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Background: In HLA-alloimmunized patients, the unexpected failure of HLA-matched platelet transfusions usually raises the suspicion about concomitant platelet-specific antibodies. As the reported frequency of platelet-specific antibodies in multitransfused patients varies widely, the aim of this study was to determine the prevalence of such antibodies in a population of chronic thrombocytopenic patients with HLA antibodies.Study Design and Methods: From 1985 to 1997, 11,777 determinations of HLA antibodies were performed in 1330 hematologic patients receiving chronic platelet support. Fifty-two patients with HLA alloimmunization that lasted more than 1 month were selected. The search for platelet-specific antibodies was performed by using a monoclonal antibody immobilization of platelet antigens assay, thus allowing the identification of platelet-specific antibodies directed against the platelet glycoproteins (GP) Ib/IX, GPIIb/IIIa, and GPIa/IIa. Specificity of the platelet-specific antibodies was further investigated by using a solid-phase assay with chloroquine-treated platelets.Results: Only 2 (3.8%) of the 52 patients had platelet-specific antibodies. One antibody reacted with an epitope of the GPIIb/IIIa that was present in all the panel platelets, and that probably was an autoantibody. The other was an anti-HPA-5b.Conclusions: The prevalence of platelet-specific antibodies in patients with HLA alloimmunization is very small. The search for concomitant platelet-specific antibodies would be indicated only when other causes of refractoriness to HLA-matched platelets are ruled out. [ABSTRACT FROM AUTHOR]

Bone Marrow Transplantation (2003) 31, 517-518. doi:10.1038/sj.bmt.1703907 [ABSTRACT FROM AUTHOR]

Presents a study that evaluated the safety and efficacy of high-dose granulocyte colony-stimulating factor alone for PBSC mobilization in children. Methodology; Findings; Conclusion.

Background: The implementation of evidence-based clinical practice guidelines (CPG) can improve patients care. To date, the impact of implementation strategies has not been evaluated in our context. This study is aimed to evaluate the effectiveness of a multifaceted tailored intervention targeting clinician education for the implementation of three cardiovascular risk-related CPGs (type 2 diabetes, hypertension and dyslipidemia) in primary care at the Basque Health Service compared with usual implementation., Methods: We conducted a cluster randomized controlled trial in two urban districts with 43 primary care units (PCU). Data from all patients diagnosed with diabetes, hypertension and all those eligible for coronary risk (CR) assessment were included. In the control group, guidelines were introduced in the usual way (by email, intranet and clinical meetings). In the intervention group, the implementation also included a specific website and workshops. Primary endpoints were annual HbA1c testing (diabetes), annual general laboratory testing (hypertension) and annual CR assessment (dyslipidemia). Secondary endpoints were process, prescription and clinical endpoints related with guideline recommendations. Analysis was performed at a PCU level weighted by cluster size., Results: Significant differences between groups were observed in primary outcomes in the dyslipidemia CPG: increased CR assessment for both women and men (weighted mean difference, WMD, 13.58 and 12.91%). No significant differences were observed in diabetes and hypertension CPGs primary outcomes. Regarding secondary endpoints, annual CR assessment was significantly higher in both diabetic and hypertensive patients in the intervention group (WMD 28.16 and 27.55%). Rates of CR assessment before starting new statin treatments also increased (WMD 23.09%), resulting in a lower rate of statin prescribing in low risk women. Diuretic prescribing was higher in the intervention group (WMD 20.59%). Clinical outcomes (HbA1c and blood pressure control) did not differ between groups., Conclusions: The multifaceted implementation proved to be effective to increase the CR assessment and to improve prescription, but ineffective to improve diabetes and hypertension related outcomes. In order to obtain real improvements when cardiovascular issues are tackled, perhaps other or additional interventions need to be implemented besides education of professionals., Trial Registration: Current Controlled Trials, ISRCTN 88876909 (retrospectively registered on January 13, 2009).

Background: The appropriate care for people with cardiovascular risk factors can reduce morbidity and mortality. One strategy for improving the care for these patients involves the implementation of evidence-based guidelines. To date, little research concerning the impact of such implementation strategies in our setting has been published. Aims. To evaluate the effectiveness of a multifaceted tailored intervention in the implementation of three cardiovascular risk-related guidelines (hypertension, type 2 diabetes and dyslipidemia) in primary care in the Basque Health Service compared with usual implementation., Methods/design: A two-year cluster randomized clinical trial in primary care in two districts in the Basque Health Service. All primary care units are randomized. Data from all patients with diabetes, hypertension and those susceptible to coronary risk screening will be analyzed.Interventions. The control group will receive standard implementation. The experimental group will receive a multifaceted tailored implementation strategy, including a specific web page and workshops for family physicians and nurses.Endpoints. Primary endpoints: annual request for glycosylated hemoglobin, basic laboratory tests for hypertension, cardiovascular risk screening (women between 45-74 and men between 40-74 years old). Secondary endpoints: other process and clinical guideline indicators., Analysis: Data will be extracted from centralized computerized medical records. ANALYSIS will be performed at a primary care unit level weighted by cluster size., Discussion: The main contribution of our study is that it seeks to identify an effective strategy for cardiovascular guideline implementation in primary care in our setting., Trial Registration: Current Controlled Trials, ISRCTN88876909.

Introduction: The treatment of colorectal cancer (CRC) is usually surgical and involves morbidity-mortality. The aim of this study is to quantify the postoperative mortality in our hospital and to determine their risk factors., Materials and Methods: Prospective observational study from 1996 to 2007 included 1017 patients who underwent surgery for CRC in our hospital. Identification of independent risk factors for postoperative mortality by multivariate analysis., Results: The mean age was 67.8 years. The surgery was elective in 879 (86.5%) and was considered curative in 878 (86.1%). The postoperative mortality was 3.6% (37 patients), 2.5% in the elective surgery and 10.9% in the urgent. The independent risk factors identified were: type of surgery (odds ratio for urgent vs. elective=2.8), American Society of Anesthesiologists (ASA) grade (odds ratio for ASA III-IV vs. I-II=2.4), age (odds ratio for age > or = 85 vs. < or = 74=7.6 and age 75-84 vs. < or = 74=2.4)., Conclusions: We found a low postoperative mortality, which was mainly associated with age over 75 years, ASA III or IV stages and urgent surgery., (Copyright 2009 AEC. Published by Elsevier Espana. All rights reserved.)

Objective: To evaluate the viability, safety and short-term results of laparoscopic colon surgery during the first few years after its introduction in our department., Method: Between January 2002 and December 2005, laparoscopic surgery was performed in patients with surgical indication for benign colon disease. After 2003, patients with malignant disease were also included. A database was created and demographic data, surgical indication, technique, conversion rate, morbidity and postoperative length of stay were recorded. All patients were operated on by the same team of three surgeons., Results: Ninety consecutive patients, with a mean age of 59.2 years (20-88) underwent laparoscopic surgery. Of these, 53 were men (59%). In total, 32 patients had previously undergone one or more open laparotomies (35.5%). Surgery was indicated for benign disease in 60 patients (66%). Distribution was left colon in 79 patients and right colon in 11 patients. The most frequent technique was sigmoidectomy (67.7%). The conversion rate was 12.2%. Operating time was 199 min. (120-340) and length of postoperative stay was 7.5 days (4-57). Morbidity was 18.8% and mortality was 1.1%., Conclusions: Laparoscopic surgery of the colon is safe and reproducible. Our short-term results are similar to those of previous studies. We believe that prior experience of laparoscopic surgery is important and that a stable surgical team minimizes the effect of the learning curve.

Idiopathic hypereosinophilic syndrome (HES) in children is a very rare disorder; certain clinical differences with adult HES have been described, with no pediatric case with the imatinib-responsive FIP1L1-PDGFRA fusion gene reported to date. The authors describe the clinical course of three children with HES in whom FIP1L1-PDGFRA fusion gene was studied and report the first child with this rearrangement.

Objective: Thrombotic thrombocytopenic purpura (TTP) or Moschovitz' syndrome is rare and is even rarer in childhood. Clinically, it is characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. The etiology is still unknown, although different factors such as large von Willebrand factor multimers and prostacyclin have been implicated. The acute form is more frequent, and in most cases the course is fulminant if treatment is not initiated. Laboratory data typically reveal hemolytic anemia, with schistocytes on the peripheral smear, diminished serum haptoglobin, and thrombocytopenia., Material and Methods: We present the clinical cases of two children, aged 4 and 7 respectively, with TTP, but with different evolution and treatment. Evolution was favorable in both patients. The first child recovered spontaneously. In the second plasmapheresis was required and produced remission of all the symptomatology. Normality has been maintained for 36 and 24 months respectively, and the children have presented no clinico-biological alterations.

Introduction: Haemophagocyte lymphohistiocytosis (HLH) is a hematological disorder, autosomal recessive and in which there is benign proliferation of histiocytes with intense phagocytic activity of hematopoietic cells. The clinical features include fever, pancytopenia, coagulation disorders, liver dysfunction, the presence of histiocytes and haemophagocytes in the bone marrow, lymph nodes, spleen and liver. The nervous system is always involved and sooner or later patients develop a nervous system disorder with variable symptoms which may include irritability, disorders of consciousness, convulsions, ataxia, nystagmus or signs of intracranial hypertension., Clinical Case: Onset of the disease showing purely neurological features is rare. We therefore describe the case of an 8 month old baby with HLH with a purely neurological condition involving irritability, horizontal rapid eye movements and vertical saccadic movements of both eyes and focal convulsive seizures. Initial complementary examinations were normal, except for study of the CSF with a lowered protein level and cells (monocytes). Finding hepatosplenomegaly and pallor, together with the laboratory investigations, made it advisable to do a bone marrow punch biopsy to detect haemophagocytes which would be diagnostic of HLH. In spite of chemotherapy there was rapid neurological deterioration, with alterations of the white matter and hydrocephaly which required insertion of a ventriculo-peritoneal shunt. The patient died when he was 10 months old., Conclusions: The cases of HLH in which cerebromeningeal disorders alone precede systemic symptoms are extremely rare. Hence the interest in reporting this case, so that it may be borne in mind in other cases of acute neurological onset. In this case initially there was encephalitis alone, but this was rapidly followed by systemic complications.

The first D(IVa) variant described so far in Spain is presented. This variant was identified, by means of a commercial monoclonal antibodies panel, in a RhD positive patient with anti-D in her serum. Differential diagnosis is discussed and the clinical relevance of identifying these variants is commented.

In recent years clinical factors have largely surpassed alloimmunization as the predominant cause of platelet refractoriness. This makes it necessary to properly identify and weigh the non-immune factors that have a major impact of refractoriness. A case-control study is suitable for such an analysis, and to our knowledge has not previously been performed to assess this issue. Fifty-two refractory patients were compared with 52 control patients who were transfused at the same time. Only one transfusion event was analysed per patient. Clinical and laboratory data were recorded at the time of selected transfusion, and their association with refractoriness was investigated by the contingency table method and the Cox stepwise logistic regression. There were 16 (31%) patients with HLA antibodies in the index group and only one in the control group. The corrected count increment in the group of patients refractory due to HLA antibodies was significantly lower than that in non-alloimmunized refractory patients [median (range): 48.5 (-3560, 4614) and 4058 (-4417, 6886), respectively; U = 493, P < 0.0001]. In the multivariate analysis, factors associated with refractoriness were the presence of HLA antibodies (odds ratio (OR) 50.7; 95% CI 5.5-463); fever (odds ratio 7.2; 95% CI 2.5-21) and BMT because of chronic myeloid leukaemia (odds ratio 7.3; 95% CI 1.8-30). The latter two were the only factors that remained independently associated with refractoriness after excluding alloimmunized patients and their controls. We conclude that HLA antibodies are strongly associated with platelet transfusion refractoriness, but account for less than a third of these patients. Fever and BMT because of chronic myeloid leukaemia were the only non-immune factors independently associated with refractoriness.

Idiopathic myelofibrosis is a chronic myeloproliferative syndrome for which there is no effective treatment. The good results obtained with interferon in other chronic myeloproliferative syndromes have led their being tested in idiopathic myelofibrosis, but to date the experience is scarce. Four patients out of a total of 12 diagnosed with idiopathic myelofibrosis over a period of 3 years were selected for interferon treatment. Patients with low leukocyte or platelet counts or with contraindication for administration of the drug were excluded. Alpha-2b interferon was administered at an initial dose of 3 MU/day which was increased at 4-6 weeks to 5 MU/day in cases of insufficient response and if tolerance so permitted. In patients in whom favorable response was observed a maintenance schedule was initiated with low doses of interferon. Treatment was discontinued in two patients due to bad tolerance at 6 and 8 weeks of initiation of treatment with no response having been observed until that time. In the other patients favorable response was reported to interferon after 5 months of treatment with disappearance of the symptomatology, normalization of the hemo-peripheral values and a marked reduction of splenomegaly. This responses was accompanied by a decrease in bone marrow fibrosis in one case and total disappearance of the same in the other patient. Alpha-2b interferon constitutes an effective therapy for a selected number of patients with idiopathic myelofibrosis. Greater experience would allow the identification of the subgroup of patients who may benefit from this type of treatment.

Transient appearance of anti-erythrocyte autoantibodies was demonstrated in two women in the first stages of Rh alloimmunization. Any mimicking antibodies, or concurrent autoimmune haemolytic anaemia could be reasonably discarded in both patients. These facts are discussed on the basis of polyclonal activation followed by clonal selection driven by the antigen, as a mechanism of the humoral immune response. Investigation of such cases seems of great interest in subjects with recent alloimmunization in order to assess the incidence of this phenomenon and the antigen systems involved.

The clinico-haematological and evolutive features of five patients with Ph'-positive chronic myelogenous leukaemia (CML) whose initial profile suggested the diagnosis of essential thrombocythaemia (ET) were analysed. The patients were women with severe thrombocytosis (greater than or equal to 1000 x 10(9)/L) and moderate leucocytosis (less than 25 x 10(9)/L), and only two of them had splenomegaly. Increased basophil count in peripheral blood was present in all cases, and peripheral myelocytosis was seen in three. The molecular analysis showed rearrangement of the BCR gene in the three patients on which it was performed. Increasing leucocyte count was seen in the three patients with extended follow-up, this reaching values in accordance with CML. Finally, the three patients who died suffered a blastic crisis. The analysis of this series, along with others reported in the literature, suggests that such cases correspond to atypical forms of CML rather than actual TE patients.