Results from the CLUES study: a cluster randomized trial for the evaluation of cardiovascular guideline implementation in primary care in Spain.

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.

The CLUES study: a cluster randomized clinical trial for the evaluation of cardiovascular guideline implementation in primary care.

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.

Idiopathic hypereosinophilic syndrome in children: report of a 7-year-old boy with FIP1L1-PDGFRA rearrangement.

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.

Platelet-specific antibodies in HLA-immunized patients receiving chronic platelet support.

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.

[Neurological form of onset in haemophagocytic lymphohistiocytosis].

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.

[Microangiopathic hemolytic anemia and thrombocytopenia. Thrombotic thrombocytopenic purpura].

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.

[Identification of a variant D(IVa) with anti-D in serum].

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.

Clinical and laboratory factors associated with platelet transfusion refractoriness: a case-control study.

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.

Influence of the red blood cell preparation method on the efficacy of a leukocyte reduction filter.

The performance of a leukocyte reduction bedside filter with different types of RBC concentrates was analyzed. Three types of RBCs were prepared: buffycoat-depleted RBCs suspended in saline-adenine-glucose-mannitol (SAGM)-additive solution (BC-RBCs; n = 20), RBCs suspended in SAGM-additive solution without buffy coat removal (SAGM-RBCs; n = 20), and RBCs drawn in CPDA-I conservative solution and processed for component preparation by the platelet-rich plasma method (CPDA-RBCs; n = 20). The units were filtered within 8 h of collection.

[Alpha interferon treatment of idiopathic myelofibrosis].

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.

[Transient appearance of anti-erythrocyte autoantibodies during Rh alloimmunization].

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.

Anti-Sa cold agglutinin of IgA class requiring plasma-exchange therapy as early manifestation of multiple myeloma.

The case of a patient (Col) with multiple myeloma presenting as chronic cold agglutinin (CA) syndrome is reported. The CA (Col) was a monoclonal IgA/k paraprotein which recognizes an antigen fully expressed in adult and newborn erythrocytes, sialidase sensitive and partially resistant to proteases. Hemagglutination-inhibition studies showed that immunodominant N-acetylneuraminic acid bound alpha 2-->3 to O-glycans of glycophorins represents the CA(Col) epitope.

Penicillamine-induced rapidly progressive glomerulonephritis in a patient with rheumatoid arthritis.

A 67-year-old woman with rheumatoid arthritis presented rapidly progressive glomerulonephritis (RPGN) after 5 months of D-penicillamine (250 mg/day) treatment. Light microscopy study showed severe glomerulonephritis with crescent formation in 60% of the glomeruli and infiltration of inflammatory cells in the wall of an arteriole. Immunofluorescence revealed scanty granular IgG, IgA and C3 deposits along the capillary walls and mesangium.

[Chronic myeloid leukemia beginning as thrombocythemia. Analysis of 5 cases].

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.