title: [Guidelines for diagnosis, treatment and monitoring of primary immune thrombocytopenia].
abstract: The consensus document on the diagnosis, treatment and monitoring of primary immune thrombocytopenia was developed in 2010 by specialists with recognized expertise in this disease under the auspices of the Spanish Society of Hematology and Hemotherapy and the Spanish Society of Pediatric Hematology and Oncology, with the aim to adapt to Spain the recommendations of the recently published international consensus documents. The decision to start treatment is based on bleeding manifestations and platelet count (<20×10(9)/L). The first-line treatment is corticosteroids, albeit for a limited period of 4-6 weeks. The addition of intravenous immunoglobulin is reserved to patients with severe bleeding. Splenectomy is the most effective second-line treatment. For patients refractory to splenectomy and those with contraindications or patient refusal, the new thrombopoietic agents are the drugs of choice due to their efficacy and excellent safety profile. The other treatment options have highly variable response rates, and the absence of controlled studies does not allow to establish clear recommendations. Monitoring should be individualized. In patients without active treatment, blood counts are recommended every 3-6 months, and the patient should be instructed to consult in case of bleeding, surgery or invasive procedure and pregnancy. In most of the pediatric population, the disease tends to spontaneous remission. High-dose corticosteroids in short course and intravenous immunoglobulin are the treatment of choice. Second- and further-line treatments should be monitored in specialized centers.
authors.affiliation: Servicio de Hematología, Hospital Universitario y Politécnico La Fe, Valencia, España. sanz_mig@gva.es
authors.forename: Miguel Ángel
authors.initials: MÁ
authors.lastname: Sanz
authors.forename: Vicente
authors.initials: V
authors.lastname: Vicente García
authors.forename: Antonio
authors.initials: A
authors.lastname: Fernández
authors.forename: M Fernanda
authors.initials: MF
authors.lastname: López
authors.forename: Carlos
authors.initials: C
authors.lastname: Grande
authors.forename: Isidro
authors.initials: I
authors.lastname: Jarque
authors.forename: Rafael
authors.initials: R
authors.lastname: Martínez
authors.forename: María Eva
authors.initials: ME
authors.lastname: Mingot
authors.forename: Emilio
authors.initials: E
authors.lastname: Monteagudo
authors.forename: Josep M A
authors.initials: JM
authors.lastname: Ribera
authors.forename: David
authors.initials: D
authors.lastname: Valcárcel
mesh_terms: D000368:Aged; D001565:Benzoates / Q000627:therapeutic use; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006834:Hydrazines / Q000627:therapeutic use; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis / Q000628:therapy; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis / Q000628:therapy; D011720:Pyrazoles / Q000627:therapeutic use; D011788:Quality of Life; D011961:Receptors, Fc / Q000627:therapeutic use; D053628:Receptors, Thrombopoietin / Q000819:agonists; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use; D013156:Splenectomy; D013926:Thrombopoietin / Q000627:therapeutic use
chemical_list: D001565:Benzoates; D005938:Glucocorticoids; D006834:Hydrazines; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D011720:Pyrazoles; D011961:Receptors, Fc; D053628:Receptors, Thrombopoietin; D011993:Recombinant Fusion Proteins; D013926:Thrombopoietin; C488777:romiplostim; C520809:eltrombopag