Treatment with fostamatinib in patients with immune thrombocytopenia: Experience from the Andalusian region in Spain-The Fostasur Study.

Immune thrombocytopenia (ITP) is characterized by low platelet counts (PLTs) and an increased risk of bleeding. Fostamatinib, a spleen tyrosine kinase inhibitor, has been approved as a second-line treatment for ITP. Real-world data on fostamatinib are lacking. This observational, retrospective, multicentre study, conducted in the Andalusia region of Spain, evaluated 44 adult primary ITP patients (47.7% female; median age 58 years; newly diagnosed ITP 6.8%; persistent 13.6%; chronic 79.5%; median four prior treatments) after ≥ 4 weeks of fostamatinib therapy. The median PLT at the initiation of fostamatinib was 15 × 109/L. Common reasons for starting fostamatinib were refractoriness or intolerance to prior therapy, oral medication preference, history of thrombosis and cardiovascular risk. Dosing was individualized based on efficacy and tolerance. After 2 weeks, global response rate was 56.8% (response and complete response). Response rates were 70.5%, 62.5% and 64% at 4 weeks, 12 weeks and at the end of the study respectively. Adverse events were mild, and no patients discontinued as a result. This real-world study demonstrated a response rate similar to fostamatinib as seen in the pivotal clinical trials while including newly diagnosed patients and allowing for individualized dosing.

Recommendations for the Clinical Approach to Immune Thrombocytopenia: Spanish ITP Working Group (GEPTI).

Primary immune thrombocytopenia (ITP) is a complex autoimmune disease whose hallmark is a deregulation of cellular and humoral immunity leading to increased destruction and reduced production of platelets. The heterogeneity of presentation and clinical course hampers personalized approaches for diagnosis and management. In 2021, the Spanish ITP Group (GEPTI) of the Spanish Society of Hematology and Hemotherapy (SEHH) updated a consensus document that had been launched in 2011. The updated guidelines have been the reference for the diagnosis and management of primary ITP in Spain ever since. Nevertheless, the emergence of new tools and strategies makes it advisable to review them again. For this reason, we have updated the main recommendations appropriately. Our aim is to provide a practical tool to facilitate the integral management of all aspects of primary ITP management.

Management of acquired hemophilia A: results from the Spanish registry.

The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed for a median of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only 1 patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy. Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), whereas no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within 2 months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of nonsurvivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other nonsurvivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.

Incidence, characteristics and clinical profile of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in patients with pre-existing primary immune thrombocytopenia (ITP) in Spain.

Infections are one of the well-known precipitating factors for relapses in patients with immune thrombocytopenia (ITP). Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection can sometimes lead to or be associated with thrombocytopenia due to an increase in peripheral platelet destruction from inflammatory hyperactivation. Currently, we do not know if SARS-CoV-2 infection modifies the natural evolution of chronic or persistent ITP or if previous immunosuppression of patients with ITP influences the incidence and severity of coronavirus disease 2019 (COVID-19) in this group. The present study was an observational, multicentre, national series of 32 adult patients with pre-existing ITP and subsequent SARS-CoV-2 infection, collected by the Spanish ITP Group [Grupo Español de Trombocitopenia Inmune (GEPTI)].

La consulta de terapia antitrombótica: progresando hacia la Enfermería de Práctica Avanzada / Anticoagulant therapy clinic: Moving towards Advanced Nursing Practice

En la actualidad, en nuestro país, alrededor de un millón de personas reciben anticoagulación oral. El fármaco más empleado es el acenocumarol, que requiere de controles de coagulación para constatar que el paciente se encuentra dentro de su rango terapéutico. Los pacientes suelen empezar este tratamiento en una consulta hospitalaria y, cuando se encuentran estabilizados, son derivados a atención primaria, donde son seguidos por sus enfermeras comunitarias.La práctica habitual es que estas enfermeras asuman los cambios de dosis cuando los pacientes están fuera de rango; no obstante, este aspecto no es realizado por las enfermeras hospitalarias a pesar de disponer de suficiente experiencia y conocimientos para un adecuado manejo de este tipo de pacientes.En la Unidad de Gestión Clínica de Hematología del Hospital Universitario Virgen de la Victoria de Málaga se ha implantado un modelo de Enfermería de Práctica Avanzada que incluye diversos aspectos de la atención y cuidados a los pacientes en terapia antitrombótica, entre los que se encuentra la dosificación de su tratamiento siguiendo un catálogo de rangos terapéuticos y diagnósticos

There is currently around one million people receiving oral anticoagulants in Spain. The drug most used is acenocoumarol, which requires coagulation monitoring to ensure that the patient is within its normal therapeutic range. Patients usually start this treatment in a hospital clinic and, when they are stabilised, they are referred to primary care, where they are followed-up by their community nurses. The usual practice is that nurses are responsible for changes in the dose when the patients are outside the range. This practice is not performed by hospital nurses, despite having sufficient experience and knowledge to adequately manage these types of patients. An Advanced Nursing Practice model has been introduced into the Haematology management unit of the Hospital Universitario Virgen de la Victoria, Málaga. This involves various aspects of attention and care of patients on anticoagulant therapy, and includes adjusting the doses of their treatment following a catalogue of therapeutic and diagnostic ranges

[Anticoagulant therapy clinic: moving towards Advanced Nursing Practice]. / La consulta de terapia antitrombótica: progresando hacia la Enfermería de Práctica Avanzada.

There is currently around one million people receiving oral anticoagulants in Spain. The drug most used is acenocoumarol, which requires coagulation monitoring to ensure that the patient is within its normal therapeutic range. Patients usually start this treatment in a hospital clinic and, when they are stabilised, they are referred to primary care, where they are followed-up by their community nurses. The usual practice is that nurses are responsible for changes in the dose when the patients are outside the range. This practice is not performed by hospital nurses, despite having sufficient experience and knowledge to adequately manage these types of patients. An Advanced Nursing Practice model has been introduced into the Haematology management unit of the Hospital Universitario Virgen de la Victoria, Málaga. This involves various aspects of attention and care of patients on anticoagulant therapy, and includes adjusting the doses of their treatment following a catalogue of therapeutic and diagnostic ranges.

Una experiencia de Enfermería de Práctica Avanzada en pacientes con anticoagulación oral / An Advanced Nursing Practice experience in patients receiving oral anticoagulation therapy

En países de nuestro entorno se están desarrollando modelos de Enfermería de Práctica Avanzada (EPA) en diversos ámbitos. Aunque de manera aislada,dentro de nuestras fronteras se están realizando desde hace tiempo diferentes prácticas avanzadas de Enfermería, sin que hasta el momento exista un reconocimiento explícito de ello.En entornos muy específicos, como son las consultas de tratamiento anti -coagulante oral (TAO), siguiendo recomendaciones de la Asociación Española de Hematología y Hemoterapia (AEHH), se están adoptando medidas que tienen elementos característicos de la Enfermería de Práctica Avanzada. En este trabajo se describe la experiencia del cambio organizativo que se está llevando a cabo en la consulta de TAO del Hospital Universitario Virgen de la Victoria mediante un acuerdo de colaboración multidisciplinar que todavía se encuentra en proceso de desarrollo. Todos los pacientes con controles de INR dentro de su rango terapéutico son controlados directamente por las enfermeras de la consulta, lo que actualmente supone el 60% del total de visitas diarias. Los pacientes que acuden por primera vez son atendidos por la enfermera, que realiza la historia clínica y proporciona educación sanitaria. Finalmente, se incluyen algunas reflexiones sobre el desarrollo de la EPA en España (AU)

Models of Advanced Nursing Practice (ANP) are being developed in countries within our setting involving different scopes. Even though in specific sites of our country, these advance nursing practices have been implemented for a long time now, they have not as of yet being explicitly acknowledged.In very specific settings such as Oral Anticoagulation Therapy Units, and following the recommendations issued by the Spanish Association of Hematology and Hemotherapy (AEHH), measures are being adopted which bear the characteristic elements of Advanced Nursing Practices.This work described the organisational change experience that is being carried out at the Oral Anticoagulation Therapy Unit of the Hospital Universitario Virgen de la Victoria via a multidisciplinary collaboration agreement that is still under development. All patients with INR controls within their therapeutic range are followed up directly by the unit´s nurses, which presently account for 60% of the total number of daily visits. Patients seeking consultation for the first time are seen by the nurse who takes their medical history and provides patient education. Lastly, some thoughts on the development of ANP in our country are includedin the paper for consideration (AU)