The Treatment Options and Clinical Significance of Immune Thrombocytopenia Patients with Splanchnic Vein Thrombosis as the Initial Manifestation / 中国实验血液学杂志

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.

Cambio de diagnóstico y de categoría diagnóstica en pacientes con artritis idiopática juvenil en 7 años de seguimiento / Change of diagnosis and diagnostic category in patients with juvenile idiopathic arthritis within 7 years of follow-up

Resumen: Introducción: Al menos 50% de los pacientes pediátricos portadores de artritis idiopática juvenil (AIJ) continuará control en reumatología adulto. La clasificación de la Liga Internacional de Asociaciones de Reumatología (ILAR) vigente, actualmente en revisión, difiere de la clasificación de las artritis inflamatorias del adulto. Se ha reportado cambios de categoría en 10,8% de los pacientes durante el seguimiento. Objetivo: Analizar los pacientes con AIJ seguidos al menos 7 años para objetivar cambios de diagnós tico en la transición, e identificar factores de mal pronóstico funcional. Pacientes y Método: Estudio retrospectivo en base a registros clínicos. Se incluyó a la totalidad de los pacientes con AIJ controla dos en policlínico pediátrico del Hospital de Puerto Montt entre el año 2005 y 2017, que cumplieron siete o más años de seguimiento. Se realizó análisis descriptivo en base a variables clínicas: categoría diagnóstica, tiempo de evolución al diagnóstico, actividad clínica y serológica, y tiempo de evolución al inicio de la terapia farmacológica. Resultados: Se evaluaron 18 pacientes, 3 Oligo-articular (OA) persistente, 1 OA extendida, 4 Poli-articular (PA) factor reumatoide (FR) negativo, 4 PA FR positivo, 5 Sistémicas, 1 Psoriática, todos con seguimiento mayor a 7 años. Once de 18 niños fueron transfe ridos a adultos. Tres de 11 cambiaron de diagnóstico a Artritis Reumatoide (AR) más otra enferme dad autoinmune: Síndrome de Sjögren + Lupus eritematoso sistémico, Púrpura trombocitopénico inmune, Enfermedad autoinmune no clasificada y cinco de 11 niños de categoría ILAR: OA a Artritis reumatoide juvenil, OA extendida a PA FR negativo, 3 Sistémicas a PA FR negativo. Edad de inicio, formas poli-articulares, retrasos en diagnóstico y comienzo de terapia se asociaron a secuelas e infla mación persistente. Conclusiones: Ocho de once pacientes transferidos cambiaron denominación diagnóstica y/o presentaron otras enfermedades autoinmunes. Algunos factores de mal pronóstico deben mejorar.

Abstract: Introduction: At least 50% of pediatric patients with Juvenile Idiopathic Arthritis (JIA) will require continued fo llow-up in adult rheumatology. The present International League of Associations for Rheumatology (ILAR) classification, currently under revision, differs from its classification of inflammatory arthritis in adults. Category changes have been reported in 10.8% of patients during follow-up. Objective: To analyze JIA patients in follow-up for at least 7 years to detect diagnosis changes during transition to adult care, identifying factors of poor functional prognosis. Patients and Method: Retrospective study based on medical records of JIA patients seen at the pediatric polyclinic of the Puerto Montt Hospital between 2005 and 2017, who were monitored for at least 7 years. Descriptive analysis was performed according to clinical variables: diagnostic category, evolution before diagnosis, clinical and serological activity, and evolution before starting drug therapy. Results: We evaluated 18 pa tients, corresponding to 3 patients with persistent oligoarticular arthritis (OA), 1 with extended OA, 4 with polyarticular arthritis (PA) rheumatoid factor (RF) negative, 4 with PA RF positive, 5 with syste mic JIA, and 1 with psoriatic arthritis, all have had follow-up more than 7 years. 11 out of 18 patients transitioned to adult care. Three out of 11 patients changed diagnosis to Rheumatoid Arthritis (RA) plus another autoimmune disease such as Sjögren's Syndrome + Systemic Lupus Erythematosus, Immune thrombocytopenia, or unclassified autoimmune disease, and 5 out of 11 children changed ILAR category from OA to Juvenile Rheumatoid Arthritis, extended OA to PA RF negative, and 3 from Systemic arthritis to PA RF negative. Age of onset, polyarticular forms, delay in diagnosis, and the start of therapy were associated with sequelae and persistent inflammation. Conclusions: Eight of the eleven JIA patients who transitioned to adult care changed their diagnosis or presented other autoimmune diseases. Some factors of poor prognosis must improve.

Idiopathic thrombocytopenic purpura in a patient with situs inversus totalis: case report and literature review / Púrpura trombocitopênica idiopática em paciente com situs inversus totalis: relato de caso e revisão da literatura

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.

Peliosis hepatis presenting with massive hepatomegaly in a patient with idiopathic thrombocytopenic purpura

Peliosis hepatis is a rare condition that can cause hepatic hemorrhage, rupture, and ultimately liver failure. Several authors have reported that peliosis hepatis develops in association with chronic wasting disease or prolonged use of anabolic steroids or oral contraceptives. In this report we describe a case in which discontinuation of steroid therapy improved the condition of a patient with peliosis hepatis. Our patient was a 64-year-old woman with a history of long-term steroid treatment for idiopathic thrombocytopenic purpura . Her symptoms included abdominal pain and weight loss; the only finding of a physical examination was hepatomegaly. We performed computed tomography (CT) and magnetic resonance imaging (MRI) of the liver and a liver biopsy. Based on these findings plus clinical observations, she was diagnosed with peliosis hepatis and her steroid treatment was terminated. The patient recovered completely 3 months after steroid discontinuation, and remained stable over the following 6 months.

Cardiac surgery in a patient with immunological thrombocytopenic purpura: Complications and precautions.

Immune thrombocytopenic purpura (ITP) patients are at high-risk for bleeding complications during and after cardiac surgeries involving cardiopulmonary bypass. We report a patient with ITP with severe coronary artery disease and mitral valve regurgitation who underwent uncomplicated coronary artery bypass grafting and mitral valve replacement. Three weeks later, the patient was readmitted in a very low general condition with signs of pericardial tamponade. We describe our experience of managing the case.

Hyperglycemic Hyperosmolar Syndrome Caused by Steroid Therapy in a Patient with Lupus Nephritis

A 51-yr-old female was referred to our outpatient clinic for the evaluation of generalized edema. She had been diagnosed with idiopathic thrombocytopenic purpura (ITP). She had taken no medicine. Except for the ITP, she had no history of systemic disease. She was diagnosed with systemic lupus erythematosus. Immunosuppressions consisting of high-dose steroid were started. When preparing the patient for discharge, a generalized myoclonic seizure occurred at the 47th day of admission. At that time, the laboratory and neurology studies showed hyperglycemic hyperosmolar syndrome. Brain MRI and EEG showed brain atrophy without other lesion. The seizure stopped after the blood sugar and serum osmolarity declined below the upper normal limit. The patient became asymptomatic and she was discharged 10 weeks after admission under maintenance therapy with prednisolone, insulin glargine and nateglinide. The patient remained asymptomatic under maintenance therapy with deflazacort and without insulin or medication for blood sugar control.

Asociación de la púrpura trombocitopénica inmunológica y el síndrome de Guillain-Barré: a propósito de un caso / Association of immunologic thrombocytopenia and the Guillain-Barré syndrome: with regard to a case

La púrpura trombocitopénica inmunológica es una enfermedad autoinmune, benigna, de aparición frecuente, caracterizada por la presencia de anticuerpos dirigidos contra las glicoproteínas de la membrana plaquetaria que producen una disminución del recuento plaquetario y manifestaciones hemorrágicas cutáneo-mucosas. El diagnóstico de esta entidad se realiza por exclusión de otras causas de trombocitopenia. El síndrome de Guillain-Barré es también una enfermedad de naturaleza autoinmune donde la pérdida de la tolerancia inmunológica trae como consecuencia la aparición de anticuerpos dirigidos contra los gangliósidos de los nervios periféricos. Se presenta una paciente femenina de 40 años con diagnóstico de una púrpura trombocitopénica inmunológica crónica que comenzó con una parálisis motora ascendente, sin toma respiratoria, parálisis facial y dolor intenso en las regiones dorsal y lumbar. Fue diagnosticada como un síndrome de Guillain-Barré e inmediatamente se comenzó tratamiento con vitaminoterapia y esteroides a altas dosis. Después de varios meses de seguimiento y rehabilitación presentó una evolución satisfactoria con remisión de todos los síntomas neurológicos

The immunologic thrombocytopenic purpura is an autoimmune, benign, of frequent appearance disease characterized by the presence of antibodies directed to glycoproteins of platelet membrane producing a decrease of platelet count and cutaneous-mucosal hemorrhagic manifestations. The Guillain-BarrÚ syndrome is also a disease autoimmune by origin where the loss of immunological tolerance causes the appearance of antibodies directed to gangliosides of peripheral nerves. This is the case of female patient aged 40 diagnosed with a chronic immunologic thrombocytopenic purpura beginning with an ascendant motor paralysis, without respiratory compromise, facial paralysis and intense pain in dorsal and lumbar regions and also a diagnosis of Guillain-BarrÚ syndrome with immediate treatment based on vitamin-therapy and high dose of steroids. After some months of follow-up and rehabilitation there was a satisfactory evolution with remission of all neurological symptoms

Angioplastia coronária em pacientes com infarto do miocárdio e púrpura trombocitopênica idiopática / Coronary angioplasty in a patient with myocardial infarction and idiopathic thrombocytopenic purpura

A púrpura trombocitopênica idiopática (PTI) é um distúrbio autoimune, caracterizado clinicamente por plaquetopenia e sangramentos mucocutâneos. Trata-se de doença rara na população geral e a ocorrência de infarto agudo do miocárdio (IAM) em pacientes com PTI é ainda menos frequente. Neste artigo os autores descrevem um caso com PTI no qual foi praticada angioplastia coronária tranbsluminal na fase evolutiva do IAM.

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by the presence of thrombocytopenia and mucocutaneous bleeding. It is a rare condition and the occurrence of an acute myocardial infarction (AMI) in patients with ITP is even less common. In the present manuscript the authors report a patient with ITP who underwent percutaneous transluminal coronary angioplasty in the follow-up phase of an AMI.

Púrpura trombocitopénico inmunológico (PTI): a propósito de un caso / Idiopathic thrombocytopenic purpura: a case report

Paciente de sexo masculino de 22 años que ingresa por cuadro de inicio agudo de hemorragia muco cutánea. Al ingreso se pesquisa severa trombocitopenia asociada a anemia normocítica. Se realiza estudio, concluyéndose diagnóstico de Purpura trombocitopénico inmunológico y anemia secundaria at sangrado concomitante. Se inicia tratamiento con corticoides 1 mg/kg/día, con mala respuesta a los 15 días de tratamiento, por lo que se realiza esplenectomía laparoscópica con respuesta favorable.

Spontaneous bilateral peripapillary, subhyaloid and vitreous hemorrhage with severe anemia secondary to idiopathic thrombocytopenic purpura.

A 42-year-old female presented to us with a complaint of sudden painless loss of vision in both eyes of three days duration. Visual acuity was 20/100 for distance in both eyes. Fundus examination showed bilateral peripapillary hemorrhages, with subhyaloid and vitreous hemorrhage in both eyes. Hematological investigations revealed hemoglobin (HB 7 gm %) and severe thrombocytopenia (12,000/ ul). She was referred to a hematologist where a diagnosis of idiopathic thrombocytopenic purpura (ITP) was made. She was treated for systemic condition with regular ophthalmic follow-up. Over the next nine months, retinal hemorrhages completely resolved and the patient regained her visual acuity. The purpose of this case report is to highlight the clinical presentation of severe anemia, which is different from previous reports and the role of an ophthalmologist in first detecting the Idiopathic thrombocytopenic purpura (ITP), which led to successful recovery.

Púrpura trombocitopénica inmunitaria. Guía de diagnóstico y tratamiento / Guideline for diagnosis and treatment of immune thrombocytopenia

El manejo de la púrpura trombocitopénica inmunitariaes motivo de discusión en lo concerniente a evolución, diagnóstico, pronóstico y tratamiento.Se han publicado varias guías con 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 a realizar inicialmente, plantealos distintos diagnósticos diferenciales, desarrolla aspectos relativos a evolución y pronóstico, y enumeralos tratamientos disponibles para las formas agudas y las crónicas, así como para el manejo de las emergencias.

Outcome of Immunosuppressive Therapy with Helicobacter pylori Eradication Therapy in Patients with Chronic Idiopathic Thrombocytopenic Purpura

We initiated this study to investigate whether combining Helicobacter pylori eradication with immunosuppressive therapy provides an additional benefit to patients with idiopathic thrombocytopenic purpura (ITP) that has relapsed or has not responded to steroid and/or danazol therapy in patients who have H. pylori infection. Thirty- four patients with chronic ITP that had relapsed or failed to steroid and/or danazol therapy were assessed for H. pylori infection. Of the 21 confirmed cases, 12 patients were given H. pylori eradication therapy alone (EA), while 9 patients received eradication therapy combined with immunosuppressive therapy (EI). The response rate was not significantly different between patients in the EA and those in the EI group (41.7% in the EA group vs. 66.7% in the EI group, p=0.345). The median platelet count at 6 months after therapy was higher in the EI group patients (75X10(9)/L in the EI group patients vs. 18x109/L in the EA group patients, p=0.028). The median response duration was also longer in the EI group patients (9 months in the EI group patients vs. 3 months in the EA group patients, p=0.049). These results show that a significant benefit is gained by the use of H. pylori eradication combined with immunosuppressive therapy over the use of eradication therapy alone for patients with chronic ITP.

Trombocitopenia y embarazo / Thrombocytopenia and pregnancy

La trombocitopenia es la causa más común de sangrado en la población general, siendo también la principal causa de alteraciones de la coagulación en las pacientes obstétricas, observándose en aproximadamente el 10% de ellas. Son múltiples las causas de esta alteración, dentro de las cuales las más comunes son: trombocitopenia gestacional, trombocitopenia relacionada a preeclampsia y a enfermedades autoinmunes. La complicación anestesiológica más importante relacionada con la trombocitopenia es la producción de un hematoma peridural secundario a una anestesia neuroaxial. El objetivo de esta revisión es dar a conocer las diversas causas de trombocitopenia en pacientes embarazadas y su enfrentamiento desde el punto de vista anestesiológico.

Thrombocytopenia is the most common cause of bleeding in the general population, being also the principal cause of coagulation deficits in obstetric patients, with an incidence of 10% in this population. The most common ethiologies are: gestational thrombocytopenia, preeclampsia and autoimmune disease related thrombocytopenia. The most important anesthesiological complication in relation to thrombocytopenia is the formation of an epidural hematoma secondary to a neuraxial puncture. The objective is to review the different kinds of thrombocytopenia during pregnancy and the anesthesiological approach.

Características clínicas del púrpura trombocitopénico inmune: revisión de 52 casos / Clinical characteristics of inmune thrombocytopenic purpura

Introducción: El Púrpura Trombocitopénico Inmune (PTI) suele ser autolimitado pero 10-20 por ciento persisten a los 6 meses, es decir de evolución crónica. Su tratamiento es controvertido y existen pocos datos nacionales. Objetivo: Conocer algunas características clínicas y de laboratorio del PTI, su relación con evolución crónica y el manejo actual. Método: Estudio retrospectivo de los 52 pacientes con PTI evaluados en Hospital Luis Calvo Mackenna, entre marzo 1998 y febrero 2003. Se consignó: sexo, edad, manifestaciones clínicas, conducta terapéutica y recuento plaquetario (RP) al diagnóstico, 15-60 días y 6 meses. Se aplicaron Test de Fisher y Odd Ratio. Resultados: Mediana de edad: 4,4 años (0,7 a 16,1), el RP fue < - 20 000 x mm3 en 37/52. No hubo hemorragia del sistema nervioso central. Se manejó con observación clínica 34/52, corticoides 17/52 e inmunoglobulinas endovenosas con corticoides 1/52. Completaron control (6 meses) 48/52 pacientes. Presentaron curso crónico 11/48, asociado a RP 15 días < - 20 000 x mm3 (p = 0,01) OR = 9 (IC95 por ciento: 1,26-80,16) y RP a los 60 días < - 50 000 x mm3 (p = 0,0000003) OR= 124 (IC95 por ciento: 7,77 - 4951,52). Conclusiones: La mayoría de los pacientes requirieron sólo observación clínica. Presentaron evolución crónica 23 por ciento siendo factores de riesgo RP a los 15 días £ 20 000 x mm3 y RP a los 60 días £ 50 000 x mm3.

Primary Percutaneous Coronary Intervention for Acute Myocardial Infarction with Idiopathic Thrombocytopenic Purpura: A Case Report

Acute myocardial infarction (AMI) is rare in patients with idiopathic thrombocytopenic purpura (ITP). We describe a case of an AMI during thrombocytopenia in a patient with chronic ITP. A 47-yr-old woman presented with anterior chest pain and a low platelet count (21,000/microliter) at admission. Urgent coronary angiography revealed total occlusion of proximal right coronary artery and primary percutaneous coronary intervention (PCI) was performed successfully. This case suggests that primary PCI may be a therapeutic option for an AMI in patients with ITP, even though the patient had severe thrombocytopenia.

Idiopathic thrombocytopenic purpura complicating pregnancy.

Idiopathic thrombocytopenic purpura is a relatively common but unrecognised auto-immune disorder among women of child bearing age. Nearly one-third of patients present with bleeding tendency in pregnancy. Management necessitates care of mother during pregnancy and delivery and care of the baby. Steroids remain the chief, economical and effective method of raising platelet counts, but platelet transfusion and human intravenous high dose alpha-globulin may be required. Neonatal thrombocytopenia must be expected and managed. Management of patient, since it concerns two lives, should be individualised and carefully planned in consultation with haematologist and paediatrician.

Angioplastia transluminal coronariana em portador de púrpura trombocitopênica idiopática / Percutaneous transluminal coronary angioplasty in a patient with idiopathic thrombocytopenic purpura

A associação entre a doença arterial coronariana e a púrpura trombocitopênica é rara e implica em algumas dificuldades quando a revascularização se faz necessária. Relatamos um caso dessa associação, com importante comprometimento coronariano, tratado percutaneamente com o implante de stent.