Moderate haemophilia A: Recommendations from a Spanish panel of experts.

Introduction: Diagnosing moderate haemophilia A (MHA) solely based on deficient FVIII protein levels limits its optimal management and delays the initiation of prophylaxis. Updating protocols and incorporating new variables into its diagnosis could prevent underestimating disease severity, avoiding early arthropathies and impairing patients' quality of life.

Aim: To propose recommendations to improve the comprehensive management of people with MHA.

Management of Urgent Bleeding in Patients with Hemophilia A: Focus on the Use of Emicizumab.

Management of patients with hemophilia A (HA) requires the knowledge and experience of specialized health care professionals. However, these patients may need to be attended in emergencies, outside the referral hospital, where health care professionals do not know about hemophilia and/or new innovative treatments. This study aimed to develop a simple and practical algorithm that could be used in emergency situations by nonspecialized treaters in HA and bleeding with or without factor VIII (FVIII) inhibitors under emicizumab prophylaxis.

Tumour lysis syndrome.

Tumour lysis syndrome (TLS) is a life-threatening emergency characterised by a massive cytolysis with the release of intracellular electrolytes, nucleic acids, and metabolites into the circulation. TLS comprises laboratory derangements (hyperuricaemia, hyperkalaemia, hyperphosphataemia, and hypocalcaemia) responsible for acute kidney injury. In patients with hematologic malignancies after cytotoxic therapy or spontaneously and also in advanced solid tumours.

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.

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.

Metastatic cutaneous plasmacytoma presenting as a perianal giant mass.

There are 4 types of plasma-cell neoplasia: classic multiple myeloma (MM), extramedullary plasmacytoma without MM, solitary plasmacytoma of bone, and plasma-cell leukemia. Cutaneous involvement may be seen in all 4 types of plasma-cell neoplasia. Specific cutaneous involvement in patients with MM is very uncommon.

Origin of the frameshift codons 41/42 (-TCTT) mutation in the first cases described in the Spanish population.

Thalassemias are hereditary anemias. In beta-thalassemia (beta-thal), beta-globin synthesis is either deficient or absent. A high incidence of beta-thal is found in populations of Mediterranean and African origin.

Acute tumor lysis syndrome during oral fludarabine treatment for chronic lymphocytic leukemia. Role of treatment with rasburicase.

Introduction: Oral fludarabine is the first oral formulation of a purine analogue available for clinical use which has produced encouraging results in patients with B-cell chronic lymphocytic leukemia (CLL). It has a similar tolerability profile to that of the intravenous formulation. Acute tumor lysis syndrome (TLS) induced by fludarabine appears to be a fairly unusual complication during the treatment of CLL.

[Molecular characterization of heterozygous beta-thalassemia in Lanzarote, Spain].

Background And Objective: The aim of this study was to determine the molecular defects of heterozygous beta thalassaemia and to ascertain their distribution in Lanzarote.

Patients And Method: Molecular characterization was achieved by real time polymerase chain reaction (RT-PCR LightCycler, Roche), PCR-ARMS (PCR-amplification reaction mutations system) and DNA sequencing on an automated DNA sequencer.

Results: Two hundred forty-three heterozygous beta thalassaemia carriers were included between July 1991 and February 2007.

[Use of the radiological exploration in a medical specialities department].

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.

Paraneoplastic hyperamylasaemia in association with multiple myeloma.

Marked hyperamylasaemia associated with an amylase-producing multiple myeloma appears to be a fairly unusual phenomenon. The present report describes a fatal case of multiple myeloma associated with paraneoplastic hyperamylasaemia without evidence of pancreatic or salivary gland involvement. Serum and urine amylase levels paralleled the myeloma response to chemotherapy and disease progression.

[Osteonecrosis of the jaw in patients with multiple myeloma during and after treatment with zoledronic acid].

Background And Objective: To analyze the incidence, the clinical features, and the factors associated with the development of osteonecrosis of the jaw (ONJ) in patients with multiple myeloma (MM) treated with zoledronic acid.

Patients And Methods: Sixty-four patients diagnosed with MM and treated with zoledronic acid between August 1996 and March 2006 were included. Demographic data, predisposing factors, the type of antineoplastic therapy received and the infusions of biophosphonate were recorded.

[Delayed response to rituximab of cold agglutinin haemolytic disease].

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.

[Prevalence of hemoglobinopathies in pregnant women in the Lanzarote health sanitary area].

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.