Immune thrombocytopenic purpura following CoronaVac vaccination: a case report

Immune thrombocytopenic purpura (ITP), an autoimmune disorder, has been documented as a result of SARS-CoV-2 infection and a vaccination side effect. The COVID-19 pandemic has led to the creation of CoronaVac vaccine and has been widely administered in Brazil.Patient, in the case, is an 82-years-old female who received the vaccine two days before an acute episode of gingivorrhagia and diffuse cutaneous petechiae. Other exams were made to look for other causes of secondary thrombocytopenia and all the results were normal. The patient showed improvement on the platelet levels three day after the beginning of the treatment with high dosage methylprednisolone.Knowing that other kinds of vaccine can generate ITP, the SARS-CoV-2 vaccine could be related to the symptoms.

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.

Trombocitopenia inmune. Guía de diagnóstico y tratamiento / Guideline for diagnosis and treatment of immune thrombocytopenia

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.

Application of vincristine-loaded platelet therapy in three dogs with refractory immune-mediated thrombocytopenia

Three dogs presented with refractory immune-mediated thrombocytopenia (IMT). All patients failed to respond to prednisone, which is considered a mainstay of immunosuppressive therapy. Vincristine-loaded platelets (VLPs), which act selectively on mononuclear phagocytes,were introduced. After the VLPs were transfused, two dogs responded quickly withimproved clinical signs while the third dogwith recurrent IMT was euthanized due to its deteriorating condition. This case report describesthe efficacy of VLP therapy in refractory IMT patients.

Características clínicas, epidemiológicas y de tratamiento de niños con púrpura trombocitopénica inmune, hospitalizados en el Instituto Nacional de Salud del Niño. Año 2011-2012 / Clinical characteristics, epidemiological and treatment of the children with immune thrombocytopenic purpura, hospitalized at the National Institute of Child Health. Year 2011-2012

O Describir las características clínicas, epidemiológicas y de tratamiento de niños con Púrpura Trombocitopénica Inmune, hospitalizados en el Instituto Nacional de Salud del Niño durante el periodo 2011-2012. Metodología: Estudio observacional, descriptivo, retrospectivo. Se realizó un muestreo no probabilístico por conveniencia, conformada por 54 niños hospitalizadas con el diagnóstico de Púrpura Trombocitopénica Inmune en Instituto Nacional de Salud del Niño durante el año 2011 a 2012. Para describir las variables cualitativas se usó frecuencias absolutas y porcentajes. Resultados: Del estudio entre las características clínicas el 55.6 por ciento de los pacientes presentó púrpura trombocitopénica inmune de tipo aguda, siendo el inicio de la enfermedad principalmente insidiosa (88.9 por ciento), de curso progresivo (81.5 por ciento), con menos de 10 días de duración (70.4 por ciento). El 27.8 por ciento de la aparición de la enfermedad se dio entre los meses de octubre a diciembre, el 64.8 por ciento tuvo enfermedades previas. Las manifestaciones clínicas más frecuentes fueron petequias de color rojo (85.2 por ciento), la equimosis superficial (61.1 por ciento), la epistaxis (53.7 por ciento) y la hemorragia nasal (51.9 por ciento). Más de la mitad de pacientes tuvieron 2 episodios de hemorragia. Respecto a las pruebas de diagnóstico, más de la tres cuartas partes presentó menos de 12 gr/dl de hemoglobina la cuarta parte de su tiempo de sangría fue menor a 5 minutos, 79.6 por ciento tuvo un tiempo de protombina de 13 seg. a más. Asimismo, al 72.2 por ciento de los pacientes su tiempo de tromboplastina fue entre 36 seg. a más, la cuarta parte presento un tiempo de coagulación menor a 7 min y el 51.9 por ciento de pacientes tuvieron valores de 200 a 400 mg/dl de fibrinógeno. Al 14.8 por ciento de pacientes que se le realizó la prueba de velocidad corpuscular media tuvo valores superiores a 25 mm/hora. Con respecto al recuento plaquetario...

To describe the clinical, epidemiological and treatment characteristics of children with immune thrombocytopenic purpura, hospitalized at the National Institute ofChild Health during the period 2011-2012. Methodology: Observational, descriptive, retrospective study. It was performed a non-probability convenience sample, consisting of 54 children hospitalized with the diagnosis of Immune Thrombocytopenic Purpura in National Institute of Child Health during 2011-2012. To describe qualitative variables, absolute frequencies and percentages were used. Results: Among clinical features, 55.6 per cent of patients had immune thrombocytopenic purpura type acute, being the beginning of the disease mainly insidious (88.9 per cent), progressive course (81.5 per cent), less than 10 days (70.4 per cent). The 27.8 per cent of the onset of illness occurred between the months of October to December, 64.8 per cent had previous illnesses. The most frequent clinical manifestations were red petechiae (85.2 per cent), superficial ecchymosis (61.1 per cent), epistaxis (53.7 per cent) and nasal hemorrhage (51.9 per cent). More than half had 2 episodes of bleeding. Regarding diagnostic tests, more than three quarters had less than 12 g/dl of hemoglobin, the fourth bleeding time was less than 5 minutes, and 79.6 per cent had a prothrombin time of 13 sec more. Also, 72.2 per cent of patients, thromboplastin time was between 36 sec more, a quarter presented a clotting time less than 7 min and 51.9 per cent of patients had values of 200 to 400 mg/dl of fibrinogen. In 14.8 per cent of patients whom performed the mean corpuscular velocity test had values above 25 mm/hour. Respect to platelet count, 44.5 per cent had between 10000 and 20000 platelets/mrn3. Among the treatments received, 1.8 per cent had expectant treatment. Of those who received no expectant treatment, 1.9 per cent was applied anti-D immunoglobulin, 61.1 per cent and 73.6 per cent of patients were administered...

Octaploidy in idiopathic thrombocytopenic purpura.

We report a case of an elderly 68-year-old male who presented in our hospital with chief complaints of petechial rashes and ecchymosis over extremities and bleeding from the oral cavity since 3–4 days prior to hospitalization. He saw a physician before coming to our hospital and received one dose of IV methylprednisolone and oral wysolone. He had come to our hospital for further management. Bone marrow karyotyping was done and chromosomal analysis revealed two cell lines. Eighty percent of the cells analyzed revealed apparently normal male karyotype. However, 20% cells analyzed revealed a total of 184 chromosomes, suggesting octaploidy.

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.

Idiopathic thrombocytopenic purpura and oral surgery: case report / Púrpura trombocitopênica idiopática e cirurgia bucal: relato de caso

OBJECTIVES: To review the function of platelets in the blood clotting mechanism. To address,conceptualize and classify thrombocytopenic purpura, especially idiopathic thrombocytopenic purpura (ITP),emphasizing the immunological aspects involved in the etiology of the disease. To address the clinicalmanifestations of the disease and the appropriate therapy. To present the case study of a patient with ITPwho requires oral surgical intervention. DISCUSSION: Leukopenia in ITP can be subclinical. The firstmanifestation of the disease can be severe hemorrhaging due to small lacerations or minor medical anddental surgical procedures. The cause of the platelet reduction is idiopathic; an autoimmune reaction in whichthe antibodies destroy the platelets appears to participate in the process. A variety of situations can occur,leading to mild to severe thrombocytopenia. One frequent aspect is the instability of the platelet count,which oscillates inconsistently and may be related to infections and other factors that have not been clearlydetermined, including stress. In the case presented here, the leukopenia in the patient was mild in presentation.However, it was decided that the patient should be referred to a hematologist for preparation and clearance.Oral surgery that compromises the bone, such as exodontia, can present significant difficulties in localizingand clamping intraosseous vessels, which does not only occur in small soft tissue surgeries. CONCLUSIONS:Platelet destruction in ITP occurs from a complex process that is comprised of multiple components of theimmune system. The platelets are prematurely destroyed by antibodies that are aimed at the platelet glycoprotein,which can results in serious, even fatal consequences. It is important to emphasize the significance of themedical history and the appropriate physical examination during the diagnostic process, as well as collaborationwith the patient’s medical...

OBJETIVOS: Revisar a função das plaquetas no mecanismo de coagulação sanguínea. Conceituare classificar a púrpura trombocitopênica, especialmente a púrpura idiopática, enfatizando osaspectos imunológicos envolvidos na etiologia da doença. Enfocar as manifestações clínicas dadoença e a terapia apropriada. Apresentar um caso de manifestação clínica da doença e a terapiaapropriada. Apresentar o caso de uma paciente com púrpura que necessitava de intervençãocirúrgica bucal. DISCUSSÃO: A leucopenia na púrpura trombocitopênica idiopática pode sersubclínica. A primeira manifestação da doença pode ser uma hemorragia severa por causa depequenas lacerações ou pequenos procedimentos médicos e odontológicos. A causa da reduçãodas plaquetas é idiopática; uma reação autoimune na qual os anticorpos destroem as plaquetasparece participar do processo. Uma variedade de situações pode ocorrer, levando àtrombocitopenia discreta a severa. Um aspecto frequente é a instabilidade da contagem plaquetária,que oscila inconsistentemente e pode relacionar-se com infecções e outros fatores ainda nãoclaramente determinados, incluindo estresse. No presente caso, o paciente apresentava discretaleucopenia. Entretanto, decidiu-se que o paciente deveria ser avaliado por um hematologista,para preparação e liberação para cirurgia, pois a cirurgia bucal que compromete osso, como aexodontia, pode apresentar dificuldades significativas na localização e pinçamento de vasosintraósseos, o que não acontece em pequenas cirurgias de tecidos moles. CONCLUSÕES: Adestruição plaquetária na púrpura trombocitopênica ocorre por meio de um complexo processo,com múltipos componentes do sistema imune. As plaquetas são destruídas prematuramente poranticorpos dirigidos contra as glicoproteínas plaquetárias, o que pode resultar em sérias – emesmo fatais – consequências. É importante enfatizar o significado da história médica e o examefísico adequado no processo diagnóstico...

Púrpura trombocitopênica imune: diagnóstico e tratamento / Immune thrombocytopenic purpura: diagnosis and treatment

Objetivo: revisar os critérios vigentes para o diagnóstico e o tratamento da púrpura trombocitopênica imune da criança. Fonte dos dados: foram selecionados os artigos da literatura da base MedLine mais relevantes dos últimos 20 anos relativos ao termo púrpura trombocitopênica...

Objective: to assess the current criteria for immune thrombocytopenic purpura (PTI) diagnosis and tretament. Data source: MedLine database articles published in the last twenty years selecte upon the therm thrombocytopenic purpura...

Retrospective analysis of patients with Idiopathic thrombocytopenic purpura from Eastern Anatolia

We evaluated the clinical features and the effects of various treatment modalities on the clinical course in patients diagnosed with idiopathic thrombocytopenic purpura [ITP]. Retrospective investigation of the medical records of 168 patients at our center between 1994 and 2005 was done. Of the 168 patients, 115 [68.4%] were women and 53 [31.6%] men. At initial diagnosis, the median age of the patients was 33 years [range: 15-91] and 139 [82.7%] had signs of bleeding. Follow-up was complete in 130 patients and the median follow-up was 27 months [range: 3-132]. Initial treatment with either standard or high-dose steroid as first-line therapy was begun in 123 [73.2%] of the 168 patients. Complete remission [CR] was achieved in 56% of the patients. Sixty-one [61] patients who were followed up regularly received second-line therapies. CR was achieved in 45.8% of the patients who received steroids as second-line therapy. Within a median follow-up of 7 months, 27.2% of these patients relapsed. Splenectomy was performed in 26 patients and CR was obtained in 72% of the 25 patients regularly followed up. CR obtained by splenectomy was significantly higher than that obtained by steroids [p < 0.001]. The 10-year disease-free survivals in patients who used steroids and who underwent splenectomy were 15 and 61.6%, respectively. Steroid therapy is effective both in the initial and relapse periods. Splenectomy is the treatment of choice for those ITP patients refractory to steroid therapy and younger than 40 years of age

Púrpura trombocitopénico idiopático / Idiopathic thrombocytopenic purpura

El púrpura trombocitopénico idiopático (PTI) es una enfermedad autoinmune caracterizada por recuento bajo de plaquetas y hemorragias mucocutáneas, sin alteraciones en la médula ósea y en ausencia de otras causas de trombocitopenia. En niños suele ser autolimitada, y puede ser precedida por una infección viral o una inmunización.

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.

Purpura trombocitopénico idiopatico y embarazo: cesarea versus parto vaginal / Idiopathic thrombocytopenic purpura and pregnancy: cesarean section versus vaginal delivery

Si bien el púrpura trombocitopénico idiomático (PTI) es una patología poco frecuente, su importancia radica en que existe una alta morbimortalidad materno-fetal durante el embarazo, el parto y el puerperio. Es en el parto donde actualmente existe mayor controversia, con respecto a que vía es más recomendable, cesárea o vía vaginal. Nuestro propósito es acortar la brecha de la duda con respecto a ésta incógnita en base a la literatura disponible actualmente tanto a nivel nacional como internacional presentando una revisión bibliográfica sobre el PTI en el embarazo dando una pequeña reseña sobre la fisiopatología de ésta, diagnóstico y tratamiento, centrándonos en la controversia sobre que vía de parto es la más indicada. Se adoptó la estrategia de búsqueda desarrollada en la revisión electrónica de bases de datos, bajo criterios definidos que permitieron identificar los estudios con mejor evidencia posible. En nuestra búsqueda se encontraron 18 trabajos que cumplieran con nuestros criterios de búsqueda. En la literatura no encontramos un trabajo prospectivo randomizado con un buen grado de evidencia y fuerza que afirme una vía por sobre otra. Los trabajos encontrados corresponden a reportes de casos y guías de expertos que recomiendan un manejo por sobre otro. Podemos concluir que la vía del parto se definiría según condiciones obstétricas y no por el nivel de plaquetas fetales encontrados. Además, algunos de los procedimientos para determinar los niveles de plaquetas estarían relacionado a un mayor riesgo de morbimortalidad que la patología base.

Assessment of IVIG treatment in children and its complications [Babol; 1999-2004]

IVIG is used in patients incapable of producing antibodies and in autoimmune disorders. IVIG infusion rarely causes undesirable reactions due to the speed of infusion. So, this study was performed to determine the indications and complications of IVIG infusion. This descriptive study was performed on 265 patients, hospitalized in Amirkola pediatric hospital, needing IVIG infusions, from October 1999 to June 2004. Data was analyzed by SPSS. T-test, chi square and fisher exact test were used to compare drug complications based on age and speed of infusion in two genders. In this research, 265 patients with 871 infusions were studied. One hundred and thirty one patients [49.4%] were male and 134 [50.6%] were female. Two patients [0.7%] had autoimmune hemolytic anemia, one patient [0.3%] had ataxia telangectasia, 9 patients [3.4%] had refractory seizure, 2 patients [0.7%] had guillain-barre syndrome, 83 patients [31.3%] had immune thrombocytopenic purpura, 46 patients [17.3%] had kawasaki, 51 patients [19.2%] had immunodeficiency and 72 patients [27.1%] were premature neonates. Among the studied patients, 17 infusions [1.95%] had complications, which were mild in 14 patients [82.5%], moderate in 2 patients [11.7%] and severe in 1 patient [5.8%]. Complication was the same in both genders, and it had a significant difference with the speed of drug infusion [P<0.05]. The most common cause of IVIG infusion is immune thrombocytopenic purpura. Complications are usually mild and had relationship with the speed of drug infusion

Deplecão de célula B no tratamento de citopenias auto-imunes / B-Cell depletion in the treatment of autoimmune cytopenias

A morbidade associada ao tratamento de citopenias auto-imunes tornou necessária a busca por novas terapêuticas. Baseado no fato de que o rituximab reage especificamente contra o antígeno CD 20, induzindo deplecão de células B e conseqüentemente levando à diminuicão na producão de auto-anticorpos, cinco pacientes com citopenias auto-imunes foram tratados com esta droga. Os pacientes eram refratários à terapia convencional e receberam 375 mg/my de rituximab semanalmente, por um período de quatro semanas. Todos os pacientes apresentaram melhora, seja pelo aumento do número de células (níveis de hemoglobina ou contagem de plaquetas), seja pela suspensão do uso de corticoesteróides. Não foram observadas reacões importantes durante infusão do medicamento, ou mesmo episódios de infeccão durante acompanhamento subseqüente. Desta forma, o rituximab se mostrou eficaz e seguro para pacientes portadores de anemia hemolítica e púrpura trombocitopênica de etiologia imunológica, sugerindo que esta droga deva fazer parte do arsenal terapêutico utilizado nestas doencas auto-imunes.

Prolonged remission after splenectomy for refractory Evans syndrome--a case report and literature review.

We describe a patient with Evans syndrome (autoimmune hemolytic anemia and autoimmune thrombocytopenia) who was refractory to steroids and intravenous immunoglobulin. She responded to splenectomy and has remained in clinical remission for 3 years. In the majority of cases, splenectomy rarely induces a durable remission but it may be beneficial in a small group of patients, hence should be considered as alternative therapy in the management of these patients.