Immune thrombotic thrombocytopenic purpura in older patients: Results from the Spanish TTP Registry (REPTT).

Immune thrombotic thrombocytopenic purpura (iTTP) is an ultra-rare disease that seldom occurs in the elderly. Few reports have studied the clinical course of iTTP in older patients. In this study, we have analysed the clinical characteristics at presentation and response to therapy in a series of 44 patients with iTTP ≥60 years at diagnosis from the Spanish TTP Registry and compared them with 209 patients with <60 years at diagnosis from the same Registry. Similar symptoms and laboratory results were described in both groups, except for a higher incidence of renal dysfunction among older patients (23% vs. 43.1%; p = 0.008). Front-line treatment in patients ≥60 years was like that administered in younger patients. Also, no evidence of a difference in clinical response and overall survival was seen in both groups. Of note, 14 and 25 patients ≥60 years received treatment with caplacizumab and rituximab, respectively, showing a favourable safety and efficacy profile, like that observed in patients <60 years.

Agreements and uncertainties in autologous haematopoietic stem cell mobilization and collection. A Spanish consensus document.

Although many experts position statements on autologous stem cell mobilization have been published, there are some aspects that are still under discussion. A Spanish Hematologist expert group was summoned to settle on agreements and uncertainties on PBSCs mobilization, including factors not always considered; as apheresis and cytometry key factors that determine a successful PBSC collection. This document reviews critical factors that define poor mobilizer patients and the tools to better collect the desired stem cells for a successful autologous haematopoietic stem cell transplant.

Liberal red blood cell transfusions impair quality of life after cardiac surgery.

BACKGROUND: The optimal blood management after cardiac surgery remains controversial. Moreover, blood transfusions may have an impact on long-term outcomes. OBJECTIVE: The aim of this study is to characterize the impact of liberal red blood cell transfusions on Health-Related Quality of life (HRQoL) after cardiac surgery. METHODS: We studied a cohort of 205 consecutive patients after ICU discharge. Baseline characteristics and clinical data were recorded, and HRQoL was assessed using the EuroQoL-5D instrument, applied 6 months after ICU discharge. A specific question regarding the improvement in the quality of life after the surgical intervention was added to the HRQoL questionnaire. Risk factors related to impaired quality of life were identified using univariate comparisons and multivariate regression techniques. RESULTS: The median (interquartile range, IQR) of transfused red blood cells was 3 (1-4). Among 205 patients, 178 were studied 6 months after discharge. Impairment in at least one dimension of the EuroQoL-5D questionnaire was observed in 120 patients, with an overall score of 0.8 (IQR 0.61-1). The number of red blood cell transfusions was related to an impaired HRQoL (OR 1.17 per additional unit, 95% confidence interval 1.03-1.36, p=0.03), a trend to lower visual analog scale score (coefficient -0.75 per additional unit, 95% confidence interval -1.61 to 0.1, p=0.09) and an absence of improvement in HRQoL after surgery compared to the previous status (OR 1.13, 95% confidence interval 1.03-1.25, p=0.01). CONCLUSIONS: Liberal red blood cell transfusions increase the risk of impaired HRQoL after cardiac surgery.

Utility of red blood cell apheresis in autologous blood donation.

UNLABELLED: Although autologous blood donation is an alternative to allogeneic transfusion, some authors had questioned its cost-effectiveness. New techniques, like red blood cell apheresis could improve the cost-effectiveness of autologous blood transfusion, therefore we have valued the efficiency of this procedure in autologous blood donation. MATERIALS AND METHODS: We studied 131 patients undergoing different types of surgery who entered the preoperative autologous blood donation program over a one year period. Apheresis was performed with the MCS 3p from Haemeonetics. RESULTS: We were able to collect 304 red blood cell units from 131 patients. The average yield per procedure was two units (88 cases, 67.2%). In 41 patients (31.3%), we collected 3 units and, in two cases, 4 units were collected. The mean volume of the units was 255 (191-280). 18 (13.7% patients had an adverse reaction. Most of these were mild. Only in one case was it necessary to stop the procedure. 202 units (66.4%) were transfused to 97 patients (74%). 12 (9.2%) patients also used allogeneic transfusions (mean units: 0.18+/-0.05 with a range 1-5). CONCLUSION: Red blood cell apheresis is a useful procedure in autologous blood donation.

[Complications of plateletpheresis procedures]. / Complicaciones de los procedimientos de tromboaferesis.

PURPOSE: Thrombopheresis procedures have been recently expanded with the development or different programmes. Taking into account that this reasonably safe procedure is not devoid of complications, it would be desirable to select those individuals with lower risk of suffering adverse side effects as donors. The thrombopheresis procedures performed in our hospital between 1986 and 1997 were analysed in order to establish the useful guidelines for such selection. MATERIAL AND METHODS: All the thrombopheresis procedures performed in the Asturias Central Hospital blood bank in the 1986-1987 period were analysed. The first procedure per donor, along with all data referred to adverse effects appearing during thrombopheresis, were collected. Sex, age, body, weight, blood cells count (before and after thrombopheresis) and serum calcium levels (before and after thrombopheresis) were taken as variables with predictive value for adverse effects. With regard to the procedure, the model of cell separator, the duration of the procedure, the amount and type of anticoagulant solution and the prophylactic use of calcium ions were assessed. RESULTS: A total number of 1,024 thrombophereses were analysed. Some types of adverse effect were seen in 259 instances (25.3%). Of these, 70.3%, were mild, 29.3% moderate and 0.4% severe. The commonest adverse effect was perioral paraesthesia. Of the different variables studied, female sex and low weight acquired predictive value with respect to the occurrence of adverse effects. Prophylactic administration of calcium did not prevent the appearance of complications. CONCLUSIONS: The thrombopheresis procedures may present adverse side effects in a high percentage of cases, which, although mostly mild, require specialised personnel for identification and management. Males weighing over 70 kg are less prone to suffer such effects. Oral administration of calcium before the apheresis does not prevent the adverse reactions.

Factor V Leiden (R506Q) and risk of venous thromboembolism: a case-control study based on the Spanish population.

Resistance to activated protein C (APC) is a frequent cause of thrombophilia. Most patients showing APC-resistance have a G to A mutation at codon 506 of the factor V that converts arginine to glutamine. This mutation is present in populations worldwide with frequencies ranging from 0.01 to 0.05. Genotyping of 150 control individuals from the Spanish population showed that 3.33% of them carried the mutation. Several studies have measured resistance to APC (following a classical functional assay) and have determined the factor V genotype in a number of thrombophilic patients, in an attempt to compare the predictive value of both laboratory methods. To assess the incidence of the factor V mutation among Spanish thrombophilic patients, we genotyped 51 of these. The frequency of mutation carriers rose from 3.33% in the controls to 53% in the patients. We found significant differences for the thrombosis-free survival curves and for the age at the first thrombotic event between patients who carried or did not carry the mutation. Analysis of relatives of 16 patients who carried the factor V mutation suggests the existence of additional genes that modulate the effect of the factor V gene in the development of venous thrombosis among carriers of the G to A mutation.

[Adequacy of blood component transfusion according to previously established criteria]. / Adecuación de la transfusión de hemoderivados a criterios previamente establecidos.

PURPOSE: Transfusion is not devoid of adverse effects; therefore every transfusional procedure must be indicated correctly. With this in mind in 1991 transfusion guidelines were published by our Hospital Transfusion Committee. Four years later, a retrospective audit was performed. MATERIAL AND METHODS: We have evaluated all blood component orders (order is a transfusion request) during one month, classifying them as appropriate or inappropriate according to the transfusion guidelines. The evaluation was performed 24 hours after transfusion. The results obtained were compared with the ones obtained before the published transfusion guidelines. RESULTS: 481 transfusion orders were studied. 87 percent (420/481) were considered appropriate according to the transfusion guidelines. These 481 orders referred to the transfusion of 1178 units to 293 patients. 12 percent of the transfused units was considered as inappropriate. 16 percent of the patients (48/239) had at least on inappropriate transfusion procedure. The comparison with the data obtained in 1991 showed a significant increase of the appropriate transfusion orders (74 versus 87 percent). The use of fresh frozen plasma showed the greatest incidence in inappropriate use, mainly in haemorrhagic disorders without factor deficit. The patients with haemoglobin levels between 70 and 100 g/L without signs of hypoxia were the most important cause of inappropriate use of red cell concentrates. And the patients with platelet levels > 20 x 10(9)/L were the major cause of inappropriate use of platelet concentrates. CONCLUSION: Transfusion guidelines knowledge improves the use of blood components.

[Thrombophilia in patients with venous thromboembolism]. / Trombofilia en pacientes con tromboembolismo venoso.

PURPOSE: To assess the incidence of congenital and acquired thrombophilia and to analyse the clinical characteristics of a group of patients with high risk criteria for thrombophilia. PATIENTS AND METHODS: Two hundred and eighty-five consecutive patients seen at the anticoagulant outpatient clinic of the Oviedo Central Hospital between 1987 and 1993 were evaluated. The patients had to meet one or more of the following: 1) venous thrombosis (VT) under 45 years of age; 2) repeat VT; 3) family history of VT; 4) unusual VT location (mesenteric, brain, etc.). The study was performed 4 to 7 months after the first acute episode and at least one month after suppression of anti-vitamin K treatment. The following test were carried out: blood cell counts, basic coagulation tests (APTT, PT, TT, RT and fibrinogen), lupus-like anticoagulant detection, with and without platelet extract, diluted tissular thromboplastin inhibition test, antibodies, anticardiolipin, liver and kidney functional screen, cholesterol, HDL, triglycerides and glycaemia. The venous occlusion test after 20-minute stasis was used for the global fibrinolysis study. The statistical evaluation was performed with the SPSS programme. RESULTS: Biologic alterations were present in 98 patients (35%), 12% corresponding to congenital thrombophilia and 23% to acquired thrombophilia. The study was normal in 187 patients (65%). Of the patients with congenital thrombophilia, 4.9% had protein C (PC) deficit, 3.4% protein S (PS) deficit, and 2.4% antithrombin III (AT-III) deficit. Of the patients with acquired thrombophilia, 4.5% had antiphospholipid antibodies and 18% had impaired fibrinolysis. Of all the data analysed, the patient history was found of scarce predictive value as to the risk for thrombophilia. Significant differences were found for family history of VT (p < 0.0005) and for the association of more than one criteria for inclusion (p < 0.001). CONCLUSIONS: No conclusions could be drawn from this study regarding the prophylactic attitude in patients with congenital abnormalities or anti-phospholipid antibodies. It is recommended to assess in such patients PC, PS and AT-III activities.

[Alloimmunization in polytransfused patients. Usefulness of selecting erythrocytes compatible with antigens different from ABO and D]. / Aloinmunización en pacientes politransfundidos. Utilidad de seleccionar hematíes compatibles para antígenos differentes al ABO y D.

PURPOSE: Antibody formation against red blood cells' antigen is a very important complication due to transfusions, and it can make the following transfusions difficult. To avoid this, it has been proposed giving identical red blood cells for the antigens more frequently involved in sensitizations. To evaluate this fact, we have accomplished a study with the transfused patients in our Hospital since 1990. MATERIAL AND METHODS: 10,308 transfused patients in the Hospital Nitra. Sra. de Covadonga (Oviedo), between January 1990 and March 1994, were studied. The patients have been included in two groups: The first one was constituted by 4,226 patients from the Haematology and the Nephrology Departments, who received red blood cells units compatible with ABO and CcDEe antigens. The second group was formed by the remainding patients transfused with red blood cells compatible only with ABO and D antigens. RESULTS: All 165 antibodies were detected in 132 patients, which means an incidence of 1.3 percent. In 63 cases, antibodies were present before the first transfusion. In the remaining patients, an allosentitization of 0.2 percent in group 1 and 1 percent in group 2 (p < 0.0001) was shown. This difference cannot be explained only for transfusing red blood cells with the same Rh phenotype in the group 1, because a lower immune response had persisted when we analyzed the other antibodies. More than 70 percent of antibodies appeared before the 10th transfusion. DISCUSSION: A lower sensitization exist in the patients of group 1. This seems to be caused by a state of immunosuppression for their disease or their treatments. However, in some patients, the risk of allosensitization persists, so we think it is a good practice to transfuse red blood cells without the most immunogenic antigens in haematological and nephrological patients who already have one antibody.

[Chronic posttransfusion graft-vs-host disease in a patient with non-Hodgkin's lymphoma]. / Enfermedad de injerto contra huésped postransfusional en un paciente con linfoma no Hodgkin.

We report a case of a graft-versus-host disease (GVHD) in a patient with non Hodgkin Lymphoma who received multiple blood transfusion for anemia and thrombocytopenia. Although WBC-reduction filters were used, the patient developed a transfusion associated graft-versus-host disease. We do not recommend WBC-reduction filters to prevent postransfusional-GVHD.