Independent validation of the adjusted GAPSS: Role of thrombotic risk assessment in the real-life setting.

Objective The objective of this report is to independently validate the adjusted Global Antiphospholipid Syndrome Score (aGAPSS) to predict thrombosis in a cohort of patients with APS and/or autoimmune disease. Methods This retrospective cohort study included 319 consecutive patients with APS and/or autoimmune disease. Data on clinical manifestations, conventional cardiovascular risk factors and aPL profile were collected. The aGAPSS was calculated for each patient by adding together the points corresponding to the risk factors. Results Among the 319 patients included (mean age: 48.0; SD 15.47), conducted over a mean period of 52 months (range: 19-394), 219 fulfilled the current APS classification criteria (PAPS diagnosed in 130 patients and APS associated autoimmune disease (aAPS) in 89 patients), and 100 patients with autoimmune disease without APS (AD). A total of 201 patients (63.0%) had a history of one or several thrombotic manifestations, 189 (86.3%) of them APS patients: 118 PAPS (mean age: 50.14; SD 15.47) and 71 aAPS (mean age: 48.13; SD 15.81). Higher aGAPSS baseline values were seen in patients with thrombosis 6.58 (SD 3.36) when compared with those without 4.90 (SD 4.33) ( p = 0.001). Conclusions This study has shown that even when anti-phosphatidylserine/prothrombin antibodies (aPS/PT) are not computed in an adjusted model of GAPSS (aGAPSS), this score represents an improvement in assessment of the risk prediction of thrombosis in APS patients and/or autoimmune disease. However, cut-off values may differ from other kinds of cohorts, which suggests that baseline characteristics in divergent groups of patients can account for differences in cut-off values of GAPSS.

Effect of addition of rituximab to salvage chemotherapy on outcome of patients with diffuse large B-cell lymphoma relapsing after an autologous stem-cell transplantation.

BACKGROUND: We have investigated if rituximab-based salvage regimens improve response rates and survival of patients with diffuse large B-cell lymphoma (DLBCL) relapsing after an autologous stem-cell transplantation (ASCT). PATIENTS AND METHODS: We have retrospectively analyzed 82 patients with DLBCL who received salvage therapy for relapse or progression after ASCT. Patients were divided into two groups, according to whether rituximab-based salvage regimens were given (n = 42, 'R-' group) or not (n = 40, 'R+' group) after ASCT. RESULTS: Patients in the R+ group had better complete remission (CR) (55% versus 21.4%, P = 0.006) and overall response (OR) (75% versus 40.4%, P = 0.001) rates, and better 3-year event-free survival (EFS) (37% versus 9%, P = 0.002) and overall survival (OS) (50% versus 20%, P = 0.005) than patients in the R- group. Patients retreated with rituximab had better CR (42.9% versus 21.4%, P = 0.032) and OR (66.7% versus 40.4%, P = 0.019) rates, and better OS (36.2% versus 20% at 3 years, P = 0.05) and EFS (36.2% versus 9% at 3 years, P = 0.05) than patients who received chemotherapy alone at relapse after ASCT. CONCLUSIONS: The addition of rituximab to salvage chemotherapy improves response rates and EFS in patients with relapsed DLBCL after ASCT. These patients may benefit from rituximab retreatment, although larger prospective studies are needed to confirm these results.

Utilización de estudios de diagnóstico radiológico en una unidad de especialidades médicas / Use of the radiological explorations in a medical specialities department

RESUMEN Fundamento y objetivo: Analizar la utilización de los estudios radiográficos y la adecuación de cada solicitud a las guías de indicación de exámenes radiológicos. Pacientes y métodos: Estudio prospectivo que incluye las exploraciones radiológicas en 202 pacientes hospitalizados entre enero y abril de 2006. Se recogieron los datos demográficos, el diagnóstico, los exámenes radiológicos, el objetivo clínico de la solicitud y el servicio solicitante. La indicación se evaluó por dos médicos en relación a las recomendaciones de las guías internacionales. Resultados: El 27,3% de las solicitudes de las exploraciones radiológicas no se adecuaron a los criterios de indicación. El servicio peticionario, el tipo de estudio radiográfico y el objetivo diagnóstico de la radiografía se asociaron con una indicación apropiada (p < 0,0001). Entre las solicitudes con intención diagnóstica, la detección de alteraciones radiológicasse asoció con una indicación adecuada (p < 0,0001). Conclusión: El uso apropiado de las exploraciones radiológicas fue similar a lo publicado en otros estudios. La indicación inadecuada parece asociarse con el uso de la radiografía simple, con estudios de seguimiento de la enfermedad y con las solicitudes de la Unidad de Medicina Intensiva

Background and objectives: To analyse the use of the radiological examinations and the adequacy of every request to the guidelines of indication of radiological investigations. Patients and methods: A prospective survey that includes the explorations carried out in 202 patients hospitalised between January and April of 2006. The demographic data, the diagnosis, the radiological exams, the clinical aim of the requests and the requesting department were analysed. The appropriateness was assessed according to the international criteria of adequacy by two experienced physicians. Results: Twenty-seven percent out of the requests of radiological investigations was no adapted to the reference criteria. The requesting department, the type of radiographies and the diagnostic aim of the radiological studies were associated with an appropriate indication (p <0.0001). Among the radiographies with diagnostic aim, the detection of radiological alterations was associated with a suitable indication of the request (p < 0.0001). Conclusion: The appropriate use of the radiological explorations was similar to other published reports. The inadequate indication seems to be associated with the use of simple radiographic explorations, with follow-up studies and with the requests from Intensive Care Unit

[Use of the radiological exploration in a medical specialities department]. / Utilización de estudios de diagnóstico radiológico en una unidad de especialidades médicas.

BACKGROUND AND OBJECTIVES: To analyse the use of the radiological examinations and the adequacy of every request to the guidelines of indication of radiological investigations. PATIENTS AND METHODS: A prospective survey that includes the explorations carried out in 202 patients hospitalised between January and April of 2006. The demographic data, the diagnosis, the radiological exams, the clinical aim of the requests and the requesting department were analysed. The appropriateness was assessed according to the international criteria of adequacy by two experienced physicians. RESULTS: Twenty-seven percent out of the requests of radiological investigations was no adapted to the reference criteria. The requesting department, the type of radiographies and the diagnostic aim of the radiological studies were associated with an appropriate indication (p < 0.0001). Among the radiographies with diagnostic aim, the detection of radiological alterations was associated with a suitable indication of the request (p < 0.0001). CONCLUSION: The appropriate use of the radiological explorations was similar to other published reports. The inadequate indication seems to be associated with the use of simple radiographic explorations, with follow-up studies and with the requests from Intensive Care Unit.

[Prevalence of hemoglobinopathies in pregnant women in the Lanzarote health sanitary area]. / Prevalencia de hemoglobinopatías en mujeres gestantes en el área sanitaria de Lanzarote.

BACKGROUND: The aim of this study is to analyze the global prevalence of carriers of heterozygous hemoglobinopathies among pregnant women settled in Lanzarote. PATIENTS AND METHODS: A epidemiologic cross-sectional observational investigation was undertaken to study the prevalence of hemoglobinopathies in 2,436 pregnant women in Lanzarote. The techniques of primary screening were hemoglobin electrophoresis on cellulose acetate at alkaline pH for the detection of hemoglobin variants, and the quantification of HbA2 and HbF for the diagnosis of b thalassemia trait. The study to confirm the diagnosis of structural hemoglobinopathies was based on hemoglobin electrophoresis on citrate agar at acid pH, isolectric focusing and high-performance liquid chromatography (HPLC). The molecular characterization of b thalassemia trait (HbA2 >3.5%) was carried out by techniques using a real time PCR procedure with specific fluorescently labelled hybridization probes and allele-specific amplification (PCR-ARMS). RESULTS: The global prevalence of hemoglobinopathies was 11.90 corresponding to 9.44 for structural hemoglobinopathies and 2.46 for heterozygous b thalassemias. A variant hemoglobin was detected on 23 women and the distribution was as follows: thirteen carriers of hemoglobin S, seven HbC trait, two HbD trait and one "unstable" hemoglobin. 82.6% of the variant hemoglobins found were from migrant population from Africa and America. CONCLUSIONS: The high prevalence of carriers of structural hemoglobinopathies in Lanzarote justifies the initiation of programs for screening for hemoglobinopathies to prevent the emergence of severe states causing disease.

[Delayed response to rituximab of cold agglutinin haemolytic disease]. / Respuesta diferida a rituximab de una enfermedad hemolítica por crioaglutininas.

The anti-CD20 monoclonal antibody rituximab has shown benefit in some patients diagnosed of severe cold agglutinin disease. Here we report our experience with rituximab in a patient with chronic haemolysis due to refractory cold agglutinin disease. An increase in the haemoglobin level was observed seven months later from rituximab administration and with a follow-up of 17 months, the patient maintains the haematological response. We suggest that rituximab can play an important role in the treatment of adult patients with refractory cold agglutinin disease with anaemia requiring transfusion. However only a few case reports of rituximab treatment in this haemolytic disease is available and there is need of large prospective studies that allow elucidate the better schedule of administration, the duration of the clinical effect, factors predictive of clinical outcome, the possible benefit of the association with other drugs and the possibility to achieve delayed and maintained responses.

Respuesta diferida a rituximab de una enfermedad hemolítica por crioaglutininas / Delayed response to rituximab of cold agglutinin haemolytic disease

El anticuerpo monoclonal anti-CD20 rituximab ha demostrado ser útil en el tratamiento de pacientes diagnosticados de enfermedad hemolítica severa por crioaglutininas. Presentamos nuestra experiencia con el empleo de rituximab en un paciente con hemólisis crónica por una enfermedad por crioaglutininas refractaria. Siete meses después de la administración del rituximab se detectó un aumento de la tasa de hemoglobina y durante el seguimiento durante 17 meses se mantuvo la respuesta hematológica. El rituximab puede jugar un papel en el tratamiento de individuos adultos con enfermedad por crioaglutininas refractarias a otros tratamientos con anemia dependientes de transfusión. Sin embargo, existen escasas series de casos que describan el empleo de rituximab en esta enfermedad hemolítica y son necesarios estudios prospectivos amplios que permitan aclarar el mejor esquema de administración, los posibles factores predictores del resultado, el beneficio de su asociación con otros fármacos y la posibilidad de obtener respuestas diferidas y mantenidas

The anti-CD20 monoclonal antibody rituximab has shown benefit in some patients diagnosed of severe cold agglutinin disease. Here we report our experience with rituximab in a patient with chronic haemolysis due to refractory cold agglutinin disease. An increase in the haemoglobin level was observed seven months later from rituximab administration and with a follow-up of 17 months, the patient maintains the haematological response. We suggest that rituximab can play an important role in the treatment of adult patients with refractory cold agglutinin disease with anaemia requiring transfusion. However only a few case reports of rituximab treatment in this haemolytic disease is available and there is need of large prospective studies that allow elucidate the better schedule of administration, the duration of the clinical effect, factors predictive of clinical outcome, the possible benefit of the association with other drugs and the possibility to achieve delayed and maintained responses

Artritis aguda gotosa y linfoma no Hodgkin / Acute gouty arthritis and non-Hodgkin´s lymphoma

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Carcinoma epidermoide de pulmón y tuberculosis en paciente con infección por el virus de la inmunodeficiencia humana / Epidermoidal lung carcinoma and tuberculosis in a patient with human immunodeficiency virus

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Mieloma múltiple y carcinoma de pulmón de células pequeñas

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Leucemia primaria de células plasmáticas / Primary plasma cell leukemia

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Síndrome neuroléptico maligno. Un caso asociado al tratamiento con Halazepam y Fluoxetina / Neuroleptic malignant syndrome. One case associated to treatment witt Halazepan and Fluoxetine

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