionRed blood cell (RBC) transfusion is often essential during trauma resuscitation but is associated with high cost and potential adverse outcomes. This study aimed to determine the incidence of potentially avoidable RBC transfusions (PAT) among adult major trauma patients. Materials and methodsA retrospective review of data collected by <name blinded> Registry on patients presenting between Jan 2006 and Dec 2011 was conducted. Eligible patients received at least 1 unit of RBC in the first 24h following presentation to the Emergency Department (ED). Episodes of PAT were determined according to haemodynamic stability and post-transfusion haemoglobin levels. ResultsThere were 621 patients included, of whom 224 (36.1%; 95% CI: 32.3–40.0) received PAT. Of them, 132 (58.9%) were haemodynamically stable on arrival and did not require a surgical procedure. Patients with PAT had significantly lower injury severity scores (30 vs 34, p<0.01), higher presenting systolic blood pressure (129 vs 112mm Hg, p<0.01) and a lower frequency of a shock index ≥1 (24.1 vs 65.0%, p<0.01), compared to those without PAT. They also had a significantly lower mortality (13.4 vs 21.7%, p<0.01). ConclusionsPAT after trauma was common and often delivered to haemodynamically stable patients who did not require surgical procedures. Clinical decision pathways for trauma resuscitation should aim to limit PAT.

Hyperhaemolysis syndrome responsive to splenectomy in a patient with δβ-thalassaemia: a discussion on underlying mechanisms.

Hyperhaemolysis syndrome responsive to splenectomy in a patient with δβ-thalassaemia: a discussion on underlying mechanisms.

Hyperhemolytic Transfusion Reactions in Non-Sickle Cell Anemia Patients

AbstractAbstract 1183 IntroductionHyperhemolytic transfusion reaction syndrome (HHTR) has been appreciated in allo-immunized patients with sickle cell anemia. In these patients, transfusion precipitates hemolysis of both donor RBCs and the recipient's native RBCs, leading to post-transfusion hemoglobin level below baseline. Recognition is important because it is life-threatening and has unique management. Despite the severe degree of anemia, the most important treatment for HHTR is generally to halt the cascade of hemolysis by withholding transfusion. We present the first case series of HHTR in non-sickle cell patients observed in a tertiary academic medical center. MethodsWe reviewed records of four patients treated by one of us. We also retrospectively search for any additional patient charts from those with diagnosis of HHTR from 1996 to 2012 from institutional database and also searched patients under ICD-9 codes for hemolytic transfusion reactions (999.83 to 85) from 2009 to 2011. Patients with sickle cell disease were excluded. HHTR was defined as post-transfusion hemoglobin level below baseline accompanied by clinical and laboratory evidences of hemolysis, and the recurrence of HHTR after repeated transfusions. ResultsFour non-sickle cell patients who fulfilled criteria of HHTR were identified. Mean age was 34.7. Three were African-Americans, one was Caucasian, and all were females. Two patients had iron deficiency anemia, one had systemic lupus erythematosus, and one had no past medical history. The average amount of transfused PRBC was 5.83±0.3units per person. After transfusion, hemoglobin reached nadir (mean decrease from baseline 38%) within two to three weeks of initial transfusion. Corrected reticulocyte counts were typically low (<2%). All had symptomatic hemolysis often accompanied by chills, jaundice, elevated LDH and unconjugated bilirubin. Post-transfusion analysis of allo-immunization revealed three patients with de novo development of multiple allo-antibodies. The most common alloantibodies were anti-C, c and E. Various therapies including IV steroid, IVIg and erythropoietin were applied, but the most effective management was withholding further transfusion. One patient with consecutive transfusions expired after severe hemolysis. Three patients survived from HHTR and hemoglobin recovered to 72% of their baselines. ConclusionWe have demonstrated the first case series of HHTR in non-sickle cell patients and defined its clinical characteristics. HHTR in non-sickle cell patients results in 38% decline of hemoglobin within two to three weeks of transfusion. De novo development of allo-antibodies strongly suggests that the exaggerated destruction of self and non-self cells may be secondary to acute induction of allo-immunity against non-self RBCs and subsequent complement activation leading to bystander hemolysis of self RBCs. HHTR is a unique and lethal complication of transfusion which occurs in both sickle and non-sickle cell patients. Withholding transfusion is a crucial strategy to HHTR. Thus, it is important not to reserve the diagnosis to sickle cell patients of African-American descent, but to recognize that this can occur in any transfused patient. [Display omitted] Disclosures:No relevant conflicts of interest to declare.

The physician compliance of red blood cell transfusion by computerized transfusion decision support system

“When should we trigger a transfusion?” is always a critical question between the patient’s benefits and risks in red blood cell (RBC) transfusion. A computerized transfusion decision support system (CTDSS) has been used since September 2004 in an academic medical center with 1400 beds. In this study, the factors affecting RBC transfusion were investigated. In total 20,551 RBC-transfusion episodes between January and December 2008 were reviewed. The nearest hemoglobin concentration before transfusion is defined as the transfusion trigger. The physician compliance, the factors associated with the transfusion triggers and posttransfusion hemoglobin increment were investigated. The physician compliance is 83.1%. The transfusion trigger is 8.32±1.84 (mean±standard deviation) g/dL. The transfusion triggers are statistically significant in terms of both different order sources and disease types (p<0.05).The posttransfusion hemoglobin level increased in two-thirds of the episodes. The percentages of hemoglobin increments after transfusion are dependent on the transfusion triggers. Appropriate transfusion practice may reduce the overuse of blood components and improve transfusion quality. CTDSS should be more powerful to intervene in the appropriateness of transfusion practice.

Renal Artery Injury During Robot-Assisted Renal Surgery

Laparoscopic partial nephrectomy (LPN) is becoming the standard of care for incidentally diagnosed, small renal tumors. With its seven degrees of freedom and three-dimensional vision, the DaVinci robotic surgical system has been used to assist in LPNs. The main disadvantage of robot-assisted surgery, however, is the lack of tactile feedback. We present a case of renal artery injury during robot-assisted renal surgery. Robot-assisted partial nephrectomy (RPN) was planned for 47-year-old man with a 3.5-cm right renal mass. After standard bowel mobilization, renal hilar dissection was performed. In the attempt to complete the dissection posteriorly, however, there was sudden profuse bleeding. The intraperitoneal pressure immediately increased to 20 mm Hg, and an additional suction device was inserted through the 5-mm liver retractor port. On inspection, there was an injury at the takeoff of the posterior segmental artery. A decision was made to convert to robot-assisted laparoscopic radical nephrectomy. The main renal artery and renal vein were controlled with Hem-o-Lok clips. The estimated blood loss was 2,000 mL. Four units of packed red blood cells were transfused intraoperatively. The post-transfusion hemoglobin level was 12.6 g/dL. There were no other perioperative complications. The surgeon should keep in mind that the robotic arms are very powerful and can easily injure major vessels because of lack of tactile feedback. A competent and experienced tableside surgeon is very important in robot-assisted surgery because the unsterile console surgeon cannot immediately react to intraoperative complications.

Predicting Change of Hemoglobin After Transfusion in Hemodynamically Stable Anemic Patients in Emergency Department

To investigate factors associated with change of post-transfusion hemoglobin level, and to derive an equation that predicts post-transfusion changes in hemoglobin levels in hemodynamically stable anemic patients who visited emergency department. A retrospective medical record review of patients who were hemodynamically stable and transfused with packed red blood cells was undertaken. Patients were randomly divided into two groups. One group (derivation group, 70% of total patients) was analyzed for factors associated with changes in post-transfusion hemoglobin levels, and linear regression analysis was performed to derive a prediction equation. The derived prediction equation was then externally validated with the other group (validation group, 30% of total patients). A total of 196 patients were enrolled. The 137 patients (70% of total patients) in the derivation group were analyzed for factors associated with changes in post-transfusion hemoglobin. Of those, body surface area and initial hemoglobin level were significantly correlated with changes in post-transfusion hemoglobin levels (p < 0.05). From these variables, linear regression analysis resulted in a prediction equation. The derived equation was validated externally with the 59 patients (30% of total patients) in the validation group and found to have an excellent correlation (r = 0.73, intraclass correlation = 0.84, p < 0.05). Post-transfusion hemoglobin level in hemodynamically stable adult patients was associated with initial hemoglobin levels and body surface area. These factors must be considered when transfusing hemodynamically stable adult patients with anemia.

Effect of Triphaladi Avaleha as an adjuvant therapy in the management of Thalassemia

Thalassemia is the most common inherited single-gene (autosomal recessive) disorder in the world. Scientists worldwide predict that thalassemia will become a considerable health issue in the next century. It is a new disease entity for Ayurvedic medicine, and hence, it is called Anukta Vyadhi in Ayurveda; but we can understand it by careful scrutiny of the clinical presentation and the investigation results described in the available literature. Modern medical management is aimed at maintaining the hemoglobin level at 10 – 12 g/dl. A post-transfusion hemoglobin level of 9.5 g/dl is said to be sufficient to maintain active life. Thus, blood transfusion therapy is the only treatment, but it can result in hemosiderosis (iron overload), a complication with a fatal outcome. The transfusional iron overload is compounded by increased intestinal absorption of iron. The most important factors associated with survival, and also those deciding the outcome of bone marrow transplant (the only curative therapy) are, age at which chelation therapy is introduced and the success with which serum ferritin is maintained below 2500 ng/ml. Iron chelators used in modern medicine to achieve this goal are expensive and associated with side effects, and hence, associated with poor adherence to the treatment. The present study is an endeavor to explore the efficacy of Triphaladi Avaleha as an iron chelator in the management of thalassemia, in comparison to a control group managed by routine modern therapy.

The utility of an automated electronic system to monitor and audit transfusion practice

Transfusion laboratories with transfusion committees have a responsibility to monitor transfusion practice and generate improvements in clinical decision-making and red cell usage. However, this can be problematic and expensive because data cannot be readily extracted from most laboratory information systems. To overcome this problem, we developed and introduced a system to electronically extract and collate extensive amounts of data from two laboratory information systems and to link it with ICD10 clinical codes in a new database using standard information technology. Three data files were generated from two laboratory information systems, ULTRA (version 3.2) and TM, using standard information technology scripts. These were patient pre- and post-transfusion haemoglobin, blood group and antibody screen, and cross match and transfusion data. These data together with ICD10 codes for surgical cases were imported into an MS ACCESS database and linked by means of a unique laboratory number. Queries were then run to extract the relevant information and processed in Microsoft Excel for graphical presentation. We assessed the utility of this data extraction system to audit transfusion practice in a 600-bed adult tertiary hospital over an 18-month period. A total of 52 MB of data were extracted from the two laboratory information systems for the 18-month period and together with 2.0 MB theatre ICD10 data enabled case-specific transfusion information to be generated. The audit evaluated 15,992 blood group and antibody screens, 25,344 cross-matched red cell units and 15,455 transfused red cell units. Data evaluated included cross-matched to transfusion ratios and pre- and post-transfusion haemoglobin levels for a range of clinical diagnoses. Data showed significant differences between clinical units and by ICD10 code. This method to electronically extract large amounts of data and linkage with clinical databases has provided a powerful and sustainable tool for monitoring transfusion practice. It has been successfully used to identify areas requiring education, training and clinical guidance and allows for comparison with national haemoglobin-based transfusion guidelines.

Effects Of Reinfusion Systems On Transfusion Needs After Total Knee Arthroplasty

Background: Total Joint arthroplasty can lead to significant blood loss. This loss often requires replacement with banked allogenic or pre-donated antologous blood. Both forms of replacement involve substantial financial cost and can be associated with significant complications to the patient. The purpose of this study was to evaluate the effect that reinfusion of postoperative drained blood has on the transfusion requirements following total knee arthroplasty(TKA) using the ConstaVac re-infusion system. Methods: A retrospective analysis of 91 consecutive TKA performed at one institution was carried out. 22 cases were excluded. The remaining 69 cases formed the basis of the study. The ConstaVac reinfusion system was used in 43 of these cases and a standard Bellovac drainage system was used in the remaining 26 cases. Data was collected on patient\'s age, side affected, operative duration, tourniquet use, volumes drained and reinfused, transfusion requirements and preand post-operative as well as post transfusion haemoglobin levels. Results: Transfusion of packed red cells was required in 50% of cases in which the standard drainage system was used compared with 19% of cases in which the re-infusion system was used. This result was statistically significant using chi-squared analysis. (p< 0.05) Conclusion: We recommend that a postoperative reinfusion system should be used to decrease the transfusion requirements following TKA.

The clinical management of thalassaemia

The clinical management of thalassaemia will be discussed in respect to the pathophysiology of the homozygous disease. Social and preventive aspects and management of thalassaemia trait are beyond the scope of this presentation. 1. The decision to transfuse is based on the overall assessment of the patient, taking into account the haemoglobin level at presentation, the age of presentation, growth and development, the general well-being of the child, the degree of marrow expansion present and the degree of gastrointestinal iron absorption. 2. Pre- and post-transfusion haemoglobin levels Haemoglobin should be maintained between a pre-transfusion level of between 9 and 10 g/dl and 14 to 15 g/dl post-transfusion. It is important to monitor the pre- and posttransfusion haemoglobin levels. 3. Investigations Clinical photography, serum ferritin levels, bone age, chest X-ray and skull X-ray should be undertaken regularly. 4. Splenectomy The assessment of the need for splenectomy is made from calculations of the transfusion requirement according to the mean haemoglobin level. Prior to splenectomy, clotting studies should be performed and pneumovax should be given. Prophylactic penicillin post-splenectomy may be given to reduce the incidence of beta-haemolytic streptococcal infection. 5. Iron chelation therapy After baseline endocrine, cardiac, hepatic and ophthalmological assessments are made, the patient is commenced on subcutaneous infusions of desferrioxamine for iron chelation therapy. Vitamin C is given to potentiate the effect of the desferrioxamine, but in small doses (100 mg/infusion), after the patient has been chelated for at least one to two mth. In the future we will be able to perform young red cell exchange transfusion on the cell separator, which will increase transfusion intervals and will reduce the iron load. Newer chelating agents will be discussed.