ABSTRACT
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.
ABSTRACT
BACKGROUND AND OBJECTIVE: Diagnosis and management of primary immune thrombocytopenia (ITP) have changed dramatically in the last decade. The aim of the study was to obtain information about the opinion of the Spanish ITP Group (GEPTI) members regarding the best clinical practices for diagnosis and management of adult patients with ITP. MATERIALS AND METHODS: A two-round Delphi method was carried out by sending to 129 experts a 90-item questionnaire developed by 11 specialists, with a 4-point Likert scale ("never," "sometimes," "frequently," and "always") for the assessment of responses. RESULTS: Forty out of the 129 experts participated in the survey (participation rate 30.2%) and 39 completed the questionnaire (response rate 97.5%). Salient consensus points included the following: the need to indicate workup studies from a sustained platelet count < 100 x 109/L in the absence of a clear etiology; bone marrow aspiration in elderly patients with suspected ITP; beginning treatment in asymptomatic patients with a platelet count < 20 x 109/L; not exceeding 6-7 weeks of corticosteroid therapy; switching from corticosteroids to one thrombopoietin receptor agonist (TRA); switching to other TRA or other options as combinations of them with immunosuppressive drugs in case of failure; how to reduce tapering TRA; treating patients with symptomatic persistent ITP and platelet count > 20 x 109/L; and considering mucosal or severe bleeding as a basic criterion for hospital admission. CONCLUSIONS: The present consensus document provides a reference framework for the management of patients with ITP in clinical practice.
ABSTRACT
BACKGROUND AND OBJECTIVE: The high mortality attributable to severe malaria by Plasmodium falciparum is related to the grade of parasitemia. Automated erithrocytapheresis (AE) is a safe alternative to exchange transfussion, with the same potential benefits but less undesirable side effects. Literature on this technique is scarce, consisting of isolated reports or short series. The objective of this study is to describe the clinical picture and outcome observed in 6 severely ill malaria patients in whom EA was applied as complimentary therapeutic technique. PATIENTS AND METHOD: An observational prospective descriptive study was carried out of all inpatients with severe malaria in a single hospital between 1996 and 2006 in whom clinical, epidemiological and parsitological data were analyzed. RESULTS: This series included 2 women and 4 men, with a median age of 43 years. In all cases, the infection was acquired in West Sub-Saharan Africa. No patient had received antimalarial prophylaxis and all were infected by Plasmodium falciparum. The grade of parasitemia was between 10% and 35%. The number of severity criteria was between one and 4, the more frequent being hyperbilirrubinemia. All patients received conventional intravenous treatment. The total length of admission oscillated between 5 and 37 days, while the length of stay in the Intensive Care Unit varied between one and 17 days. All patients survived. CONCLUSIONS: AE is a safe technique, with the same advantages that blood exchange but lacking many of its disadvantages. A isolated parasitemia above 10%, or when a parasitaemia above of 5% is associated with any additional World Health Organization-2000 criteria of clinical severity, should constitute an indication for AE.
Subject(s)
Cytapheresis , Erythrocyte Transfusion , Erythrocytes , Malaria, Falciparum/therapy , Adult , Africa South of the Sahara , Antimalarials/therapeutic use , Female , Humans , Intensive Care Units , Length of Stay , Malaria, Falciparum/diagnosis , Malaria, Falciparum/drug therapy , Malaria, Falciparum/parasitology , Male , Middle Aged , Parasitemia/diagnosis , Prospective Studies , Quinine/therapeutic use , Severity of Illness Index , Time Factors , World Health OrganizationABSTRACT
FUNDAMENTO Y OBJETIVO: La elevada mortalidad de la malaria grave por Plasmodium falciparum serelaciona con el grado de parasitemia. La eritrocitaféresis automatizada (EA) es una alternativasegura a la exanguinotransfusión, con los mismos beneficios potenciales pero menores efectossecundarios. Sin embargo, son escasas las referencias sobre la eficacia e indicaciones de estatécnica. El objetivo de este trabajo ha sido describir las características clínicas y evolución de6 pacientes con malaria grave en los que se empleó esta técnica terapéutica complementaria.PACIENTES Y MÉTODO: Se ha realizado un estudio observacional, descriptivo y retrospectivo de todoslos pacientes con malaria ingresados en un único hospital entre 1996 y 2006. En cadacaso se recogieron los datos clínicos, epidemiológicos y parasitológicos básicos.RESULTADOS: La serie se compone de 2 mujeres y 4 varones, con una media de edad de 43años. En todos los casos la infección fue adquirida en África subsahariana. Ningún pacientehabía efectuado quimioprofilaxis antipalúdica y la especie causal fue Plasmodium falciparum.El grado de parasitemia osciló entre el 10 y el 35%. De los criterios de gravedad, cuyo númeroosciló entre 1 y 4, el más frecuente fue la hiperbilirrubinemia. Todos los pacientes recibierontratamiento convencional. La duración total del ingreso osciló entre 5 y 37 días, y la estanciaen la unidad de vigilancia intensiva, entre 1 y 17 días. Todos los pacientes sobrevivieron.CONCLUSIONES: En resumen, la EA es una técnica segura, con las mismas ventajas que la exanguinotransfusión,pero sin muchos de sus efectos adversos. De acuerdo con los datos de la bibliografíay estas observaciones, podemos señalar que una parasitemia aislada mayor del 10%o una parasitemia superior al 5% asociada a algún criterio de gravedad son indicación para larealización de EA
BACKGROUND AND OBJECTIVE: The high mortality attributable to severe malaria by Plasmodium falciparumis related to the grade of parasitemia. Automated erithrocytapheresis (AE) is a safe alternativeto exchange transfussion, with the same potential benefits but less undesirable sideeffects. Literature on this technique is scarce, consisting of isolated reports or short series. Theobjective of this study is to describe the clinical picture and outcome observed in 6 severely illmalaria patients in whom EA was applied as complimentary therapeutic technique.PATIENTS AND METHOD: An observational prospective descriptive study was carried out of all inpatientswith severe malaria in a single hospital between 1996 and 2006 in whom clinical, epidemiologicaland parsitological data were analyzed.RESULTS: This series included 2 women and 4 men, with a median age of 43 years. In all cases,the infection was acquired in West Sub-Saharan Africa. No patient had received antimalarialprophylaxis and all were infected by Plasmodium falciparum. The grade of parasitemia was between10% and 35%. The number of severity criteria was between one and 4, the more frequentbeing hyperbilirrubinemia. All patients received conventional intravenous treatment. Thetotal length of admission oscillated between 5 and 37 days, while the length of stay in the IntensiveCare Unit varied between one and 17 days. All patients survived.CONCLUSIONS: AE is a safe technique, with the same advantages that blood exchange but lackingmany of its disadvantages. A isolated parasitemia above 10%, or when a parasitaemia above of5% is associated with any additional World Health Organization-2000 criteria of clinical severity,should constitute an indication for AE