[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.

[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.