Since the introduction of prophylaxis, physicians have tried to convert the clinical phenotype of severe haemophilia (SH) into that of moderate haemophilia (MH), but the outcome of patients with SH has never been compared to that of patients with MH.
Material and methods
The outcome of 80 patients with SH on long-term, intermediate dose prophylaxis was compared to that of 40 patients with MH in a single-centre study. Data on treatment history, activities (assessed by the IPAQ and HAL), quality of life (assessed by the SF-36 and EQ5D), and 5-year bleeding and clotting factor consumption were collected for patients born between 1970–1995.
The median age of the patients was 24 years (IQR 18–30). All patients with SH received long-term prophylaxis, which was started at a median age of 4.8 years (IQR 3.2–6.2). Among the patients with MH, ten (25%) received prophylaxis, starting at a median age of 10.8 years (IQR 3.8–13.8). The annual number of bleeds, including joint bleeds, was significantly higher in patients with SH (median 2.0 joint bleeds/year, IQR =0.8–3.7) than in patients with MH (median 0.8 joint bleeds/year, IQR =0–1.2). Due to greater use of prophylaxis, the annual clotting factor consumption of SH patients (median 2,120 IU/kg; IQR 1,514–2,768), was higher than that of MH patients (median 133 IU/kg; IQR 49–468). Patients with SH showed slightly but significantly more loss of clinical function (assessed by the Haemophilia Joint Health Score): a median of 8 points (IQR 3–15) vs a median of 2 points, IQR 0–6). Quality of life, as measured by the SF-36, EQ5D and physical activity, was similar between patients with disease of different severity, as well as compared to that of the general population.
When comparing unselected cohorts, the bleeding pattern of patients with SH does not appear to be fully converted to that of the milder bleeding pattern of MH by long-term, intermediate-dose prophylaxis, although activities and quality of life were similar.
moderate haemophilia; severe haemophilia; comparison; joint bleeding; HJHS
Blood loss during total joint arthroplasty strongly influences the time to recover after surgery and the quality of the recovery. Blood conservation strategies such as pre-operative autologous blood donation and post-operative cell salvage are intended to avoid allogeneic blood transfusions and their associated risks. Although widely investigated, the real effectiveness of these alternative transfusion practices remains controversial.
Materials and methods
The surgery reports of 600 patients undergoing total joint arthroplasty (312 hip and 288 knee replacements) were retrospectively reviewed to assess transfusion needs and related blood management at our institute. Evaluation parameters included post-operative blood loss, haemoglobin concentration measured at different time points, ASA score, and blood transfusion strategies.
Autologous blood donation increased the odds of receiving a red blood cell transfusion. Reinfusion by a cell salvage system of post-operative shed blood was found to limit adverse effects in cases of severe post-operative blood loss. The peri-operative net decrease in haemoglobin concentration was higher in patients who had predeposited autologous blood than in those who had not.
The strengths of this study are the high number of cases and the standardised procedures, all operations having been performed by a single orthopaedic surgeon and a single anaesthesiologist. Our data suggest that a pre-operative autologous donation programme may often be useless, if not harmful. Conversely, the use of a cell salvage system may be effective in reducing the impact of blood transfusion on a patient’s physiological status. Basal haemoglobin concentration emerged as a useful indicator of transfusion probability in total joint replacement procedures.
total hip arthroplasty; total knee arthroplasty; pre-operative autologous blood donation; post-operative blood cell salvage system; blood management
Transfusion therapy remains the main treatment for patients with severe haemoglobinopathies, but can cause adverse reactions which may be classified as immediate or delayed. The use of targeted prevention with drugs and treatments of blood components in selected patients can contribute to reducing the development of some reactions.
The aim of our study was to develop an algorithm capable of guiding behaviours to adopt in order to reduce the incidence of immediate transfusion reactions.
Materials and methods
Immediate transfusion reactions occurring over a 7-year period in 81 patients with transfusion-dependent haemoglobinopathies were recorded. The patients received transfusions with red cell concentrates that had been filtered prestorage. Various measures were undertaken to prevent transfusion reactions: leucoreduction, washing the red blood cells, prophylactic administration of an antihistamine (loratidine 10 mg tablet) or an antipyretic (paracetamol 500 mg tablet).
Over the study period 20,668 red cell concentrates were transfused and 64 adverse transfusion reactions were recorded in 36 patients. The mean incidence of reactions in the 7 years of observation was 3.1‰. Over the years the incidence gradually decreased from 6.8‰ in 2004 to 0.9‰ in 2010.
Preventive measures are not required for patients who have an occasional reaction, because the probability that such a type of reaction recurs is very low. In contrast, the targeted use of drugs such as loratidine or paracetamol, sometimes combined with washing and/or double filtration of red blood cells, can reduce the rate of recurrent (allergic) reactions to about 0.9‰. The system for detecting adverse reactions and training staff involved in transfusion therapy are critical points for reliable collection of data and standardisation of the detection system is recommended for those wanting to monitor the incidence of all adverse reactions, including minor ones.
transfusion; adverse reactions; washed red blood cells; haemovigilance
Haemoglobin screening methods need to be highly sensitive to detect both low and high haemoglobin levels and avoid unnecessary rejection of potential blood donors. The aim of this study was to evaluate the accuracy of measurements by HemoCue in blood donors.
Materials and methods
Three hundred and fourteen randomly selected, prospective blood donors were studied. Single fingerstick blood samples were obtained to determine the donors' haemoglobin levels by HemoCue, while venous blood samples were drawn for measurement of the haemoglobin level by both HemoCue and an automated haematology analyser as the reference method. The sensitivity, specificity, predictive values and correlation between the reference method and HemoCue were assessed. Cases with a haemoglobin concentration in the range of 12.5–17.9 g/dL were accepted for blood donation.
Analysis of paired results showed that haemoglobin levels measured by HemoCue were higher than those measured by the reference method. There was a significant correlation between the reference method and HemoCue for haemoglobin levels less than 12.5 g/dL. The correlation was less strong for increasing haemoglobin levels. Linear correlation was poor for haemoglobin levels over 18 g/dL. Thirteen percent of donors, who had haemoglobin levels close to the upper limit, were unnecessarily rejected.
HemoCue is suitable for screening for anaemia in blood donors. Most donors at Yazd are males and a significant percentage of them have haemoglobin values close to the upper limit for acceptance as a blood donor; since these subjects could be unnecessarily rejected on the basis of HemoCue results and testing with this method is expensive, it is recommended that qualitative methods are used for primary screening and accurate quantitative methods used in clinically suspicious cases or when qualitative methods fail.
blood donors; haemoglobin; haemoglobinometer; HemoCue; false deferral
Haemodilution during resuscitation after massive haemorrhage may worsen the coagulopathy and perpetuate bleeding.
Materials and methods
Blood samples from healthy donors were diluted (30 and-60%) using crystalloids (saline, Ringer’s lactate, PlasmalyteTM) or colloids (6% hydroxyethylstarch [HES130/0.4], 5% human albumin, and gelatin). The effects of haemodilution on platelet adhesion (Impact R), thrombin generation (TG), and thromboelastometry (TEM) parameters were analysed as were the effects of fibrinogen, prothrombin complex concentrates (PCC), activated recombinant factor VII (FVIIa), and cryoprecipates on haemodilution.
Platelet interactions was already significantly reduced at 30% haemodilution. Platelet reactivity was not improved by addition of any of the concentrates tested. A decrease in TG and marked alterations of TEM parameters were noted at 60% haemodilution. HES130/0.4 was the expander with the most deleterious action. TG was significantly enhanced by PCC whereas rFVIIa only caused a mild acceleration of TG initiation. Fibrinogen restored the alterations of TEM parameters caused by haemodilution including those caused by HES 130/0.4. Cryoprecipitates significantly improved the alterations caused by haemodilution on TG and TEM parameters; the effects on TG disappeared after ultracentrifugation of the cryoprecipitates.
The haemostatic alterations caused by haemodilution are multifactorial and affect both blood cells and coagulation. In our in vitro approach, HES 130/0.4 had the most deleterious effect on haemostasis parameters. Coagulation factor concentrates did not improve platelet interactions in the Impact R, but did have favourable effects on coagulation parameters measured by TG and TEM. Fibrinogen notably improved TEM parameters without increasing thrombin generation, suggesting that this concentrate may help to preserve blood clotting abilities during haemodilution without enhancing the prothrombotic risk.
haemodilution; coagulation factor concentrates; platelet function; thrombin generation; thromboelastometry
During transportation, platelet concentrates (PC) usually undergo a long period without agitation. Whether this interruption improves quality and viability or, contrariwise, has deleterious effects on PC stored for 48 hours (h) is unknown. The aim of this study was to investigate the effects of metabolic resting (6 h of interruption of agitation) vs continue agitation of PC stored for 48 h in the blood bank of Tehran.
Materials and methods
PC were prepared from platelet-rich plasma and stored in permeable bags in a shaker/incubator for 42 h at room temperature (20–24 ºC). Then, simply by stopping the agitator, the PC remained stationary (“resting”) without agitation for 6 h (WCA6h), prior to transfusion. In vitro measurements of platelet quality were carried out just after completion of the resting period and the results were compared with those of PC continuously agitated in the same day (designated as the control group, CA6h). The in vitro variables measured were swirling, ristocetin-induced aggregation (GPIb-related function), lactate dehydrogenase (LDH) concentration, platelet factor 4 (PF4) release and P-selectin expression (activation markers).
The mean platelet counts of the control group (CA6h) and rested (WCA6h) PC were not statistically different (P =0.548). Likewise, the mean pH values were not significantly different: WCA6h (7.16±0.08) and CA6h (7.22±0.16) (P =0.300). Although ristocetin-induced aggregation did not differ significantly between CA6h (79.2±4.4) and WCA6h (66.65±28.55) (P =0.186), WCA6h showed significantly less PFA release (P =0.015) and lower P-selectin expression (P =0.006).
We observed that PC stored under agitation for 42 h at 22–24 ºC in permeable bags and then rested for 6 h had better preserved pH, swirling and LDH and less platelet activation then PC kept under continuous agitation for the whole 48 h storage period.
platelet resting; metabolism; agitation effect; PF4; P-selectin
Volume reduction is a widely used procedure in umbilical cord blood banking. It concentrates progenitor cells by reducing plasma and red blood cells, thereby optimising the use of storage space. Sepax and AXP are automated systems specifically developed for umbilical cord blood processing. These systems basically consist of a bag processing set into which cord blood is transferred and a device that automatically separates the different components during centrifugation.
The aim of this study was to analyse and compare cell recovery of umbilical cord blood units processed with Sepax and AXP at Valencia Cord Blood Bank. Cell counts were performed before and after volume reduction with AXP and Sepax.
When analysing all the data (n =1,000 for AXP and n= 670 for Sepax), the percentages of total nucleated cell recovery and red blood cell depletion were 76.76±7.51% and 88.28±5.62%, respectively, for AXP and 78.81±7.25% and 88.32±7.94%, respectively, for Sepax (P <0.005 for both variables). CD34+ cell recovery and viability in umbilical cord blood units were similar with both devices. Mononuclear cell recovery was significantly higher when the Sepax system was used.
Both the Sepax and AXP automated systems achieve acceptable total nucleated cell recovery and good CD34+ cell recovery after volume reduction of umbilical cord blood units and maintain cell viability. It should be noted that total nucleated cell recovery is significantly better with the Sepax system. Both systems deplete red blood cells efficiently, especially AXP which works without hydroxyethyl starch.
cord blood; volume reduction; haematopoietic progenitors
Point-of-care thromboelastometry (ROTEM®) can be used to assess coagulation in whole blood. In the ROTEM® FIBTEM test, cytochalasin D eliminates the contribution of platelets to the whole blood clot; hence, only the remaining elements, including fibrinogen/fibrin, red blood cells and factor XIII, contribute to clot strength. We investigated the relationships between FIBTEM maximum clot firmness (MCF), whole blood fibrinogen concentration and plasma fibrinogen concentration to determine the impact of haematocrit on these parameters during cardiac surgery.
Materials and methods
The relationships between FIBTEM MCF and both whole blood fibrinogen concentration and plasma fibrinogen concentration (Clauss assay) were evaluated pre-operatively and after cardiopulmonary bypass/protamine administration in haematocrit-based subgroups.
The study included 157 patients. The correlation coefficient rho between FIBTEM MCF and plasma fibrinogen concentration was 0.68 at baseline and 0.70 after protamine, while that between FIBTEM MCF and whole blood fibrinogen concentration was 0.74 at baseline and 0.72 after protamine (all P <0.001). In subgroup analyses based on haematocrit levels, pre-operative FIBTEM MCF and whole blood fibrinogen concentration were both significantly higher (P <0.05) for the lowest haematocrit subgroup, but plasma fibrinogen concentration was similar in all groups. After protamine, no significant differences were observed between the lowest haematocrit group and the other groups for any of the three parameters.
The effect of haematocrit on blood clotting is not reflected by plasma fibrinogen concentration, in contrast to FIBTEM MCF which incorporates the contribution of haematocrit to whole blood clot firmness. This effect does, however, appear to be negligible in haemodiluted patients.
cardiac surgery; fibrinogen; FIBTEM; haematocrit; thromboelastometry
Premature babies may receive multiple transfusions during the first weeks of their life. Strong associations exist between the receipt of blood transfusions and the development of the major consequences of prematurity such as retinopathy and chronic lung disease. The possible physiological link between the receipt of blood and disease is unclear, but iron-induced oxidative damage and/or bacterial colonisation would promote these conditions. Premature babies are poorly equipped to deal with any increases in iron and oxidative load that they may acquire via blood transfusions. To determine whether there are any relationships between these factors, we studied iron and oxidative status of just expired (i.e. 36 days old) paediatric red blood cell (RBC) packs.
Materials and methods
Just expired paediatric RBC packs were obtained from the local blood bank. The extracellular medium surrounding the RBC was separated by centrifugation and the following parameters measured: total iron concentration, total iron binding capacity, non-transferrin-bound iron [NTBI], haemoglobin, total and reduced ascorbate, and malondialdehyde concentration.
The extracellular fluid of the paediatric packs (n =13) was rich in iron, a high percentage of which (36%) was present as potentially toxic NTBI. It was highly redox active with limited antioxidant protection and iron-binding capacity.
The extracellular medium surrounding packed RBC could potentially be toxic if administered to patients with limited iron sequestering and antioxidant capacity, such as premature babies. Further studies are required to determine at what point during storage these changes become potentially harmful so that clinical studies can examine the optimal storage time for blood destined for premature babies.
iron; oxidative stress; packed red blood cells; storage
Prediction of transfusion is presumed to reduce wastage rates in pre-operative autologus blood donation (PABD) and unnecessary providing and cross-matching in allogeneic transfusion. The clinical utility of published algorithms in predicting transfusions was analysed.
Materials and methods
In a cohort of 195 patients undergoing total hip arthroplasty, after PABD, expected transfusion needs were predicted with two published algorithms (A and B). The algorithms were then compared to actual transfusions. Assumptions and formulae of these algorithms were varied in an attempt to improve their prognostic utility.
The optimal variation of A resulted in allogeneic transfusions (PABD setting) or uncross-matched transfusions (allogeneic setting) of 27.3%, and a wastage rate of autologous units or unnecessary cross-matching of 73.8%, compared to 33.3% and 76.6%, respectively, for the original algorithm. The original version of algorithm B resulted in (allogeneic) transfusions of 78.8%, and a wastage rate or unnecessary cross-matching of 46.2%. The former could be improved by a variation of the algorithm to 69.7%. Comparing the optimal variations of both algorithms, the more elaborate algorithm A reduced overall transfusion risk significantly better (P =0.001). The two algorithms were not statistically different in reducing resource consumption (P =0.09).
Although the prognostic utility of algorithm A was significantly better for reducing overall transfusion risk, both algorithms were unable to meaningfully identify patients who would benefit from PABD or cross-matching. The algorithms could not increase the percentage of PABD patients transfused, or the percentage of cross-matched patients transfused in the allogeneic setting. Furthermore, they could neither reduce transfusion risk nor resource consumption.
allogeneic transfusion; clinical prediction rule; pre-operative autologous blood donation; primary total hip arthroplasty; transfusion risk
Timely and efficient recall of products known or suspected to be non-conforming is an important measure in the prevention of adverse events and in patients' safety. Product recall in the transfusion service is regulated by professional standards and legal acts, but publications presenting results related to the implementation of these procedures are quite rare.
Materials and methods
Data from the Croatian Institute of Transfusion Medicine (CITM) on the procedures of product recall during an 11-year period (2000–2010) were retrospectively analyzed. Reasons for product recall, their frequency, level of severity and efficiency of the procedures are presented and discussed.
During the study period, there were 245 procedures of product recall, for an average of 22 (18–29) procedures/year, all of low extent (1–25 products). Recall was required for 1/3,571 blood products issued, while the frequency of laboratory test report recalls was 1/5,447 patients. The leading reasons for product recall were suspected bacterial contamination of blood products (30.2%) and suspected or demonstrated non-conformity of laboratory test reports (28.6%). In total, 99 (40.4%) product recalls were categorized as class I, 30 (12.2%) as class II and 116 (47.3%) as class III.
According to the available literature data, the product recall procedures were performed quite infrequently by the CITM and were of low extent. There was a remarkable decreasing trend in the rate of product recall due to non-conformities or errors made at the CITM, along with a constant or increasing rate of recalls because of biological variability of blood products.
recall; blood products; test reports
In 2001, the criteria for blood donor eligibility in Italy were modified by a ministerial decree from a permanent deferral for "men who have sex with men" to an individual risk assessment of sexual behaviours. The aim of this study was to evaluate the impact of this change in donor screening criteria on the human immunodeficiency virus epidemic among blood donors in Italy.
Materials and methods
We used the data obtained from the Italian blood donor epidemiological surveillance system. We compared data collected in 2009 and 2010, when the individual risk assessment policy was applied, with data collected in 1999 when permanent deferral was applied for men who have sex with men based on a declaration of sexual orientation. We evaluated the change over time in the relative proportion of HIV antibody-positive donors who likely acquired the infection from men who have sex with men vs heterosexual sexual exposure; the relative risk was calculated using 1999 as the reference year.
In all 3 years, the majority of HIV antibody-positive donors reported sexual exposure as a risk factor for HIV infection; this proportion increased over time, although not statistically significantly. Heterosexuals always accounted for at least 40% of all HIV antibody-positive cases. The rate of HIV antibody-positive donors increased similarly in men who have sex with men and heterosexuals; specifically, the rate of HIV antibody-positive cases per 100,000 donors was more than 2-fold higher among men who have sex with men in 2009–2010 than in 1999 (2009–2010 vs 1999, RR =2.8; P =0.06), and that among heterosexuals was 1.5 fold higher (P =0.18).
When comparing the period before (1999) and after (2009–2010), the implementation of the individual risk assesment policy in 2001, no significant increase in the proportion of men who have sex with men compared to heterosexuals was observed among HIV antibody-positive blood donors, suggesting that the change in donor deferral policy did not lead to a disproportionate increase of HIV-seropositive men who have sex with men.
blood donors; HIV infection; sexual behaviour; MSM; heterosexual
RhD alloimmunisation; antibody investigation; anti-D and anti-C specificity; anti-G
The criteria for erythrocyte transfusion in stable premature infants are currently controversial. Haemodynamic measurements are not common in transfusion practice. The purpose of this study was to determine whether haemodynamic measurements could be helpful as objective criterion for transfusion decisions. We, therefore, evaluated clinical and haemodynamic changes in stable, anaemic, premature infants before and after transfusion using our current blood transfusion protocol based on a haematocrit threshold (<24%) and the neonatologist’s discretion.
Material and methods
Stable premature infants with a haematocrit level ≤30% were prospectively enrolled into the study. Cerebral, intestinal and renal blood flow velocities, cardiac function parameters and vital signs were measured up to three times following every routine haematocrit analysis. Moreover, transfused infants were evaluated three more times: directly before transfusion, and 24 hours and 72 hours after transfusion.
Thirty-six infants were enrolled and 23 of them were transfused. Subgroup analysis of transfused infants showed a significant decrease in cerebral blood flow velocities, cardiac output and heart rate. These changes persisted after transfusion. In the entire cohort, the degree of anaemia correlated with the increase of cerebral blood flow velocities, heart rate and cardiac output.
Cerebral blood velocities in the anterior cerebral artery might represent an objective Doppler sonographic criterion indicating the need for transfusion. The measurement of these velocities is non-invasive and quick and easy to perform. However, a randomised, controlled trial is necessary before a formal recommendation can be made.
anaemia of prematurity; Doppler ultrasound; premature infant; transfusion
Health care officials and legislators need accurate data on prevalence and numbers of individuals with bleeding disorders in order to plan and allot their budgets; the manufacturers of coagulation factors also need these data to estimate the amount of factors required to prevent scarcity of these products.
Materials and methods
We surveyed the prevalence of haemophilia A, haemophilia B, von Willebrand's disease and rare bleeding disorders in North-Eastern Iran. The survey was done in the period from September 2009 to March 2011. Information was collected from the medical records in three major hospitals and a haemophilia centre; the patients' updated data were obtained by telephone.
Overall in the current survey 552 patients with inherited coagulation disorders were identified and their medical records obtained. Of these, 429 (77.5%) had common bleeding disorders (haemophilia A, haemophilia B, von Willebrand's disease), 85 (15.6%) had rare bleeding disorders (deficiency of coagulation factors V, VII, X, XIII, I, XI, combined factor V and VIII deficiency) and 38 (6.9%) had platelet disorders.
The commonest bleeding disorders were haemophilia A (n=287, 51.9%), haemophilia B (n=92, 16.6%), von Willebrand's disease (n=50, 9%), factor V deficiency (n=21, 3.8%), factor VII deficiency (n=19, 3.4%), factor X deficiency (n=2, 0.36%), combined factor V and VIII deficiency (n=28, 5.8%), factor XIII deficiency (n=11, 1.99%), factor XI deficiency (n=2, 0.4%), afibrinogenaemia (n=2, 0.36%) and platelet disorders (n=38, 6.9%).
There is notable population of individuals with bleeding disorders in North-Eastern Iran.
haemophilia A and B; von Willebrand's disease; platelet disorders; rare bleeding disorders; North-Eastern Iran
Thrombotic thrombocytopenic purpura is a rare, life-threatening disease characterised by microangiopathic haemolytic anaemia, thrombocytopenia and symptoms related to organ ischaemia, mainly involving the brain and the kidney. It is associated with a deficiency of ADAMTS13, a plasma metalloprotease that cleaves von Willebrand factor. The congenital form (Upshaw-Schulman syndrome) is rare and is associated with mutations of the ADAMTS13 gene on chromosome 9q34. The clinical symptoms of congenital thrombotic thrombocytopenic purpura are variable, with some patients developing their first episode during the neonatal period or childhood and others becoming symptomatic in adulthood.
Materials and methods
We describe a case of thrombotic thrombocytopenic purpura, who presented to our attention with a relapsing form of the disease: the first episode occurred at the age of 13 months. Phenotype and genotype tests were performed in the patient and his family.
The undetectable level of ADAMTS13 in the patient was caused by two novel heterozygote missense mutations on the ADAMTS13 gene: one mutation is c.788C > T (p.Ser263Phe) on exon 7 and the second is c.3251G > A (p.Cys1084Tyr) on exon 25 of the ADAMTS13 gene. All the relatives who have been investigated were found to carry one of these missense mutations in a heterozygous state.
Although Upshaw-Schulman syndrome is a rare disease, it should be considered in all children with thrombocytopenia and jaundice in the neonatal period. In fact, once a child is confirmed to carry mutations of the ADAMTS13 gene causing early thrombotic thrombocytopenic purpura, prophylactic treatment should be started to avoid recurrence of symptoms. Genotype tests of relatives would also be important for those women in the family who could be carriers of ADAMTS13 mutations, particularly during pregnancy.
ADAMTS13; thrombotic thrombocytopenic purpura; mutation; Upshaw-Schulman syndrome; TTP
Sickle cell disease is the commonest haemoglobinopathy in Africa, the Middle East and India. In recent years, its incidence has increased dramatically also in Europe and North America because of the high rate of migration of people from endemic areas. From January 2009 to January 2010 the number of foreign residents in the province of Ferrara (Italy) increased by 12.2%: most of the immigrants were from countries at high risk of sickle cell disease. Since neonatal screening and prophylactic penicillin in early childhood could reduce mortality by 10 years of age to less than 2%, the aim of this study was to establish a neonatal screening programme for haemoglobinopathies in Ferrara.
Materials and methods
First we assessed how many pregnant women underwent haemoglobin analysis by high performance liquid chromatography before or during pregnancy and how many of them were carriers of haemoglobinopathies. Subsequently, we verified the feasibility of neonatal screening for sickle cell disease and other haemoglobinopathies, analysing cord blood by high performance liquid chromatography. Neonates found to be positive were managed by a multidisciplinary team to implement all the appropriate prophylactic and therapeutic measures.
We found that 59% of women who delivered at the University Hospital of Ferrara, from 2007 to 2009, had undergone high performance liquid chromatography. Of the 41% who were not tested, many were from areas in which sickle cell disease is common. Between September 26th 2010 and January 31st 2012, 1992 neonatal tests were performed and 24 carriers of haemoglobinopathies were identified (16 with HbS, 4 with HbC, 2 with HbE, 1 with HbD Punjab and 1 with HbD-Ouled Rabah); 42.6% of the mothers of these 1,992 neonates had not undergone high performance liquid chromatography during pregnancy.
Currently prevention of haemoglobinopathies in Italy is provided during the pre-conception period but only to patients with abnormal blood counts. Neonatal screening is useful and cost-effective to ensure early diagnosis and appropriate treatment for infants with sickle cell disease or other haemoglobinopathies.
neonatal; screening; haemoglobinopathies; HPLC
Although having a non-O blood type is now regarded as a risk factor for venous thromboembolism, the strength of this association is poorly defined, as is its interaction with inherited thrombophilia.
Materials and methods
The prevalence of non-O blood group and inherited thrombophilia (deficiencies of natural anticoagulants, factor V Leiden and prothrombin G20210A mutation) was assessed in a series of 712 consecutive patients with proximal deep vein thrombosis of the lower limbs who were referred to our Institution between 2004 and 2010, and in 712 age- and gender-matched healthy volunteers. Odds ratios (OR) of deep vein thrombosis and their 95% confidence intervals (CI) were computed for non-O group and thrombophilia, both separately and in combination.
A non-O blood group was present in 492 cases and 358 controls (OR 2.21; 95% CI, 1.78 to 2.75). A thrombophilic abnormality was present in 237 cases and 105 controls (OR 2.82; 2.18 to 3.66). The combination of non-O group and thrombophilia was present in 152 cases and 51 controls (OR 7.06; 4.85 to 10.28).
Having a non-O blood group is associated with an increased risk of proximal deep vein thrombosisof the lower limbs with or without pulmonary embolism. The addition of inherited thrombophilia increases the thrombotic risk conferred by non-O group alone by almost 3-fold.
ABO blood groups; venous thrombosis; inherited thrombophilia
Previous studies on the same group of patients investigated here demonstrated the effectiveness of radiosynovectomy in the treatment of chronic haemophilic synovitis even if one, two or three radiosynovectomy procedures (RS-1, RS-2, RS-3) may be necessary. The purpose of this study was to determine whether the joints’ response to each radiosynovectomy procedure behaved independently or not.
Materials and methods
One hundred and fifty-six radiosynovectomies were performed in 104 joints of 78 people diagnosed with chronic haemophilic synovitis. The patient’s mean age was 18 years. Fifty-eight patients required radiosynovectomy in a single joint, whereas 20 received treatment in more than one joint. Of the 104 joints subjected to radiosynovectomy, 33 were elbows, 47 knees and 24 ankles. Radiosynovectomy was carried out with either yttrium-90 or rhenium-186 (1–3 injections with 6-month intervals between them). Of the 104 joints, 68 required a single injection of the radioisotope (RS-1), 20 required two injections (RS-2) and 16 required three injections (RS-3). In eight cases (7.6%), the affected joints eventually required surgery.
An analysis of seven variables (number of bleeding episodes, articular pain, range of motion in flexion and extension, muscle strength in flexion and extension, and synovial thickness by imaging) demonstrated that each consecutive radiosynovectomy behaves independently in haemophilic synovitis.
Each consecutive radiosynovectomy behaves independently in haemophilic synovitis. This finding had not been documented in the literature before the present study.
haemophilia; synovitis; radiosynovectomy
Requirements for allogeneic red cell transfusion after total knee arthroplasty are still high (20–50%), and salvage and reinfusion of unwashed, filtered post-operative shed blood is an established method for reducing transfusion requirements following this operation. We performed a cost analysis to ascertain whether this alternative is likely to be cost-effective.
Materials and methods
Data from 1,093 consecutive primary total knee arthroplasties, managed with (reinfusion group, n=763) or without reinfusion of unwashed salvaged blood (control group, n=330), were retrospectively reviewed. The costs of low-vacuum drains, shed blood collection canisters (Bellovac ABT®, Wellspect HealthCare and ConstaVac CBC II®, Stryker), shed blood reinfusion, acquisition and transfusion of allogeneic red cell concentrate, haemoglobin measurements, and prolonged length of hospital stay were used for the blood management cost analysis.
Patients in the reinfusion group received 152±64 mL of red blood cells from postoperatively salvaged blood, without clinically relevant incidents, and showed a lower allogeneic transfusion rate (24.5% vs 8.5%, for the control and reinfusion groups, respectively; p =0.001). There were no differences in post-operative infection rates. Patients receiving allogeneic transfusions stayed in hospital longer (+1.9 days [95% CI: 1.2 to 2.6]). As reinfusion of unwashed salvaged blood reduced the allogeneic transfusion rate, both reinfusion systems may provide net savings in different cost scenarios (€ 4.6 to € 106/patient for Bellovac ABT, and € −51.9 to € 49.9/patient for ConstaVac CBCII).
Return of unwashed salvaged blood after total knee arthroplasty seems to save costs in patients with pre-operative haemoglobin between 12 and 15 g/dL. It is not cost-saving in patients with a pre-operative haemoglobin >15 g/dL, whereas in those with a pre-operative haemoglobin <12 g/dL, although cost-saving, its efficacy could be increased by associating some other blood-saving method.
total knee arthroplasty; allogeneic red cell transfusion; post-operative blood salvage; length of hospital stay; cost-effectiveness
High-quality evidence is lacking in several areas of haemophilia treatment, in part because little time is allocated to the treatment and care of haemophilia in university education in Italy. Physicians caring for patients with haemophilia must, therefore, rely on their information on background pathophysiology and more experienced colleagues. This makes diagnostic and therapeutic choices difficult, especially when the patient has concomitant disorders or psychological issues.
Material and methods
This article describes a course to educate young physicians who were already engaged in the management of haemophilia on the emerging and unmet issues of haemophilia care and to implement existing guidelines. Physicians (n=53) already caring for patients with haemophilia in their haematology, internal medicine, or paediatric practices in Italy attended the course. Problem-solving group activity and open discussion were the methods chosen to formulate consensus statements. During the specifically designed interactive course, three clinical cases were simulated: a young child with congenital dislocation of the hip, an adolescent refusing prophylactic treatment, and an elderly man with cardiovascular disorders. The physicians were asked questions during the course and, through a Wi-Fi console, were able to answer and discuss each case interactively.
Following discussion of each case, agreement was reached regarding general statements on the management of patients with severe haemophilia A in the three different age ranges considered.
This project helped to outline useful decision-making tools for handling diagnostic and treatment issues in the field of haemophilia.
haemophilia; KOGENIALE; prophylaxis; recombinant factor; guidelines
A prospective, 1-year study was performed among Italian first-time, volunteer blood donors, who account for 12% of all donations, in order to assess the frequency and serological patterns of hepatitis B virus infection and the presence of occult infection.
Materials and methods
Consecutive donors (n=31,190) from 21 blood transfusion centres, from age classes not subjected to universal HBV vaccination, were tested for HBsAg and anti-HBc by commercial immunoassays. Other HBV serological markers were searched for and qualitative and quantitative assessments of HBV-DNA were made in HBsAg and/or anti-HBc-positive individuals.
Of the 31,190 donors studied, 100 (0.32%) were positive for both HBsAg and anti-HBc, 2 for HBsAg (0.01%) alone, and 2,593 (8.3%) for anti-HBc. Of these last, 86.7% were also positive for anti-HBs (with or without anti-HBe), 2.9% were positive for anti-HBe without anti-HBs and 10.4% had no other HBV markers (anti-HBc alone). A general north-south increasing gradient of HBV prevalence was observed. Circulating HBV-DNA was found in 96.8% of HBsAg-positive subjects as compared to 0.55% (12/2,186) of anti-HBc-positive/HBsAg-negative subjects, with higher frequencies among anti-HBs-negative than among anti-HBs-positive ones (1.68% vs 0.37%; p <0.01) and among the 57 cases positive for both anti-HBc and anti-HBe (7%). HBV-DNA levels were significantly higher in HBsAg-positive subjects than in HBsAg-negative ones (median: 456 IU/mL vs 38 IU/mL).
The prevalence of HBV infection among Italian first-time blood donors is much lower than in the past. The presence of occult infections in this group was confirmed (frequency: 1 in 2,599), supporting the hypothesis of long-term persistence of HBV infection after clearance of HBsAg. HBsAg and nucleic acid amplification testing for blood screening and vaccination against HBV are crucial in order to further reduce the risk of transfusion-transmitted HBV towards zero.
HBV; anti-HBc; blood screening; occult infection; HBV-DNA
Peri-operative red blood cell transfusions have been associated with post-operative complications in patients undergoing elective orthopaedic hip or knee replacement surgery.
Materials and methods
We performed a post-hoc analysis of data extracted from a randomised study on transfusion triggers using pre-storage leucocyte-depleted red blood cells. Patients who were assigned to the most restrictive transfusion policy ("restrictive group") were compared with patients who were assigned to the most liberal policy ("liberal group"). End-points were red blood cell use, hospital stay, haemoglobin levels, post-operative complications and quality of life scores.
Of 603 patients, 26.4% patients in the restrictive group and 39.1% in the liberal group were transfused (P =0.001). The rate of post-operative infections was lower, although not statistically significantly so, in the restrictive group than in the liberal group (5.4% vs 10.2%, respectively) as was the rate of respiratory complications (1.7% vs 4.9%, respectively), whereas hospital stay, cardiovascular complications and mortality rate were not different in the two groups. Quality of life scores were not associated with type of transfusion policy, the number of red blood cell transfusions or the transfusion status.
A restrictive transfusion protocol was not associated with worse outcome and resulted in a lower transfusion rate compared to the liberal policy. Well-being (quality of life) was not associated with transfusion policy or with red blood cell transfusions.
restrictive transfusion policy; complication rate; orthopaedic surgery; red blood cell transfusion; quality of life
red blood cells; reverse typing; α-galactosidase; enzymatic conversion