Progress in the treatment of chronic immune thrombocytopenia / 国际儿科学杂志

Immune thrombocytopenia is a common bleeding disease characterized by isolated thrombocytopenia.Some patients last for more than 12 months and suffer from chronic immune thrombocytopenia(CITP). The pathogenesis of CITP is complex, and the traditional first-line has little improvement.In recent years, researches on second-line treatments(thrombopoietin and its receptor agonists, rituximab and splenectomy), immunosuppressive agents, all-trans retinoic acid, atorvastatin, and hematopoietic stem cell transplantation have provided new ideas for the treatment of CITP.This review summarizes the recent progress in the treatments of CITP and will be helpful for individualized treatment.

Inmunodeficiencias primarias y citopenias / Primary immunodeficiencies and cytopenias

Introducción: La visión actual de las enfermedades por inmunodeficiencia primaria (IDP) incluye un número creciente de síndromes que están asociados con la desregulación inmune y la autoinmunidad como características predominantes. Las citopenias autoinmunes pueden ser el primer signo de desregulación que precede a la presentación clásica de inmunodeficiencia primaria, con infecciones recurrentes u oportunistas. El conocimiento de un espectro de enfermedades potencialmente involucradas (hematológicas, reumatológicas e inmunológicas) es crucial para la identificación de una cierta proporción de genotipos y fenotipos de otros diagnósticos descritos. También permitirá excluir desórdenes como lupus eritematoso sistémico, inmunodeficiencia variable común, síndrome linfoproliferativo autoinmune; así como realizar diagnósticos diferenciales noveles como la deficiencia de GATA2, deficiencia de CD27, deficiencia de sensibilidad a lipopolisacáridos, síndrome fosfoinositol-3-quinasa delta activada, inmunodeficiencia ligada a X con déficit de magnesio y otros. Objetivo: Proporcionar una sinopsis conceptual de la aparición de citopenias en las IDP con el propósito de actualizar el conocimiento actual sobre dicho tema y de aumentar la percepción, tanto de hematólogos como inmunólogos, en relación a la presentación de citopenias como manifestación de estas enfermedades. Métodos: Se revisaron artículos originales y de corte experimental publicados en la década 2009 - 2019, en algunas bases de datos de la Biblioteca Virtual de Salud (BVS) de Cuba. Conclusiones: Al igual que las formas benignas autolimitadas de citopenia autoimmune post o parainfecciosas, o la neutropenia autoimmune adquirida de la infancia, que generalmente ocurren independientemente de una IDP subyacente reconocida, muchas de las citopenias que acompañan a esta enfermedad (pero no todas) están mediadas por autoanticuerpos. Es esencial entonces, que los médicos valoren, ante la evidencia clara de citopenia, que esta puede ser autoinmune(AU)

Introduction: The current view of primary immunodeficiency diseases (IDP) includes an increasing number of syndromes that are associated with immune dysregulation and autoimmunity as predominant characteristics. Autoimmune cytopenias may be the first sign of dysregulation that precedes the classic presentation of primary immunodeficiency, with recurrent or opportunistic infections. The knowledge of a spectrum of potentially involved diseases (hematological, rheumatological and immunological) is crucial for the identification of a certain proportion of genotypes and phenotypes of other diagnoses described. It will also allow excluding disorders such as systemic lupus erythematosus, common variable immunodeficiency, autoimmune lymphoproliferative syndrome; as well as making novel differential diagnoses such as GATA2 deficiency, CD27 deficiency, lipopolysaccharide sensitivity deficiency, activated delta phosphoinositol-3-kinase syndrome, X-linked immunodeficiency with magnesium deficiency and others. Objective: This review provides a conceptual synopsis of the appearance of cytopenias in the IDPs with the purpose of updating current knowledge on this topic and increasing the perception, of both hematologists and immunologists, in relation to the presentation of cytopenias as manifestation of these diseases. Methodos: Original and experimental articles published in the 2009-2019 decade were reviewed in some databases of the Virtual Health Library (VHL) of Cuba. Conclusions: As the self-limited benign forms of post or parainfectious autoimmune cytopenia, or childhood acquired autoimmune neutropenia, which generally occur independently of a recognized underlying IDP, many of the cytopenias that accompany this disease (but not all) mediated by autoantibodies. It is essential, then, that doctors assess, given the clear evidence of cytopenia, that it may be autoimmune(AU)

Research Progress on Chinese Medicine Immunomodulatory Intervention for Chronic Primary Immune Thrombocytopenia: Targeting Cellular Immunity / 中国结合医学杂志

Chronic primary immune thrombocytopenia (CITP) is the most common acquired autoimmune disease that seriously threaten the physical and mental health of patients. Compared with Western medicine treatment, the intervention and treatment of Chinese medicine (CM) has shown certain therapeutic advantages. This paper reviewed the new pathogenesis progress on T cell immune abnormality in CITP, and CM studies on interferes effects of cellular immune regulation of CITP in recent years. Qi deficiency failing to control blood and internal obstruction of blood stasis are the two common types of CM syndromes in CITP patients, the corresponding treatments include invigorating Pi (Spleen), supplementing qi, activating blood, as well as tonifying qi and activating yang, regulating Gan (Liver) to invigorate Pi. The authors also mentioned the problems in the research field of CM for CTIP treatment, and put forward new ideas for the research in the future.

Expression of immune autoantibodies in children with persistent/chronic immune thrombocytopenia / 中华实用儿科临床杂志

Objective To investigate the expression and clinical significance of platelet autoantibodies in children with persistent/chronic immune thrombocytopenia (pITP/cITP).Methods Total of 34 children diagnosed with pITP/cITP(14 cases and 20 cases,respectively)in the Department of Hematology and Oncology,Shanghai Children's Hospital from December 2013 to August 2014 were enrolled as the study group,including 20 male and 14 female,the median age of 5 years old.The study also included 20 healthy children (the healthy control group) matched with gender and age,and 24 cases of newly diagnosed ITP (newly diagnosed ITP group) serving as the control groups.Platelet-associated immunoglobulin (PAIg) and platelet-specific autoantibodies on surface of platelets were mea-sured by flow cytometry or flow cytometric bead.Results Significant elevation of PAIgA,PAIgM,PAIgD and specific autoantibodies against glucoprotein(GP) Ⅲa,and GP Ⅱb were demonstrated in children with cITP,as well as specific autoantibodies against GP Ⅰ b,GP Ⅲ a,GP Ⅱ b,and granule membrane protein 140 (GMP140) in children with pITP,compared with the healthy control group(P ＜ 0.05);the levels of GPⅨ,GP Ⅲ a,GMP140 in cITP group and GP Ⅱ b in pITP showed significant declination,compared with the newly diagnosed ITP group(P ＜ 0.05);between piTP group and cITP group,autoantibodies GPⅨ,GP Ⅰ b,GP Ⅱ b,and GMP140 in the latter were much lower(P ＜ 0.05).Significant negative relation between PAIgM and platelet count was found in cITP group (P ＜ 0.05).Receiver operating characte-ristic (ROC) curve analysis showed that the area under ROC curve (AUC) of GP Ⅲ a autoantibodies was larger than that of other platelet-autoantibodies in pITP/ciTP diagnosis.Conclusions Platelet autoantibodies play a significant role in pITP/ciTP,especially platelet-specific autoantibodies,which show a declining tendency in the course and may be the main mechanism.The detection of GPⅢa specific autoantibody is more advantageous against other autoantibodies in the diagnosis of piTP/ciTP.

Natural course of childhood chronic immune thrombocytopenia using the revised terminology and definitions of the international working group: a single center experience

BACKGROUND: The immune thrombocytopenia (ITP) criteria were newly standardized by the International Working Group. Thus, we analyzed the natural course of childhood chronic ITP to predict the prognosis based on the revised criteria. METHODS: The medical records of children with chronic ITP from May 2000 to February 2013 in our institute were reviewed. RESULTS: Forty-seven children with chronic ITP who were not undergoing corticosteroid therapy were included. Their initial platelet count was 23+/-25x10(9)/L, and age at diagnosis was 6.3+/-4.1 years. The follow-up period was 5.4+/-3.7 years. Among them, 44.7% (21/47) showed spontaneous remission and maintained a platelet count > or =100x10(9)/L. And 66.0% (31/47) maintained a platelet count > or =50x10(9)/L until the last follow-up date. The time periods required for the platelet count to be maintained > or =50x10(9)/L and > or =100 x10(9)/L were 3.1+/-2.7 and 3.6+/-2.7 years. Age at diagnosis in the > or =50x10(9)/L group (5.7+/-4.4 years) was significantly lower than the age at diagnosis in the or =100x10(9)/L group and or =50x10(9)/L, relatively safe state. Age at diagnosis of ITP and follow-up period were the factors influencing prognosis in this study.