El papel de los estimulantes de trombopoyetina (eltrombopag y romiplostim) en trombocitopenia inmune secundaria a Lupus

Introducción: la púrpura trombocitopénica inmune (PTI) es una manifestación hematológica frecuente en el lupus eritematoso sistémico (LES). Los corticoesteroides son la primera línea de manejo para la trombocitopenia moderada o grave, en conjunto con antimaláricos y otros inmunosupresores. En casos particulares, en donde la respuesta a las intervenciones iniciales no sea la adecuada, se cuenta con terapias de segunda línea. Objetivo: esta revisión narrativa se enfocará en dos medicamentos agonistas del receptor de trombopoyetina (RTPO): eltrombopag y romiplostim y en su papel en la PTI secundaria a LES. Además, se revisará su perfil farmacológico, dosis e indicaciones en el contexto de esta enfermedad. Métodos: se realizó una búsqueda de literatura en diferentes bases de datos, se analizaron artículos científicos y guías de manejo, tanto de LES como de trombocitopenia inmune, con el fin de contestar diferentes preguntas clínicas surgidas en el escenario de la práctica cotidiana. Resultados y conclusiones: el uso de los estimulantes de TPO es una alternativa terapéutica para escenarios particulares de pacientes con LES y trombocitopenia inmune. Sin embargo, son necesarios estudios enfocados en esta población específica para poder hacer recomendaciones acertadas acerca de su manejo. Los datos actuales son extrapolados de la trombocitopenia inmune primaria.

SUMMARY Introduction: Immune thrombocytopenia is a frequent hematologic manifestation in systemic lupus erythematosus (SLE). Corticosteroids are the first line of treatment for moderate to severe thrombocytopenia in this disease, in conjunction with antimalarials or other immunosuppressants. In particular cases where the response to initial interventions is not achieved, second-line therapies with different mechanisms of action are available. Objective: This narrative review will focus on two thrombopoietin receptor agonist drugs (TPO-RA): eltrombopag and romiplostim, and their role in immune thrombocytopenia secondary to SLE. In addition, its pharmacological profile, dose and indications will be reviewed in the context of this disease. Methods: A literature search was conducted in different databases; scientific articles and guidelines were analyzed, both for SLE and immune thrombocytopenia. With the purpose of answering different clinical questions that constantly arise in the scene of daily practice. Results and conclusions: The use of TPO-RA stimulants is a therapeutic alternative for particular scenarios in patients with SLE and immune thrombocytopenia, however studies focused on this particular population are necessary to be able to make strong recommendations about its utility. Current data are extrapolated from primary immune thrombocytopenia.

Mechanism research progress of iron removal by eltrombopag / 中国医师进修杂志

Eltrombopag is a kind of human thrombopoietin receptor agonist, which is used to treat thrombocytopenia and severe aplastic anemia. In recent years, it highlights the huge advantage in iron removal. It regulates the acidification of glucuronidation in the body, removing it from feces and urine before iron conjugates to the liver, so as to effectively reduce the overload of iron in the body.

Screening MPL515 mutations from 286 cases of essential thrombocythemia and primary myelofibrosis / 中华检验医学杂志

ObjectiveTo establish a simple and sensitive method to detect MPL515 mutations in peripheral blood of ET and PMF patients,and investigate the frequencies of the MPL515 and JAK2V617F mutations in Chinese patients.MethodsTotallv 261 patients of ET and 25 PMF cases were collected from Huashan Hospital of Fudan University and DNA samples were isolated from peripheral blood of these cases.SYBR GreenⅠreal-time PCR was used to detect JAK2V617F mutation.Taqman probe was designed to be specific for the three types of mutations ( MPl515wt,MPLW515L and MPIW515K).Real-time PCR was used to detect MPL515 mutations.Tbe results were confirmed by sequencing after T-A cloning.Results Among 261 ET patients,119 cases (45.6％ ) were identified as JAK2V617F mutation carriers and 7 cases (2.7％ ) were detected to be MPl515 mutation carriers,including 5 cases with MPLW515L,1 case with MPLW515K and 1 ease with MPLW515L + K.Additionally 10 cases with JAK2V617F(40.0％ ) and 3 cases with MPL515 ( 12.0％ ) were screened out in 25 PMF patients,including 1 case with MPLW515L and 2 cases with MPLW515L + K.One ET patient was found to harbor concurrent JAK2V617F and MPL515 mutations.ConclusionJAK2V617F mutation is the major molecular marker of ET and PMF,meanwhile MPL515 mutation is important and useful complement.