A lesson to learn from Hemovigilance: The impact of nurses' transfusion practice on mistransfusion.

INTRODUCTION AND AIM: In recent years, there has been a gradual increase in mistransfusion events reported to our Regional Hemovigilance Division. Our objective was to design a transfusion practice nursing survey to study the causes of the increasing mistransfusion rate. MATERIALS AND METHODS: Mistransfusion rates between 2007 and 2009 were obtained from the Balearic Island Hemovigilance Division (BIHVD), one of the 17 regional HV divisions in the Spanish Hemovigilance network. The BIHVD designed a transfusion practice nursing survey to study the causes of the increasing mistransfusion rate. Every year, 614 nurses carry out around 47,000 transfusions in our region. Data were collected through voluntary, anonymous, questionnaires which included questions about socio-professional factors, transfusion training and education, together with transfusion practice questions both related and unrelated to guidelines and nurses' attitudes. Multiple regression analysis was used to investigate which mistransfusion prediction factors were most accurate. RESULTS: The survey response rate was 363 out of 614 (59.12%). Marked deficits in nurses' education and training and low transfusion frequency had a strong negative impact on the incidence of transfusion errors (r=-0.70; p=0.01). This is supported by evidence that the performance of well-trained nurses who transfused either daily or weekly and strictly followed transfusion guidelines was associated with a lower mistransfusion rate (r=-0.93; p<0.01). CONCLUSION: Nurses' training, education and how frequently a nurse transfuses are the key factors for best transfusion practice in our region. This study illustrates the feasibility of using Hemovigilance resources to investigate the causes of mistransfusion.

Pathogen inactivation technology applied to a blood component collected from an asymptomatic carrier of Leishmania infantum: a case report.

Asymptomatic Leishmania infections have been the main cause of transfusion transmission in endemic areas. Polymerase chain reaction has been used to detect L. infantum DNA in the peripheral blood of asymptomatic Leishmania carriers. In our region, the prevalence of asymptomatic L. infantum infection in donors is markedly high (5·9% of donors studied). We investigated the ability of pathogen inactivation technology, using amotosalen and UVA illumination, to eliminate L. infantum in a blood component collected from an asymptomatic L. infantum infected donor. This is the first report of the INTERCEPT system being used to eliminate a parasite from a component collected from a donor.

ADAMTS-13 activity and von Willebrand factor levels in methylene-blue photo-inactivated plasma processed by either the Springe method or an 'in house' system.

BACKGROUND AND OBJECTIVES: Methylene blue photo-inactivated plasma (MBPIP) has been reported to be less effective than fresh-frozen plasma (FFP) in the treatment of thrombotic thrombocytopenic purpura, which suggests a reduced content of the von Willebrand factor metalloprotease ADAMTS-13 in MBPIP. MATERIALS AND METHODS: ADAMTS-13 activity and von Willebrand factor antigen (vWF:Ag) levels were measured in plasma before and after photo-oxidation by either the Springe method or a commercial 'in house' system as well as in cryoprecipitate-poor plasma (CPP) and FFP (20 units each). RESULTS: Levels of ADAMTS-13 activity in MBPIP processed by the Springe method or the commercial 'in house' system were comparable to one another and did not significantly differ from levels found in FFP [median (range): 114% (57-139%), 99% (74-123%), and 106% (70-130%), respectively]. ADAMTS-13 activity was significantly reduced in CPP [median (range): 87% (70-107%) as compared with FFP (P < 0.05). Levels of vWF:Ag decreased after photo-oxidation by both methods. CONCLUSION: In vitro ADAMTS-13 activity was conserved in MBPIP processed by the two photo-oxidation methods analysed and did not significantly differ from levels found in FFP.

Methylene blue-photoinactivated plasma vs. fresh-frozen plasma as replacement fluid for plasma exchange in thrombotic thrombocytopenic purpura.

BACKGROUND AND OBJECTIVES: Plasma exchange with fresh-frozen plasma (FFP) is the treatment of choice in thrombotic thrombocytopenic purpura (TTP). Methylene blue-photoinactivated plasma (MBPIP) has been proposed as a safer alternative to FFP, but its effectiveness in the treatment of TTP is not well established. The purpose of this study was to investigate whether MBPIP is as effective as FFP in the treatment of TTP by plasma exchange. MATERIALS AND METHODS: A retrospective analysis was carried out of 56 TTP episodes, occurring between 1990 and 2003, which had been treated by plasma exchange. MBPIP was used for fluid replacement in 27 episodes and FFP in 29. The effect of plasma (MBPIP or FFP) on treatment outcomes was analysed by multivariate logistic regression. RESULTS: Compared to patients treated with FFP, those receiving MBPIP had an increased risk of dying from progressive TTP [adjusted odds ratio (OR) = 31; 95% confidence interval (CI): 1.2 to > 100], a greater number of recurrences while on plasma exchange therapy (OR = 4.6; 95% CI: 1.2-17), and a lower probability of attaining a sustained remission within 9 days of starting plasma exchange (OR = 5.2; 95% CI: 1.3-20). CONCLUSIONS: MBPIP seems to be less effective than FFP in the treatment of TTP. It is therefore prudent to avoid MBPIP until therapeutic equivalency to FFP has been established by randomized, controlled trials.

In hereditary hemochromatosis, red cell apheresis removes excess iron twice as fast as manual whole blood phlebotomy.

The current treatment of hereditary hemochromatosis (HH) consists of performing periodic manual whole blood phlebotomies. Erythroapheresis (EPH) is considered to be an alternative procedure if the classic treatment is contra-indicated. A prospective study of 13 consecutive cases of HH were included in a periodic EPH program with the aim of assessing the efficacy, feasibility, and tolerability of EPH in the treatment of HH by induction and maintenance. Iron depletion (ferritin <20 microg/l) was achieved in all patients after a mean of 6.7 +/- 2.9 months of treatment and a mean of 13.5 +/- 7.2 EPH sessions. The procedure was well tolerated and there were no complications. After a follow-up period of 10.5 +/- 6.6 months, only four patients have required further maintenance sessions beyond 6 months after completing the induction therapy. The efficacy, speed, tolerability, and more favorable schedule of an EPH program facilitate treatment of HH.

Frecuencias alélicas de polimorfismos de genes de citocinas (IL1A, IL1B, IL1RN, IL6, IL10, IL12P40, e IFNG) en una población española / Allelic frequencies of polymorphic variants of cytokine genes (IL1A, IL1B, IL1RN, IL6, IL10, IL12p40, and IFNG) in a Spanish population

Variantes polimórficas de genes de citocinas están asociadas con susceptibilidad aumentada a padecer ciertas enfermedades inflamatorias y rechazo de trasplantes, sugiriendo un papel en su patogénesis. Si estos polimorfismos de citocinas tuvieran consecuencias funcionales, diferencias entre grupos de población tendrían relevancia significativa en diferentes enfermedades y en la evolución del trasplante. Para realizar este tipo de análisis es necesario conocer la distribución de las frecuencias de estos polimorfismos en la población sana normal. En este trabajo, describimos los métodos utilizados en nuestro laboratorio para genotipar individuos para interferón (IFNG), interleucina-10 (IL-10), IL-6, IL-1, IL-12 y el antagonista del receptor de IL-1 (IL-1RN). Se enseñan las secuencias de los oligonucleótidos y las condiciones de la reacción en cadena de la polimerasa (PCR). Hemos genotipado un único panel de caucásicos sanos del sur de Europa residentes en la isla de Mallorca y se muestran las frecuencias alélicas y genotípicas de nuestra población. Estas frecuencias no difieren de las descritas para otras poblaciones de caucásicos europeos. Por tanto, nuestros datos pueden ser útiles para estudiar polimorfismos de genes de citocinas en situaciones patológicas (AU)

Prevalence of the C282Y mutation for haemochromatosis on the Island of Majorca.

The C282Y mutation of the HFE gene has been reported to be present in most of the patients with hereditary haemochromatosis (HH) of Northern European ancestry. HH affects approximately 1/300 individuals, but it is not evenly distributed in the different European countries. In the present study, polymerase chain reaction (PCR) and restriction-enzyme digestion were used to analyse the frequency of the most important mutation in haemochromatosis (C282Y) in subjects from Majorca (Balearic Islands, Spain) and patients with haemochromatosis. The results were compared with other studies from Spain and Europe. A total of 420 Majorcan chromosomes were analysed and the C282Y mutation was observed at a frequency of 2.62%+/-0.8 (11 heterozygotes: eight men and three women). In the group of hereditary haemochromatosis probands, 13 out of 14 were homozygous for the C282Y mutation. In the distribution of the C282Y mutation, a north-west to south-east cline was detected, supporting the Celtic origin of this mutation.

[Blood donation in the Balearic Islands. Analysis of supply and demand of blood from 1982 to 1987]. / La hemodonación en las Islas Baleares. Análisis de la oferta y demanda de sangre durante los años 1982 a 1987.

BACKGROUND: The supply and consumption of blood products in the autonomous community of the Balearic Islands were evaluated for the years 1982 to 1987 to assess the degree of hemotherapy coverage. METHODS: The registries of blood banks from 1982 to 1987, the clinical records of patients with congenital coagulation deficiencies and the registries of the hospital pharmacies and pharmaceutic distributing agencies were reviewed. RESULTS: The rates of use per product (units) and per one thousand population were as follows: red blood cells 22-25, platelets 0.8-3.2, transfusional plasma 0.5-2.8, and cryoprecipitates less than 0,2. The consumptions of albumin and gammaglobulins were 80 and 7.7 kg per one million population, corresponding to 3.200 and 1.540 I of plasma, respectively. The consumption of factor VIII was 1.3-0.9 international units (IU) per inhabitant, in highly of very highly purified form, corresponding to 18.500-12.857 I of plasma. CONCLUSIONS: The results show that a supply of 25-27 blood donations per one thousand population and per year and with a fractionning of 80%, yield 5.000 I of plasma per one million population. This fulfils the needs of all blood products, except those of highly or very highly purified factor VIII.

[Infective morbidity of replacement therapy in congenital coagulation deficiencies and its effects on demand of coagulation factors]. / La morbilidad infecciosa del tratamiento sustitutivo en los déficit congénitos de coagulación y su repercusión en la demanda de los factores de coagulación.

BACKGROUND: Analysis of the infective morbidity and mortality secondary to replacement hemotherapy in the population with congenital coagulation deficiencies (CCD) and their consequences on the demand for coagulation factors. METHODS: The 46 patients with CCD diagnosed in the autonomous community (AC) of the Balearic Islands (32 with hemophilia A, 6 with hemophilia B, 4 with von Willebrand's disease 2 with factor VII, 1 with factor X, and 1 with factor XII deficiencies) were investigated for infective morbidity and use of blood products from 1982 to 1987. RESULTS: 97% of the patients had some hepatitis marker, 77% had antibodies against human immunodeficiency virus (HIV) and 17% fulfilled the criteria for the acquired immunodeficiency syndrome (AIDS). There were 7 deaths (15%). The morbidity and mortality increased with age and use of blood products. There was a 46% reduction in factor VIII use between 1982 and 1986, from a mean yearly consumption per hemophiliac patient of 33444 international units (IU) to 18080 IU. CONCLUSIONS: The study results show a high prevalence of hepatitis and HIV, an important reduction in the demand of manufactured coagulation factors and a 15% reduction in the population with CCD during the study years.

Post-remission therapy in acute myeloblastic leukemia. A randomized trial comparing early consolidation plus maintenance versus maintenance therapy alone.

During a 5-year period 203 previously untreated patients with acute myeloblastic leukemia entered an intensive induction chemotherapy regimen with daunorubicin, cytosine arabinoside, 6-thioguanine, vincristine and prednisone (DATOP). The complete remission rate was 64%. Patients in complete remission were randomly assigned to 3 courses of early consolidation with DATOP at lower dosage followed by maintenance chemotherapy, or to the same maintenance regimen in the absence of any consolidation courses. No significant differences were found between these options concerning disease-free survival (median 7.0 vs. 9.8 months; p greater than 0.10) or survival (median 15.8 vs. 19.4 months; p greater than 0.10). This study, in addition to the few previously reported randomized trials, suggests that early low-dose consolidation adds no benefit to maintenance chemotherapy in acute myeloblastic leukemia once complete remission has been achieved.

Chronic myelomonocytic leukemia associated with hereditary pyruvate kinase deficiency and multiple acquired erythrocyte abnormalities.

A congenital erythrocyte pyruvate kinase (PK) deficiency was found in a 72-year old female patient with chronic myelomonocytic leukemia (CMML). Erythrocyte PK deficiency was associated with an increase in the activity of hexokinase, 6-phosphogluconate dehydrogenase and glutathione peroxidase in erythrocytes as well as a decrease in acetylcholinesterase, glutathione reductase and glucosephosphate isomerase activities. The enzymatic abnormalities were accompanied by alterations in hemoglobin and in i antigen content of erythrocyte membrane. In addition, bone marrow ultrastructural studies showed dyshemopoietic changes in all blood cell lines and especially in erythroblasts. The present findings confirm the close relationship between CMML and acquired dyserythropoietic syndromes and constitute a new observation of the infrequent association of hereditary erythrocyte enzymopathies and leukemia. A survey of the literature is presented.