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1.
Infectio ; 25(1): 28-32, ene.-mar. 2021. tab, graf
Article in English | LILACS, COLNAL | ID: biblio-1154398

ABSTRACT

Summary The Human T-lymphotropic virus type 1 (HTLV-1), a retrovirus with oncogenic properties, affects around ten to twenty million people worldwide. The most common disorders associated with HTLV-1 infection are T-cell leukemia/lymphoma (ALT) and HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Studies have reported other clinical manifestations in HTLV-1 seropositive patients, including inflammatory disorders, co-infections with opportunistic agents, and pulmonary diseases. Objective: Here, we aim to describe a cohort of juvenile patients with confirmed HTLV-1 infection that showed clinical manifestations other than neurological symptoms. Methodology and patients: Retrospective analysis of clinical data describing background and clinical findings of 12 juvenile patients with confirmed HTLV-1 infection, attended during January 2018 to February 2020 in a pediatric referral hospital in Cali, Colombia. Results: 11 out 12 patients were from Colombia´s Pacific coast, 10 suffered from significant nutritional deficiencies. Six exhibited dermatological findings, 3 compatible with infective dermatitis. None of the cases exhibited clinical or laboratory findings suggesting ALT or HAM/TPS. Eight patients had structural lung disease assessed by chest Computed Tomography (CT) scans; 4 of them tested positive for galactomannan antigen in bronchoalveolar fluid suggesting pulmonary aspergillosis, and 2 others exhibited a positive PCR testing for tuberculosis. Three patients were diagnosed with autoimmune disorders; 1 patient with Crohn´s Diseases, 1 case of autoimmune thrombocytopenic purpura, and a patient with Vogt-Koyanagi-Harada syndrome (non-granulomatous uveitis). Conclusions: There is a broad range of clinical manifestations in pediatric HTLV-1 patients, and the clinician should consider structural pulmonary disease, opportunistic co-infections and autoimmune disorders in the diagnostic algorithm.


Resumen El Virus Linfotrópico de células T humanas tipo 1 (HTLV-1), retrovirus con propiedades oncogénicas, afecta alrededor de 10-20 millones de personas mundialmente. Las manifestaciónes más comúnmente asociadas a HTLV-1 incluyen leucemia/linfoma de células T (ALT) y mielopatía asociada a HTLV-1/ paraparesia espástica tropical (HAM/TSP). Estudios han reportado otras manifestaciones clínicas en pacientes positivos para HTLV-1, incluyendo enfermedades inflamatorias, coinfecciones con gérmenes oportunistas y enfermedad pulmonar. Objetivo: es describir clínicamente una cohorte de pacientes pediátricos con infección por HTLV-1 confirmada que presentan manifestaciones clínicas diferentes a síntomas neurológicos. Metogolodía y pacientes: Análisis retrospectivo de historia clínica describiendo procedencia y hallazgos clínicos en 12 pacientes con infección por HTLV-1 confirmada, atendidos durante el periodo de Enero de 2018 a Febrero de 2020 en un hospital pediátrico de referencia en Cali, Colombia. Resultados: Once de 12 pacientes procedían de la costa Pacífica Colombiana, 10 con deficiencias nutricionales significativas. Seis mostraron compromiso dermatológico, 3 compatibles con dermatitis infectiva. Ningún paciente presentó hallazgos clínicos o paraclínicos sugestivos de ALT o HAM/TPS. Ocho pacientes presentaron enfermedad pulmonar estructural evidenciada por TAC de tórax; 4 de ellos con antígeno galactomanan positivo en lavado broncoalveolar, sugiriendo aspergilosis pulmonar, y otros 2 resultaron con PCR positiva para tuberculosis. Tres pacientes presentaron enfermedades autoinmunes concomitantes: uno con Enfermedad de Crohn, uno con Púrpura Trombocitopénica Autoinmune, y un paciente con Síndrome de Vogt-Koyanagi-Harada. Conclusiones: Existe un amplio rango de manifestaciones clínicas en pacientes pediátricos con HTLV-1, considerando enfermedad pulmonar estructural, coinfecciones oportunistas y enfermedades autoinmunes dentro del algoritmo diagnóstico.


Subject(s)
Humans , Infant , Child, Preschool , Child , Adolescent , Viruses , Human T-lymphotropic virus 1 , Infections , Paraparesis, Tropical Spastic , Leukemia , Crohn Disease , Coasts , Concurrent Symptoms , Uveomeningoencephalitic Syndrome , Purpura, Thrombocytopenic, Idiopathic , Malnutrition , Dermatitis , Pulmonary Aspergillosis , Lung Diseases
2.
Rev. Soc. Bras. Med. Trop ; 54: e00292021, 2021. tab, graf
Article in English | LILACS | ID: biblio-1250832

ABSTRACT

Abstract Data on health problems and fatal complications associated with coronavirus disease (COVID-19) have consistently been reported. Although immune thrombocytopenia has been associated with multiple viral infections, only few studies have shown its association with COVID-19. Here, we have reported a case series of two cases pertaining to patients diagnosed with COVID-19-associated immune thrombocytopenia, elaborating on the clinical course, management, and response to treatment.


Subject(s)
Humans , Thrombocytopenia/diagnosis , Thrombocytopenia/etiology , Thrombocytopenia/therapy , Purpura, Thrombocytopenic, Idiopathic , COVID-19 , SARS-CoV-2
3.
Article in Chinese | WPRIM | ID: wpr-880179

ABSTRACT

Primary immune thrombocytopenia (ITP) is a blood system disease mediated by autoimmune mechanism. Currently, the goal of treatment for primary ITP is to keep patients' peripheral platelet count at a safe level to prevent severe bleeding. Recently, avatrombopag and fostamatinib have been approved by the FDA for the treatment of primary ITP in adults, while new drugs such as rozanolixizumab, efgartigimod, PRTX-100, decitabine and atorvastatin have shown efficacy in early clinical trials. This review summarizes the current accepted therapies for the clinical treatment of primary ITP in adults, and briefly discuss the progress of new therapies.


Subject(s)
Adult , Hemorrhage , Humans , Platelet Count , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Splenectomy
4.
Article in Chinese | WPRIM | ID: wpr-880164

ABSTRACT

OBJECTIVE@#To investigate the causes, treatment options and outcomes of immune thrombocytopenia (ITP) patients with splanchnic venous thrombosis (SVT).@*METHODS@#The clinical diagnosis, treatment and outcomes data of one 26-year-old male ITP patient with SVT as initial manifestation were collected. The possible causes and treatment options of the patients were discussed through literatures review.@*RESULTS@#The result of blood routine tests of the patient showed that Plt(17-38)×10@*CONCLUSION@#ITP combined with large scale of SVT is rare, and it is difficult to cure. It should be pay more attention to the possible thrombosis risk triggered by a transiently increased EOS in the blood stream. Promptly etiological treatment and the balance between anticoagulant therapy and bleeding risks should be taken in clinical practice.


Subject(s)
Aged, 80 and over , Anticoagulants/therapeutic use , Heparin, Low-Molecular-Weight , Humans , Male , Purpura, Thrombocytopenic, Idiopathic/complications , Splanchnic Circulation , Venous Thrombosis
5.
Article in Chinese | WPRIM | ID: wpr-880163

ABSTRACT

OBJECTIVE@#To explore the influencing factors in children with chronicity immune thrombocytopenia (ITP), and to provide basis for judging the prognosis and treatment in children with ITP.@*METHODS@#The clinical data of children with ITP admitted to The Second Affiliated Hospital of Anhui Medical University in the past 5 years were retrospectively analyzed and followed up for more than 1 year. According to the inclusion criteria, the eligible cases (328 cases in total) were selected and collected through medical record system retrieval, outpatient clinic and telephone follow-up. Independent influencing factors affecting the prognosis of children with ITP were obtained through single-factor and multi-factor logistic analysis, and their predictive value for the prognosis of ITP in children were evaluated.@*RESULTS@#Of 328 children with ITP, 208 were newly diagnosed with ITP (64%), 54 were persistent ITP (16%), 66 were chronic ITP (20%), and the remission rate within 1 year was 79.9%. The results of univariate analysis showed that, age, pre-morbidity history of infection and vaccination, antinuclear antibodies, initial absolute lymphocyte count(ALC) and treatment options were related to the prognosis of the children (P<0.05). Multivariate analysis showed that the history of infection and vaccination before onset, initial treatment options, and ALC at the time of initial diagnosis were independent factors affecting the prognosis of children with ITP (P<0.05). The time for platelet recovery to 100×10@*CONCLUSION@#The initial treatment plan combined with IVIG can reduce the occurrence of chronicity in children with ITP, and its efficacy is better than that of the single corticosteroids group (the platelet recovery time is shorter); history of preceding infection or vaccination, ALC at the time of initial diagnosis are independent factors affecting the prognosis of children with ITP, and the combination of the two shows a better predictive value for the prognosis.


Subject(s)
Child , Humans , Immunoglobulins, Intravenous , Prognosis , Purpura, Thrombocytopenic, Idiopathic , Retrospective Studies , Thrombocytopenia
6.
Article in Chinese | WPRIM | ID: wpr-880116

ABSTRACT

OBJECTIVE@#To investigate the effect of expression level changes of monocytic myeloid-derived suppressor cells (M-MDSC) to related immune function in the patients with primary immune thrombocytopenia (ITP).@*METHODS@#Peripheral blood samples were collected from 53 newly diagnosed ITP patients and 30 healthy volunteers. The quantity of M-MDSC, mRNA levels of Arg-1 and iNOS were detected. CD4@*RESULTS@#The count of M-MDSC in peripheral blood of newly diagnosed ITP patients was significantly higher than that in the control group (P < 0.01). However, the expression level of Arg-1 in peripheral blood was not significantly different between the newly diagnosed ITP group and the control group. But the expression level of iNOS in the newly diagnosed ITP patients was significantly higher than that in the control group (P < 0.01). After treatment, the count of M-MDSC in the patients with ITP was significantly lower than before treatment (P < 0.01), which showed that M-MDSC could significantly inhibit the proliferation and secretion of IFN-γ in CD4@*CONCLUSION@#M-MDSC may be related to the disorder of immune tolerance in the patients with ITP, and may become a new index to monitor the curative efficacy of ITP patients.


Subject(s)
Flow Cytometry , HLA-DR Antigens , Humans , Immunity , Myeloid-Derived Suppressor Cells , Purpura, Thrombocytopenic, Idiopathic
7.
Article in Chinese | WPRIM | ID: wpr-880115

ABSTRACT

OBJECTIVE@#To investigate the factors affecting the chronicity of childhood primary immune thrombo-cytopenia (ITP) and compare the efficiency of different first-line treatment regimens.@*METHODS@#Children with ITP hospitalized in our hospital from September 2013 to October 2018 were retrospectively analyzed.@*RESULTS@#Three hundred and one children (150 males and 151 females) were included in this study, with a median age of 8 (0.17-17) years old, and 110 (36.5%), 92 (30.6%), and 99 (32.9%) cases were grouped into newly diagnosed, persistent, and chronic ITP, respectively. The median of follow-up was 41.92 (1.07-74.03) months. At the end of the follow-up (October 2019), among the 202 newly diagnosed/persistent ITP children, 79 cases (59 newly diagnosed and 20 persistent ITP) achieved remission within 1 year after initial diagnosis, with a remission rate of 39.3%; 122 cases (50 newly diagnosed and 72 persistent ITP) developed chronic disease, with a chronicity rate of 60.7%; one case underwent splenectomy. In 99 cases with chronic ITP, 5 cases underwent splenectomy. Multivariable logistic regression analysis showed that, the insidious onset of symptoms (OR=3.754, 95%CI: 1.882-7.488, P=0.000) increased the risk of chronicity, while the positive antibody to anti-platelet membrane glycoprotein (OR=0.446, 95%CI: 0.224-0.888, P=0.021) might reduce the risk of chronicity. And no difference was found by the analysis of subtype of anti-platelet membrane glycoprotein (P=0.305). The efficacy of the first-line treatment of intravenous immunoglobulin (IVIG) alone or combined with steroid was better than that of steroid alone (P=0.028, 0.028), however, the efficiency was not significantly different between IVIG alone and combined with steroid (P=0.086).@*CONCLUSION@#Insidious onset of symptoms in pediatric ITP increases the risk of chronicity, while the positive titer of anti-platelet membrane glycoprotein may reduce the risk. In the first-line treatment for the newly diagnosed/persistent children. The efficacy of IVIG alone or combined with steroid is better than that of steroid alone.


Subject(s)
Adolescent , Child , Child, Hospitalized , Female , Humans , Immunoglobulins, Intravenous , Male , Purpura, Thrombocytopenic, Idiopathic , Retrospective Studies , Splenectomy
8.
Journal of Experimental Hematology ; (6): 1332-1337, 2020.
Article in Chinese | WPRIM | ID: wpr-827116

ABSTRACT

OBJECTIVE@#To investigate the influencing factors on prognosis of adult patients with chronic primary immune thrombocytopeuia (ITP) after rituximab treatment and predictive value of platelet (Plt) count.@*METHODS@#Clinical data of 52 adult patients with chronic primary ITP treated with rituximab from January 2012 to December 2016 were retrospectively analyzed, including 32 patients for failed in treatment as group A and 20 patients for succeeded in treatment as group B. The independent risk factors influencing the clinical efficacy of rituximab were analyzed. The influence of CD41 megakaryocyte count in bone marrow diagnosed for first time on the response rate of patients with 1-year followed-up were observed, and the Plt count were calculated to predict the clinical efficacy index and the best cut-off point.@*RESULTS@#The CD41 megakaryocyte count in bone marrow for first time treatment in group B were significantly higher than that in group A (P<0.05). Multivariate Logistic regression analysis showed that the number of CD41 megakaryocytes in bone marrow<150 at first diagnosis was the independent risk factor influencing the clinical efficacy of rituximab (OR=5.40,95%CI:1.82-15.66,P=0.00). The response rate of 1-year followed-up in patients with CD41 megakaryocyte count ≥150 at first diagnosis was significantly higher than that of CD41 megakaryocyte count <150 (P<0.05). The Plt count level in group B was significantly lower than that in group A at the 3rd, 14th, 21th, 30th, 60th, 90th, 180th, 270th and 360th days after first treatment with rituximab (P<0.05). ROC curve analysis showed that the best cut-off point for Plt count was 50×10/L and AUC was 0.68 at the 14th day after first treatment with rituximab (95%CI: 0.57-0.78, P=0.00). The predictive sensitivity and specificity of clinical efficacy in adult patients with chronic primary ITP treated with rituximab were separately 48.73% and 87.58%, and the AUC in 30th and 60th day after rituximab treatment were separately 0.74 (95%CI: 0.64-0.87, P=0.00), 0.93 (95%CI:0.82-0.98,P=0.00).@*CONCLUSION@#Adult patients with chronic primary ITP may possess long-term remission after rituximab treatment, but the prognosis is poor for patients with bone marrow megakaryocyte count <150. The Plt counts in 14th, 30th and 60th days after rituximab treatment can effectively predict the long-term clinical efficacy and guide the formulation of treatment plans.


Subject(s)
Adult , Humans , Megakaryocytes , Platelet Count , Prognosis , Purpura, Thrombocytopenic, Idiopathic , Retrospective Studies , Rituximab
9.
Journal of Experimental Hematology ; (6): 1338-1343, 2020.
Article in Chinese | WPRIM | ID: wpr-827115

ABSTRACT

OBJECTIVE@#To explore the value of has-microRNA-155 (miR-155) in peripheral blood mononuclear cells (PBMNC) in prognostic evaluation of elderly patients with primary immune thrombocytopenia (PITP).@*METHODS@#One hundred and thirty elderly PITP patients and 60 healthy volunteers in our hospital were selected. The relative expression level of miR-155 in PBMNC was detected by RT-PCR. Unconditional logistic multivariate regression analysis was used to analyze the relationship between miR-155 expression and prognosis of PITP patients, and Kaplan-Meier was further used to analyze the relationship between miR-155 and PITP recurrence.@*RESULTS@#The relative expression level of miR-155 in PBMNC of elderly PITP patients was significantly higher than that in healthy volunteers, and increased significantly with the severity of the disease (P<0.05). The overall effective rate of elderly PITP patients with miR-155 low-expression was significantly higher than that in the patients with miR-155 high-expression (96.92% vs 72.31%) by after treatment with glucocorticoid. Multivariate analysis showed that miR-155 was an independent risk factor for PITP patients. Elderly patients with high expression of miR-155 showed a higher risk of recurrence.@*CONCLUSION@#miR-155 in PBMNC has a high accuracy for PITP diagnosis, and the elderly patients with high level of miR-155 show a poor prognosis and a higher risk of recurrence.


Subject(s)
Aged , Biomarkers, Tumor , Humans , Kaplan-Meier Estimate , Leukocytes, Mononuclear , MicroRNAs , Neoplasm Recurrence, Local , Prognosis , Purpura, Thrombocytopenic, Idiopathic
10.
Einstein (Säo Paulo) ; 18: eRC5111, 2020. graf
Article in English | LILACS | ID: biblio-1056048

ABSTRACT

ABSTRACT Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.


RESUMO Situs inversus totalis é uma anormalidade congênita autossômica recessiva rara em que os órgãos mediastinais e abdominais encontram-se em posição espelhada em relação à topografia habitual. A literatura relata alguns casos de concomitância do situs inversus totalis com outras condições: anomalias espinhais, malformações cardíacas e doenças hematológicas, como púrpura trombocitopênica idiopática, que é uma doença autoimune com plaquetopenia, devido à destruição dos trombócitos ou supressão da sua produção. Esse artigo teve o objetivo de relatar coexistência de situs inversus totalis e púrpura trombocitopênica idiopática.


Subject(s)
Humans , Male , Young Adult , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Purpura, Thrombocytopenic, Idiopathic/complications , Situs Inversus/pathology , Radiography, Panoramic , Tomography, X-Ray Computed
11.
s.l; RedARETS; [2020].
Non-conventional in Spanish | LILACS, BRISA | ID: biblio-1095225

ABSTRACT

TECNOLOGÍA EVALUADA: Eltrombopag. DESCRIPCION DE LA INTERVENCIÓN: Eltrombopag (Revolade, GlaxoSmithKline) aumenta la producción de plaquetas activando el receptor de trombopoyetina, estimulando así la producción de plaquetas y reduciendo el sangrado. Eltrombopag tiene una autorización de comercialización de ANMAT para tratamiento de 'púrpura trombocitopénica inmune crónica (idiopática) (PTI) en pacientes de 1 año o más que son refractarios a otros tratamientos (p. ej. corticosteroides, inmunoglobulinas y esplenectomía). BUSQUEDA BIBLIOGRÁFICA: Se realizaron búsquedas en MEDLINE (desde 1950 hasta marzo de 2011), EMBASE (desde 1974 hasta marzo de 2019), y en el Registro Cochrane Central de Ensayos Controlados (Cochrane Central Register of Controlled Trials, CENTRAL) (Cochrane Library 2011, Número 3), para identificar todos los metaanálisis y ensayos con asignación aleatoria sobre la PTI crónica. RESULTADOS: Se incluyeron seis ensayos con 808 pacientes. Cinco estudios compararon los agonistas de receptores de TPO con placebo (romiplostim: 100, eltrombopag: 299, placebo: 175); un estudio comparó los agonistas de receptores de TPO con la atención estándar (AE) (romiplostim: 157; AE: 77). La AE incluyó una variedad de tratamientos, como glucocorticoide, inmunoglobulina anti-D, inmunoglobulina intravenosa, rituximab, azatioprina, etcétera. La supervivencia general, una de nuestras medidas de resultado primarias, no fue estudiada por estos ECAs y no fue posible calcular el número necesario a tratar (NNT). Otra medida de resultado primaria, la mejoría en los eventos de hemorragia significativa, no reveló ninguna diferencia significativa entre el grupo de agonistas de receptores de TPO y el grupo de control (placebo o AE) (versus cociente de riesgos [CR] del placebo 0,48, intervalo de confianza [IC] del 95%: 0,20 a 1,15; versus CR de la AE 0,49, IC del 95%: 0,15 a 1,63). En cuanto a las medidas de resultado secundarias, los agonistas de receptores de TPO mejoraron de forma estadísticamente significativa la respuesta plaquetaria general (versus CR del placebo 4,06, IC del 95%: 2,93 a 5,63; versus CR de la AE 1,81, IC del 95%: 1,37 a 2,37), la respuesta completa (versus CR del placebo 9,29; IC del 95%: 2,32 a 37,15) y la respuesta duradera (versus CR del placebo 14,16; IC del 95%: 2,91 a 69,01). Hubo una reducción significativa en los eventos de hemorragia general (grado 1 a 4 de la OMS) en comparación con el placebo (CR 0,78; IC del 95%: 0,68 a 0,89), pero no en comparación con la AE (CR 0,97; IC del 95%: 0,75 a 1,26). EXPLICACIONES: Funded by drug company. There is evidence that industry-sponsored trials may overestimatethetreatmenteffect(Bhandari 2004). Los intervalos de confianza son amplios e incluyen no efecto. Estudio que evaluó intervención con Romiplostin.


Subject(s)
Humans , Child, Preschool , Child , Adult , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Receptors, Thrombopoietin/therapeutic use , Technology Assessment, Biomedical , Cost-Benefit Analysis
12.
Journal of Experimental Hematology ; (6): 2039-2045, 2020.
Article in Chinese | WPRIM | ID: wpr-880012

ABSTRACT

OBJECTIVE@#To compare the effect of Sheng-Xue-Xiao-Ban Capsule (SXXBC) and indirubin to the peripheral platelets of the Idiopathic thrombocytopenic purpura (ITP) model mouse.@*METHODS@#The ITP mouse model was established by the method of passive immunization. SXXBC and indirubin were used for intervention treatment. Then the hemorrhagic phenomena of ITP mice were observed and the numbers of peripheral platelets, hemoglobin and white blood cells, bone marrow megakaryocytes and their classification and coagulation function were detected and compared.@*RESULTS@#The improvement rate of hemorrhage in SXXBC group was 40% for small dose, 60% for medium dose and 80% for high dose, while the improvement rate of hemorrhage in indirubin group was 30% for small dose, 50% for medium dose and 60% for high dose. There was no statistically significant difference in the improvement rate of hemorrhage between the two groups (P>0.05). Compared with the model control group, PLT and Hb increased in different doses of SXXBC and indirubin group 4th-8th day after drug intervention (P<0.05, 0.01). However, there was no significant difference between the different doses of SXXBC group and indirubin group (P>0.05). Compared with the model control group, the WBC in each group was significantly lower (P<0.05, 0.01) on the 4th-8th day after drug intervention; However, there was no statistical significance between the two groups of SXXBC and indirubin (P>0.05). Compared with the model control group, the total number of megakaryocytes in each treatment group were decreased (P<0.05, P<0.01), in which the number of primary megakaryocytes in the large and medium dose groups of SXXBC and indirubin were decreased (P<0.05, 0.01), and the number of juvenile megakaryocytes in the large dose group of SXXBC and indirubin were also decreased (P<0.05). The number of granular megakaryocytes were decreased in each intervention groups (P<0.05, 0.01), and the number of thromocytogenic megakaryocyte was increased in the high and medium dose groups of SXXBC and indirubin (P<0.01). The time of prothrombin was shortened in the high and medium dose groups of SXXBC and indirubin (P<0.05), and the fibrinogen (FIB) content in the high and medium dose groups of SXXBC was close to that of the normal control group.@*CONCLUSION@#Both of the SXXBC and the indirubin standard all show good hemostatic effects. Indirubin shows a positive effect on increasing the peripheral platelet and hemoglobin in ITP model mice, regulating the immune response, reducing the total number of bone marrow megakaryocytes, increasing the thromocytogenic megakaryocyte, and increasing coagulation function.


Subject(s)
Animals , Blood Platelets , Capsules , Indoles , Megakaryocytes , Mice , Purpura, Thrombocytopenic, Idiopathic/drug therapy
13.
Article in Chinese | WPRIM | ID: wpr-828122

ABSTRACT

Idiopathic thrombocytopenic purpura (ITP) is a common bloody disease with a high incidence in children, but its diagnostic method is exclusive diagnosis, and the existing detection techniques are mostly invasive, which may cause secondary injury to patients and also may increase the risk of disease. In order to make up for the lack of the detection method, this study made a preliminary exploration on the diagnosis of children's ITP from the perspective of infrared thermography. In this study, a total of 11 healthy children and 22 ITP children's frontal infrared thermal images were collected, and the pattern characteristic (PFD), average temperature (Troi) and maximum temperature (MAX) characteristics of 7 target areas were extracted. The weighted PFD parameters were correlated with the platelet count commonly used in clinical diagnosis, and the sensitivity and specificity of the weighted PFD parameters for children's ITP were calculated through the receiver operating characteristic curve (ROC). The final results showed that the difference of the weighted PFD parameters between healthy children and ITP children was statistically significant, and the parameters negatively correlated with platelet count. Under the ROC curve, the area under the curve (AUC) of this parameter is as high as 92.1%. Based on the research results of this paper, infrared thermography can clearly show the difference between ITP children and healthy children. It is hoped that the methods proposed in this paper can non-invasively and objectively describe the characteristics of ITP infrared thermal imaging of children, and provide a new ideas for ITP diagnosis.


Subject(s)
Area Under Curve , Child , Humans , Platelet Count , Purpura, Thrombocytopenic, Idiopathic , Thermography
14.
Arch. argent. pediatr ; 117(6): S243-S254, dic. 2019. Tab, graf
Article in Spanish | LILACS, BINACIS | ID: biblio-1051620

ABSTRACT

El manejo de la trombocitopenia inmune es motivo de discusión en lo concerniente a evolución,diagnóstico, pronóstico y tratamiento. Se han publicado arias guías que expresan distintas opiniones de expertos, pero no existe aún consenso mundial sobre cuál es el manejo más adecuado de la enfermedad. Esta guía establece los criterios para definir el diagnóstico; detalla el plan de estudios de laboratorio por realizar inicialmente; plantea los distintos diagnósticos diferenciales; desarrolla aspectos relativos a evolución y pronóstico, y enumera los tratamientos disponibles para las formas agudas y las crónicas, así como para el manejo de las emergencias y en algunas situaciones especiales.


Management, outcome, diagnosis, prognosis and treatment of immune thrombocytopenia are controversial. Several guidelines stating different experts' opinions have been published; however, no worldwide consensus regarding the management of the disease has still been reached. This guideline defines diagnostic criteria, states initial laboratory tests, establishes differential diagnosis, develops topics concerning outcome and prognosis, and enumerates available treatments for acute and chronic disease, as well as for management of life-threatening bleeding.


Subject(s)
Humans , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/therapy , Pediatrics
15.
Article in Chinese | WPRIM | ID: wpr-774339

ABSTRACT

OBJECTIVE@#To detect the levels of Treg, Th17, Th9 cells and expression of transforming growth factor-β (TGF-β), interleukin-17 (IL-17) and interleukin-9 (IL-9) in peripheral blood of patients with immune thrombocytopenia (ITP) and to explore its role in the pathogenesis of ITP.@*METHODS@#Fifty-four patients with ITP (ITP group) and 40 healthy volunteers (control group) were selected in our hospital. The of Treg, Th17 and Th9 cells in peripheral blood of 2 groups were measured by flow cytometry, and the expression of cytokines, such as TGF-β, IL-17 and IL-9 in the peripheral blood of 2 groups were detected by enzyme linked immunosorbent assay (ELISA).@*RESULTS@#The level of Treg cells in the peripheral blood of the ITP group was significantly decreased in comparison with the control group, while the levels of Th17 and Th9 cells significantly increased in comparison with the control group (all P<0.01). The expression of cytokine such as TGF-β in the peripheral blood of the case group significantly decreased in comparison with the control group, while the expression levels of IL-17 and IL-9 significantly increased in comparison with the control group (P<0.01). The results of Pearson correlation analysis showed that there was a positive correlation between the level of Treg cells and platelet count (PLT) in peripheral blood of the ITP group (r=0.35, P<0.05), and there were negative correlation between the level rate of Th17, Th9 cells and Plt count (r=-0.37, -0.43, P<0.05); there was a positive correlation between the expression of the TGF-β in the ITP group and Plt count (r=0.46, P<0.05), while the expression of IL-17 and IL-9 showed negative correlation with PLT (r=-0.48, -0.54, P<0.05).@*CONCLUSION@#The percentage of Treg, Th17 and Th9 cells in the peripheral blood of patients with ITP is abnormal, and the expression of TGF-β, IL-17 and IL-9 also is abnormal, which may play an important role in the pathogenesis of ITP.


Subject(s)
Flow Cytometry , Humans , Interleukin-17 , Interleukin-9 , Purpura, Thrombocytopenic, Idiopathic , T-Lymphocytes, Helper-Inducer , T-Lymphocytes, Regulatory , Transforming Growth Factor beta
16.
Laboratory Medicine Online ; : 224-231, 2019.
Article in English | WPRIM | ID: wpr-760514

ABSTRACT

MYH9-related disorders (MYH9RD) are autosomal-dominant disorders characterized by macrothrombocytopenia with or without leukocyte inclusion bodies or extra-hematological features, such as sensorineural deafness and renal impairment. MYH9RD can be misdiagnosed as an acquired form of thrombocytopenia including immune thrombocytopenic purpura (ITP). This leads to delayed diagnosis or administration of ineffective treatment. In the present study, we investigated the clinical and molecular characteristics of five unrelated Korean patients with MYH9RD and their family members, from four institutions. We reviewed clinical and laboratory data including extra-hematological manifestations. MYH9 pathogenic variants were detected by direct sequencing in all probands and the affected family members (N=10): two probands with c.5521G>A (p.Glu1841Lys) and one proband each with c.99G>T (p.Trp33Cys), c.287C>T (p.Ser96Leu), and c.3493C>T (p.Arg1165Cys). All patients had macrothrombocytopenia. Only the proband with Ser96Leu had extra-hematological manifestations. Past history revealed that two patients had been misdiagnosed with ITP and one of them had received a splenectomy. We validated the frequency of misdiagnosis (~20%) and genotype-phenotype correlations through a comprehensive review of previously reported cases of MYH9RD in Korea. It is important to suspect MYH9RD in patients with thrombocytopenia, and timely identification of macrothrombocytopenia and MYH9 pathogenic variants is required for early and accurate diagnosis of MYH9RD.


Subject(s)
Deafness , Delayed Diagnosis , Diagnosis , Diagnostic Errors , Genetic Association Studies , Humans , Inclusion Bodies , Korea , Leukocytes , Purpura, Thrombocytopenic, Idiopathic , Splenectomy , Thrombocytopenia
17.
Article in Korean | WPRIM | ID: wpr-763519

ABSTRACT

BACKGROUND: Immune thrombocytopenic purpura (ITP) in children less than one year of age is less well characterized compared to ITP in toddlers and school-age children. Since children of different ages may have differing clinical courses, better delineation of the natural history of ITP in infants is needed. METHODS: We retrospectively reviewed the admission records of 248 consecutive pediatric patients between 1 month and 15 years of age who were admitted and treated for acute ITP at Pusan National University Children's Hospital from 2009 through 2017. All patients less than 1 year of age were identified and enrolled in this study. We investigated their demographics, clinical features, laboratory examinations, response to treatment, and long-term outcomes and made a comparison to those of children aged 1 to 10 years of age. RESULTS: Ninety nine infants were identified. Male to female ratio was highest in infants and decreased with age. Seventy nine (79.8%) of the 99 infant were found to be under 6 months old. The median platelet counts at diagnosis was 6×10⁹/L. Minor bleeding (bleeding score 0–2) was significantly dominant in infant compared to older subjects. Eighty two (96.5%) out of 85 patients achieved complete remission after initial intravenous immunoglobulin (IVIG) treatment. The relapse rate after initial CR was significantly lower than older ages (P=0.003). The platelet count after IVIG treatment in infant showed more rapid response compared to older subjects (P=0.04). Follow up information at 12 months was available for 70 infants. Chronic ITP at 12 month was seen less frequently in infants than in children 1 to 10 years of age (1.4% vs. 20.2%, P<0.001). CONCLUSION: Infants with acute ITP respond more favorably to IVIG treatment and are less likely to develop chronic ITP compared to children 1 to 10 years of age.


Subject(s)
Child , Demography , Diagnosis , Female , Follow-Up Studies , Hemorrhage , Humans , Immunoglobulins , Immunoglobulins, Intravenous , Infant , Male , Natural History , Platelet Count , Purpura, Thrombocytopenic, Idiopathic , Recurrence , Retrospective Studies
18.
Journal of Experimental Hematology ; (6): 1949-1954, 2019.
Article in Chinese | WPRIM | ID: wpr-781513

ABSTRACT

OBJECTIVE@#To investigate the changes of mean platelet volume (MPV), platelet distribution width (PDW) and platelet associated antibodies (PAIg) in children with acute immune thrombocytopenic purpura (aITP), and to explore the diagnostic value of MPV, PDW, PAIg and their combination for megakaryocyte dysmaturity in aITP children.@*METHODS@#Plt count, MPV and PDW of 36 aITP children were measured by using Sysmex XN automatic blood cell analyzer, and 33 children with acquired thrombocytopenic purpura (ATP) without megakaryocyte dysmaturity. The expression of PAIg was detected by flow cytometry, and the number and classification of megakaryocytes in the bone marrow were performed by marrow cytology. The diagnostic significances of MPV, PDW, PAIg and their combination as well as the sensitivity and specificity for megakaryocytes dysmaturity in aITP were assessed through calculating the area under ROC curve (AUC), after determining the influence of each parameters on the megakaryocyte dysmaturity by Logistic regression.@*RESULTS@#MPV, PDW and PAIg of aITP children were significantly higher than those of the ATP children (P<0.05), while the Plt count and number of thromocytogenic megakaryocytes per area (1.5 cm×3 cm) were less than those of the controls (P<0.05). Count of RBC and WBC, percentages of neutrophil granulocytes and lymphocydes in aITP were similar to those in the controls(P>0.05). The results of Logistic regression showed that Plt count, MPV, PDW and PAIg were the factors influencing megakaryocyte dysmaturity in aITP children, and the regression model has a high statistical significance (χ=65.491,P=0.001) and r square (R=0.713). The AUC of the combined detection of Plt count, MPV, PDW and PAIg was 0.863, which was much higher than that of Plt count, MPV, PDW, PAIg individually or in pairs. The sensitivity and specificity of the combined detection were 79.167% and 89.697%, which were higher than those of Plt count, MPV, PDW, PAIg individually or in pairs.@*CONCLUSION@#The diagnostic significance of MPV and PDW for megakaryocyte dysmaturity in aITP are insufficient, but the diagnostic efficacy can be improved by combined examination with PAIg.


Subject(s)
Antibodies , Blood Platelets , Child , Humans , Mean Platelet Volume , Megakaryocytes , Platelet Count , Purpura, Thrombocytopenic, Idiopathic , Diagnosis
19.
Journal of Experimental Hematology ; (6): 1955-1961, 2019.
Article in Chinese | WPRIM | ID: wpr-781512

ABSTRACT

OBJECTIVE@#To establish a flow cytometric immunobead array assay (FCIA) to quantify platelet antibodies and to explore its application in the diagnosis and treatment of ITP.@*METHODS@#The guantitative standard curve was established by binding the human IgG of known concentration on antibody-coated microbeads; at the same time, the platelet-specific antigen and antibody complex was captured and levels of platelet antibodies were detected using the microbeads coated by 5 kinds of antibodies against platelets suca as GPIX (SZ1), GPⅠb (SZ2), GpⅢa (SZ21), GPⅡb (SZ22) and p-selection (SZ51). The fluorescence signal detected by flow cytometry were transformed into the conentration of platelet antibodies in samples through the quantitative standard curve, thereby establishing the method for quantititive detection of platelet-specific antibodies in plasm samples (FCIA), moreover the property, efficiency and clinical application of establishod FCIA method were evaluated.@*RESULTS@#The FCIA could detect 5 kinds of antibodies against GPIX, GPⅠb, GpⅢa, GPⅡb and β-selection within a broad range of 33.29-1280 ng/ml, 45.17-1280 ng/ml, 42.07-1280 ng/ml, 46.40-1280 ng/ml, 42.48-1280 ng/ml and 42.48-1280 ng/ml respectively, and their recovery rates were 115.23%, 112.58%, 117.47%, 107.64% and 112.67% respectively. The intra-assay coefficient of variation (CV) for anti- GPIX, -GPⅠb, -GpⅢa, -GPⅡb and p-selection antibodies was 3.54%, 3.63%, 4.66%, 6.43% and 6.67% respectively, and the inter-assay CV for above mentioned antibodies were 10.89%, 7.57%, 10.34%, 6.95% and 10.72% respectively. The detection showed that the levels of 5 kinds of platelet-specific antibodies in ITP group all were higher than those in non-ITP and healthy control groups (P<0.01). The sensitivity, specificity and accuracy of quantitatively detecting 5 kinds of antibodies for diagnosis of ITP by FCIA were 68.29%, 84.98% and 78.95% respectively, while the sensitivity, specificity and accuracy of detecting 5 kinds of antibodies by modified indirect MAIPA were 41.46%, 90.41% and 72.81% respectively.@*CONCLUSION@#The established quantitative FCIA for detection of antibodies provides a powerful tool for diaghosis and evaluation of therapeutic efficacy and prognosis of ITP patients.


Subject(s)
Antibodies , Autoantibodies , Blood Platelets , Flow Cytometry , Humans , Purpura, Thrombocytopenic, Idiopathic
20.
Journal of Experimental Hematology ; (6): 1962-1965, 2019.
Article in Chinese | WPRIM | ID: wpr-781511

ABSTRACT

OBJECTIVE@#To study the changes of Th1 and Th2 type cytokines and B lymphocyte level and their clinical significance in idiopathic thrombocytopenic purpura (ITP) patients treated by recombinant human thrombopoietin (rhTPO).@*METHODS@#The peripheral blood levels of Th1 and Th2 type of cytokines and B lymphocyte were estimated by CBA in 48 patients with ITP, and compared with those in 35 control persons of heath examination.@*RESULTS@#Before treatment, the levels of Th1 type cytokines and B lymphocyte in 48 patients with ITP were higher, and the levels of Th2 type cytokines were lower than those of healthy controls (P<0.05). The levels of the peripheral blood CD19 cells, CD5CD19 cells, IL-2 expression negatively correlated with Plt counts in ITP patients (P<0.05), the levels of IL-4 positively correlated with Plt counts (P<0.05). After treatment with rhTPO, the levels of Th1 type cytokines and B lymphocytes in 48 patients with ITP significantly decreased, and the levels of Th2 type cytokines significantly increased in comparison with those before treatment (P<0.05).@*CONCLUSION@#Peripheral blood Th1 and Th2 type cytokines express abnormally and level of B lymphocytes increases significantly in ITP patinets. The disease severity correlats with the levels of Th1 and Th2 type cytokines and B lymphocytes. Platelets increase after rhTPO treatment, showing that rhTPO can play an important role in regulating Th1 and Th2 immunologic balance and B lymphocyte level in ITP patients.


Subject(s)
B-Lymphocytes , Humans , Purpura, Thrombocytopenic, Idiopathic , Th1 Cells , Th2 Cells , Thrombopoietin
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