Safety and efficacy of romiplostim in children and adolescents with Immune thrombocytopenia: A systematic review

Abstract Objective To evaluate the efficacy and safety of romiplostim (thrombopoietin-receptor agonist) in the treatment of pediatric immune thrombocytopenia (ITP). Methods Searches were conducted in MEDLINE, EMBASE, LILACS, Cochrane Central Register of Controlled Trials and ClinicalTrials.gov (from January 2011 to August 2021). Randomized controlled trials (RCTs), double-blind, comparing romiplostim with a placebo in pediatric persistent or chronic ITP were included. The primary outcome was the overall response rate (platelets ≥ 50 × 109/L) in the absence of rescue therapy for at least two consecutive weeks. The secondary endpoints were the minimization of clinically significant bleeding and the necessity for rescue treatments and the maximization of safety (incidence of overall adverse events) and durable response (maintaining platelet counts for at least twelve weeks). Results Two double-blind randomized placebo-controlled trials (84 participants) were included in this systematic review. Our data showed that, compared to the placebo group, the proportion of patients achieving durable platelet response was significantly higher in the romiplostim group (p= 0.003, RR = 6.34, 95%CI = 1.89 - 21.23), as was the overall response in the romiplostim group (p= 0.002, RR = 3.62, 95%CI = 1.63 - 8.03). Significant bleeding incidents (p= 0.49), overall adverse events (p= 0.71) and the need for rescue treatment (p= 0.13) were not statistically different between the romiplostim and placebo groups. Conclusions Romiplostim might improve both durable and overall platelet response in children and adolescents with ITP, compared to a placebo. More clinical trials are needed to evaluate the efficacy and safety of romiplostim and to compare it with other second-line treatments that are being used in pediatric ITP.

Factores asociados a dermatoporosis en una muestra de pacientes geriátricos en México / Factors associated with dermatoporosis in a sample of geriatric patients in Mexico

Resumen Introducción: La dermatoporosis es un síndrome crónico de fragilidad cutánea, caracterizado por atrofia, púrpura y pseudocicatrices en piel. Objetivo: Determinar los factores asociados a dermatoporosis en una muestra de sujetos ≥ 60 años. Métodos: Estudio observacional, transversal, descriptivo y analítico de sujetos ≥ 60 años a quienes se realizó historia clínica, exploración física y aplicación de un autocuestionario diagnóstico de dermatoporosis. Para determinar los factores asociados se realizó análisis de regresión logística multivariado. Resultados: En 315 sujetos, la prevalencia de dermatoporosis fue de 29 %; 70 % fue del sexo femenino. Los factores asociados fueron edad > 75 años (p = 0.001), exposición solar prolongada (p = 0.002), ingesta de anticoagulantes/antiplaquetarios (p = 0.004), esteroides orales (p = 0.03) y enfermedad renal crónica (p = 0.03); así como, edad materna > 40 años en el último parto (p = 0.02), lactancia > 7 meses por embarazo y lactancia acumulada > 18 meses (p = 0.01). Se relacionaron con su ausencia, edad < 20 años en el primer embarazo y menopausia después de los 45 años. La correlación entre la autovaloración y el diagnóstico clínico fue muy alta (0.95, p < 0.001). Conclusiones: Los factores de riesgo asociados a dermatoporosis fueron similares a los previamente reportados.

Abstract Introduction: Dermatoporosis is a chronic cutaneous fragility syndrome, characterized by skin atrophy, purpura and pseudo-cicatrices. Objective: To determine factors associated with dermatoporosis in a sample of subjects aged ≥ 60 years. Methods: Observational, cross-sectional, descriptive, analytical study of subjects aged ≥ 60 years who underwent history taking, physical examination and application of a self-administered dermatoporosis diagnostic questionnaire. To determine the associated factors, a multivariate logistic regression analysis was used. Results: In 315 evaluated subjects, the prevalence of dermatoporosis was 29%; 70% were females. Associated risk factors were age > 75 years (p = 0.001), prolonged sun exposure (p = 0.002), use of anticoagulants/antiplatelet medications (p = 0.004), oral steroids (p = 0.03) and chronic kidney disease (p = 0.03); as well maternal age > 40 years at last pregnancy (p = 0.02), breastfeeding for > 7 months per pregnancy and > 18 cumulative months (p = 0.01). Age < 20 years at first pregnancy and menopause after 45 years were related to dermatoporosis absence. The correlation between self-assessment and clinical diagnosis was considerably high (0.95, p < 0.001). Conclusions: The risk factors associated with dermatoporosis were similar to those previously reported.

A Case of Off-Pump Coronary Artery Bypass Grafting Following High-Dose Dexamethasone Therapy in a Patient with Idiopathic Thrombocytopenic Purpura / 日本心臓血管外科学会雑誌

Man in his 70s, who had suffered from idiopathic thrombocytopenic purpura (ITP), was admitted to our hospital with chest pain at rest. Coronary angiography revealed obstruction of the right coronary artery and triple vessel disease. Because a bleeding tendency was expected during coronary artery bypass grafting, we performed percutaneous coronary intervention to the culprit lesion first, and then intravenous immunoglobulin and high dose dexamethasone were tried. His platelet count rose from 49,000 to 103,000/mm3, so we performed coronary artery bypass grafting. The patient had no postoperative hemorrhagic complications. We believe that high dose dexamethasone therapy is useful for patients with ITP who need surgery immediately.

Progress on diagnosis and treatment of kidney transplantation-associated thrombotic microangiopathy / 器官移植

Thrombotic microangiopathy (TMA) is a severe complication after kidney transplantation, mainly characterized by thrombocytopenia, microvascular hemolytic anemia and acute kidney injury, which may lead to kidney allograft failure or even death of the recipients. With the increasing quantity of solid organ transplantation in China and deeper understanding of TMA, relevant in-depth studies have been gradually carried out. Kidney transplantation-associated TMA is characterized with different causes and clinical manifestations. Non-invasive specific detection approach is still lacking. The diagnosis of TMA mainly depends on renal biopsy. However, most TMA patients are complicated with significant thrombocytopenia. Hence, renal puncture is a risky procedure. It is difficult to make a definite diagnosis. For kidney transplantation-associated TMA, plasma exchange, intravenous immunoglobulin and withdrawal of potential risk drugs are commonly employed. Nevertheless, the overall prognosis is poor. In this article, the classification of TMA after kidney transplantation, diagnosis and treatment of kidney transplantation-associated TMA were reviewed, aiming to provide reference for clinical diagnosis and treatment of kidney transplantation-associated TMA.

Microangiopatía trombótica como complicación de una pancreatitis aguda / Thrombotic microangiopathy as a complication of acute pancreatitis / Microangiopatia trombótica como complicação da pancreatite aguda

La púrpura trombótica trombocitopénica (PTT) es una microangiopatía trombótica poco frecuente, que se caracteriza por anemia hemolítica y plaquetopenia, con una elevada morbimortalidad. Su forma más frecuente es la PTT inmune, también denominada adquirida, provocada por la deficiencia de la enzima disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) secundaria a la presencia en plasma de autoanticuerpos. Presentamos el caso de un paciente con diagnóstico de pancreatitis aguda (PA) complicada con PTT, asociación de presentación excepcional en la práctica clínica.

Summary: Thrombotic thrombocytopenic purpura is rather an unusual thrombotic microangiopathy characterized by hemolytic anemia and plateletopenia which results in high morbimortality rates. The most frequent form of this disease is immune thrombotic thrombocytopenic purpura, also known as acquired thrombotic thrombocytopenic purpura, which is caused by enzime deficiency disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) that is secondary to antibodies in plasma. The study presents the case of a patient with a diagnosis of acute pancreatitis with a rare complication of thrombotic thrombocytopenic purpura which is exceptional in the clinical practice.

A púrpura trombocitopênica trombótica (PTT) é uma microangiopatia trombótica rara, caracterizada por anemia hemolítica e trombocitopenia, com alta morbimortalidade. Sua forma mais comum é a TTP imune, também conhecida como adquirida, que é causada pela deficiência da enzima ADAMTS13 (em inglês A disintegrin-like and metalloprotease with thrombospondin type 1 motif no. 13) secundária à presença de autoanticorpos no plasma. Apresentamos o caso de um paciente com diagnóstico de pancreatite aguda (PA) complicada por PTT, associação com apresentação excepcional na prática clínica.

Immunoadsorption therapy for a meningococcemia patient with myocarditis, adrenal hemorrhage, and purpura fulminans: a case report

Abstract Neisseria meningitidis, also known as meningococcus, is a relatively uncommon cause of invasive infection, but when it occurs, it is frequently severe and potentially life-threatening. A ten-year-old female patient developed a purpuric rash with fever. Upon arrival to the pediatric intensive care department, she was unconscious and in a poor general condition. We combined treatment with antibiotics, volume resuscitation, hydrocortisone, and CytoSorb® therapy resulted in a stabilization of hemodynamics, as well as control of hyperinflammation. We observed a significant decrease in vasopressor dosage in this patient.

Serum Syndecan-1 levels in patients with immunoglobulin A vasculitis in children

Abstract Objective The aim of this study was to evaluate the serum Syndecan-1 (SDC-1) levels in patients with immunoglobulin-A vasculitis (IgAV) in children and its relation with gastrointestinal involvements. Methods Sixty-eight children with IgAV and 48 healthy children were enrolled in this cross-sectional study. Clinical and related laboratory data were collected from a computerized hospital database. Serum SDC-1 was collected on admission prior to treatment. Results Forty-eight patients fully met the IgAV diagnostic criteria at admission (IgAV group), 20 patients with rash only and diagnosed IgAV during hospitalization (Purpura group). In IgAV group, 30 patients with gastrointestinal involvements (IgAV-GI group) and 18 patients without gastrointestinal involvements (IgAV-NGI group). SDC-1 serum levels were significantly higher in the IgAV group (86.37 ng/mL (IQR 59.16-117.14 ng/mL)) than in the controls (20.37 ng/mL (IQR 15.52-26.45 ng/mL)) and the Purpura group (32.66 ng/mL (IQR 14.87-49.89 ng/mL)). Additionally, SDC-1 (OR = 1.08) was independently associated with IgAV with a cut-off value (sensitivity and specificity) of 66.55 ng/mL (68.8%, 95.0%), and the area under the curve was 0.908. The serum SDC-1 levels of the IgAV-GI group (106.92 ± 50.12 ng/mL) were significantly higher than those in the IgAV-NGI group (67.52 ± 17.59 ng/mL). Logistic regression analysis showed that SDC-1 (OR = 1.03) was independently associated with IgAV-GI with a cut-off value of 89.39 ng/mL. Conclusions SDC-1 serum levels may mirror vascular endothelium injury and mucosal damage in IgAV. Its applicability as a surrogate biomarker in IgAV remains to be determined.

Vasculite associada à IgA em criança: relato de caso / IgA vasculitis in a child: case report

A Vasculite associada à imunoglobulina A (VIgA), também conhecida como púrpura de Henoch-Schonlein, púrpura anafilactóide ou púrpura reumática é uma vasculite de pequenos vasos associada a deposição de imunocomplexos IgA, de etiologia ainda desconhecida e que acomete principalmente crianças. Em grande parte dos casos pediátricos, é uma doença autolimitada com manifestações cutâneas, articulares, gastrintestinais e renais. O diagnóstico diferencial inclui outras vasculites, como lúpus eritematoso sistêmico, meningococcemia, coagulação intravascular disseminada e síndrome hemolítica urêmica. Neste artigo abordam-se os principais aspectos da VIgA nas crianças, salientando-se a importância do diagnóstico diferencial precoce. É apresentado o caso clínico de uma paciente do sexo feminino de 5 anos com lesões purpúricas tratada numa primeira abordagem como infecção bacteriana grave. Após reavaliação médica houve alteração terapêutica com uso de glicocorticóides resultando em melhora expressiva dos sintomas. [au]

Vasculitis associated with immunoglobulin A (VIgA), also known as Henoch-Schonlein purpura, anaphylactoid purpura or rheumatic purpura is a small vessel vasculitis associated with deposition of IgA immune complexes, of unknown etiology and affecting mainly children. In most pediatric cases, it is a self-limited disease with cutaneous, joint, gastrointestinal and renal manifestations. The differential diagnosis includes other vasculitis, such as systemic lupus erythematosus, meningococcemia, disseminated intravascular coagulation and uremic hemolytic syndrome. In this article, the main aspects of HSP in children are addressed, highlighting the importance of early differential diagnosis. The clinical case of a 5-year-old female patient with purpuric lesions treated in a first approach as a severe bacterial infection is presented. After medical re-evaluation, there was a therapeutic change with the use of glucocorticoids resulting in a significant improvement of symptoms. [au]

Púrpura de Schönlein Henoch en paciente adulto

RESUMEN La púrpura de Schönlein-Henoch es una vasculitis por fragmentación de leucocitos inmunomediada que afecta a pequeños vasos sanguíneos. Los cuatro componentes clínicos esenciales son púrpuras, dolor abdominal, artralgia y afectación renal. El caso trata de una mujer de 50 años que ingresa por dolor abdominal y hematoquecia de 72 horas de evolución, posterior a laparotomía exploratoria. Al examen físico presenta lesiones purpúricas en tronco y extremidades inferiores de 2 meses de aparición. En paraclínicos se observa hemograma con plaquetas normales, proteínas en orina 500 mg/dL, proteinuria 2,4 g/24 hs. Ante sospecha de vasculitis con plausible inclusión cutáneo-renal, se pide anticuerpos antinucleares, ANCA y se realiza biopsia cutánea evidenciándose una vasculitis neutrofílica necrotizante de pequeños vasos. En la biopsia renal se observa en inmunofluorescencia directa depósito de IgA, C3 positivo. En relación clínica de la proteinuria y compromiso cutáneo junto con la confirmación de biopsia renal se concluye en diagnóstico de púrpura de Schönlein Henoch. El interés de este caso radica en la inconsistencia de esta patología en los adultos, a pesar de que bien podría ser de una gravedad más notable dado que existe un mayor peligro de falla renal persistente.

ABSTRACT Schönlein-Henoch purpura is an immune-mediated leukocyte fragmentation vasculitis that affects small blood vessels. The four essential clinical components are purpura, abdominal pain, arthralgia, and renal involvement. This case concerns a 50-year-old woman who is admitted due to abdominal pain and hematochezia of 72 hours of evolution, after an exploratory laparotomy. On physical examination, she presents purpuric lesions on the trunk and lower extremities of 2 months of appearance. In paraclinical tests, a blood count with normal platelets, urine protein 500 mg/dL, and proteinuria 2.4 g/24 hours are observed. Suspecting vasculitis with plausible cutaneous-renal inclusion, antinuclear antibodies and ANCA are requested, and a skin biopsy is performed, showing necrotizing neutrophilic vasculitis of small vessels. In the renal biopsy, IgA deposit, C3 positive is observed in the direct immunofluorescence. In the clinical relationship of proteinuria and skin involvement together with the confirmation of renal biopsy, the diagnosis of Schönlein-Henoch purpura is concluded. The interest of this case lies in the inconsistency of this pathology in adults, despite the fact that it could be more serious given that there is a greater risk of persistent renal failure.

Thrombotic thrombocytopenic purpura as an acute complication of COVID-19

Introduction: While disseminated intravascular coagulation (DIC) is a serious complication of COVID-19, a close differential in critically ill patients with thrombocytopenia is Thrombotic thrombocytopenic purpura (TTP). Case Report: We describe the case of a middle-aged lady admitted with COVID-19 pneumonia who developed progressive thrombocytopenia, altered sensorium and renal failure. The absence of coagulation abnormalities alerted to the possibility of TTP, strengthened by presence of schistocytes in peripheral smear. Conclusions: This case highlights the need for high index of suspicion and to pay attention to normal tests as well that might give clues to the diagnosis. New onset thrombocytopenia in COVID-19 need not always indicate DIC. A careful examination of peripheral smear may help diagnosing TTP especially if coagulation profile is normal.

MULTIPLE MYELOMA COMPLICATED BY FATAL THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) – A CASE REPORT OF AUTOPSY FINDINGS IN A TERTIARY CARE CENTRE.

Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurological abnormalities, fever and renal dysfunction. Early clinical suspicion and presumptive diagnosis of TTP helps in timely initiation of treatment modalities speci?c for TTP which may prove to be lifesaving and thus augment in reducing the mortality rate of TTP which is estimated to be 80 – 90 % if left untreated. We report a case of a known case of multiple myeloma who developed TTP which proved fatal despite plasmapheresis. Signi?cant autopsy ?ndings of presence of microthrombi in the microvasculature of multiple organs is also highlighted.

Presentación inusual de infección por SARS-CoV-2 simulando púrpura de Schönlein Henoch (vasculitis por inmunoglobulina A) / Unusual presentation of SARS-CoV-2 infection simulating Schönlein Henoch purpura (immunoglobulin A vasculitis)

La vasculitis por inmunoglobulina A, anteriormente llamada púrpura Schönlein Henoch (VIgA/PSH), es la vasculitis sistémica más frecuente en la infancia. El desencadenante más común es una infección previa del tracto respiratorio superior. Se caracteriza por púrpura palpable no trombocitopénica con artralgias y/o artritis, afectación gastrointestinal y compromiso renal. SARS-CoV-2 es un virus ARN que causa la enfermedad COVID-19. Afecta frecuentemente el sistema respiratorio con presentaciones que varían desde una rinitis hasta condiciones severas como síndrome de distress respiratorio, shock séptico o síndrome de inflamación multisistémica (multi-system inflammation syndrome, MIS). Se describe el caso de un niño de 5 años de edad con clínica de VIgA/PSH como forma inicial de presentación y diagnóstico posterior de infección por SARS-CoV-2, derivado al hospital de mayor complejidad, con encefalopatía hipertensiva que presentó evolución favorable y restitución completa del cuadro clínico

Immunoglobulin A vasculitis, previously called Henoch Schonlein purpura (IgAV/ HSP), is the most common systemic vasculitis in childhood. The most common trigger is a previous upper respiratory infection. It is characterized by palpable non-thrombocytopenic purpura with arthralgia and/or arthritis, gastrointestinal and kidney involvement. SARS-CoV-2 is an RNA virus that causes COVID-19 disease. It frequently affects the respiratory system with presentations ranging from rhinitis to severe conditions such as respiratory distress syndrome, septic shock, or multi-system inflammation syndrome (MIS). We describe the case of a 5-year-old boy with symptoms of IgAV/HSP as the initial form of presentation and subsequent diagnosis of SARS-CoV-2 infection, being referred to a more complex hospital with hypertensive encephalopathy, presenting a favorable evolution and complete restoration of the clinical picture.

Purpura fulminante por déficit de proteína S (enfermedad oclusiva no inflamatoria) / Fulminant purpura due to protein S deficiency (non-inflammatory occlusive disease)

Paciente sexo masculino de 53 años de edad sin patologías de base conocidas acude por un cuadro de 72 horas de evolución de petequias pruriginosas que inician en región interna de muslos, que progresan a ambas piernas y pies de forma simétrica, luego aumentan en número y tamaño y cambio de coloración (de rojo a purpura). Se realiza biopsia de piel (pie y brazo izquierdo). Se realizan los estudios para búsqueda de etiología, donde llama la atención dosaje de proteína S disminuida (88 %)

A 53-year-old male patient with no known underlying pathologies presented with a 72-hour history of pruritic petechiae that began in the inner thighs, progressed symmetrically to both legs and feet, then increased in number and size. and color change (from red to purple). Skin biopsy (left foot and arm) is performed. Studies are performed to search for etiology, where low protein S dosage (88%) is striking.

Management of Perioperative Anticoagulation in a Patient with Antiphospholipid Antibody Syndrome Undergoing Cardiac Surgery: A Case Report

Patients with Antiphospholipid syndrome (APLS) are at high risk for both bleeding and thrombotic complications during cardiac surgery involving cardiopulmonary bypass (CPB). In this case we present a patient with APLS and Immune Thrombocytopenic Purpura who successfully underwent aortic valve replacement (AVR) with CPB despite recent craniotomy for subdural hematoma evacuation. Anticoagulation for CPB was monitored by targeting an Activated Clotting Time (ACT) that was 2× the upper limit of normal. A multidisciplinary approach was essential in ensuring a safe and successful operation.

Púrpura trombocitopénica posterior a vacunación contra COVID-19 / Thrombocytopenic purpura following COVID-19 vaccination

Resumen Introducción: la pandemia por COVID-19 ha generado un costo inexorable a los gobiernos y una mortalidad elevada en términos generales, el proceso de vacunación es la única estrategia efectiva en disminuir la morbimortalidad en general, sin embargo, dicha administración no está exenta de riesgos. Presentación del caso: presentamos el caso clínico de una paciente de género femenino de la séptima década, quien es vacunada contra el COVID-19 y tras cuatro días posteriores a la segunda dosis de vacuna anti COVID-19, comienza a presentar lesiones purpúricas que inician en miembros inferiores y ascienden progresivamente hasta el tronco asociado a artralgias intensas, dolor abdominal y náuseas. Es ingresada encontrándose recuentos plaquetarios menores a 10 mil plaquetas y se hace el diagnóstico de una púrpura trombocitopénica inmune asociada a la vacuna de AstraZeneca contra COVID-19, iniciándose terapia inmunosupresora específica. Discusión: el presente caso constituye la aparición de una púrpura trombocitopénica idiopática, patología poco frecuente con un amplio repertorio de eventos desencadenantes encontrándose las infecciones y reacciones vacunales dentro de los principales detonantes. La presente reacción vacunal es poco frecuente siendo lo anecdótico en específico la presencia de púrpura trombocitopénica inmune, hasta el momento son muy escasas las descripciones en la literatura sobre este tipo de reacción vacunal razón por la cual se decide recopilar y publicar el mismo mencionando la evidencia científica disponible más reciente sobre el tema. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2268).

Abstract Introduction: the COVID-19 pandemic has generated an inexorable cost for governments and an elevated mortality overall. Vaccination is the only effective strategy for decreasing overall morbidity and mortality; however, this vaccination is not without risks. Case presentation: we present the clinical case of a female patient in her seventies who was vaccinated against COVID-19 and four days after the second dose of the COVID-19 vaccine developed purpuric lesions beginning on her lower limbs and ascending progressively towards the trunk, associated with intense arthralgias, abdominal pain and nausea. She was admitted with platelet counts of less than 10,000, was diagnosed with immune thrombocytopenic purpura associated with the AstraZeneca COVID-19 vaccine, and was started on specific immunosuppressive therapy. Discussion: this is a case of idiopathic thrombocytopenic purpura, a rare pathology with a broad repertoire of triggering events, with infections and vaccine reactions among the main triggers. This vaccine reaction is rare, with the specific anecdotal point being the presence of immune thrombocytopenic purpura. To date, there are very few descriptions of this type of vaccine reaction in the literature, which is why we chose to compile and publish it, mentioning the most recent scientific evidence available on the subject. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2268).

Concurrencia entre el síndrome de Guillain-Barré y púrpura trombocitopénica inmune posiblemente inducidos por COVID-19 prolongado / Concurrence between Guillain-Barré syndrome and immune thrombocytopenic purpura possibly induced by long COVID-19

RESUMEN Durante la infección aguda por el SARVS-CoV-2 se produce una desregulación del sistema inmune que puede durar hasta ocho meses después de controlado el cuadro agudo. Esto, sumado a otros factores, posiblemente este asociado con un aumento del riesgo de aparición y concurrencia de enfermedades autoinmunes. La aparición simultanea del síndrome de Guillain-Barré (SGB) y púrpura trombocitopénica (PTI) se ha reportado poco en la literatura, y el SGB raramente se asocia con otra enfermedad autoinmune. Presentamos el caso de un varón que luego de un mes de tener un cuadro agudo de COVID-19 moderado, presentó concurrentemente SGB y PTI con respuesta adecuada al tratamiento.

ABSTRACT During acute SARS-CoV-2 infection, there is persistent deregulation of the immune system that can last up to 8 months after the acute condition is controlled. This, added to other factors, is possibly associated with an increased risk of the appearance and concurrence of autoimmune diseases. The simultaneous occurrence of GBS and ITP has been rarely reported in the literature, and GBS is rarely associated with another autoimmune disease. We present the case of a man who, one month after his recovery from acute moderate COVID-19, presented concurrent GBS and ITP with an adequate response to treatment.

Púrpura trombocitopénica inmunológica tras vacunación contra COVID-19

RESUMEN Se presenta el caso de un paciente masculino con púrpura trombocitopénica inmunológica que ocurre 10 días después de la aplicación de la vacuna contra la COVID-19. Se descartaron con estudios complementarios todas las causas secundarias. Con pulsos de corticoides presentó mejoría clínica y laboratorial, evolucionando favorablemente. Asumiendo la relación temporal con dicha vacuna, se presume que esta plaquetopenia fue una reacción adversa a la misma. En el país no se han notificado casos de púrpura trombocitopénica inmunológica hasta el último boletín informativo de reacciones adversas relacionadas a esta vacuna.

ABSTRACT We present the case of a male patient with immunological thrombocytopenic purpura that occurs 10 days after the application of the COVID-19 vaccine. All secondary causes were ruled out with complementary studies. With corticosteroid pulses, he presented clinical and laboratory improvement, progressing favorably. Assuming the temporal relationship with the vaccine, it is presumed that this thrombocytopenia was an adverse reaction to it. No cases of immune thrombocytopenic purpura have been reported in the country until the last bulletin of adverse reactions related to this vaccine.

Renal biopsy in children with IgA vasculitis / Biópsia renal em crianças com vasculite por IgA

Abstract Introduction Henoch-Schönlein purpura nephritis (HSN) is defined as Henoch-Schönlein purpura with kidney involvement, including hematuria and/or proteinuria. The aim of this study was to evaluate the data of HSN patients who underwent renal biopsy, and compare the main clinical and laboratory parameters that may affect renal biopsy findings, treatment protocols, and short- and long-term outcome of those patients. Methods Biopsies performed in 72 HSN patients between January 2007 to January 2017 were retrospectively evaluated. They were divided into two groups according to renal biopsy classification of the International Study of Kidney Disease in Children. Renal outcome, clinical and laboratory parameters, treatment protocols, and outcome were compared between groups. Short- and long-term follow-up of patients were evaluated. Results Of 72 patients, 47 were male (65.3%) and 44 (61.1%) were ≤10 years of age. Neutrophil-lymphocyte ratio was found higher in patients with scrotal involvement (p=0.042). Short-term unfavorable outcome was significantly higher in patients with scrotal involvement (p=0.038). Patients with hypertension and decreased creatinine clearance were found to have more unfavorable outcomes in long-term follow-up (p=0.029, p=0.040). Conclusion Cyclosporin-A and cyclophosphamide could be effective in steroid unresponsive HSN patients. Patients with scrotal involvement, decreased creatinine clearance, and hypertension should be closely monitored for sequelae of HSN.

Resumo Introdução A nefrite da púrpura de Henoch-Schönlein (NPHS) é definida como púrpura de Henoch-Schönlein com envolvimento renal, incluindo hematúria e/ou proteinúria. O objetivo deste estudo foi avaliar os dados de pacientes com NPHS que foram submetidos à biópsia renal e comparar os principais parâmetros clínicos e laboratoriais que podem afetar os achados da biópsia renal, os protocolos de tratamento e o desfecho de curto e longo prazo desses pacientes. Métodos Foram avaliadas retrospectivamente biópsias realizadas em 72 pacientes com NPHS entre Janeiro de 2007 e Janeiro de 2017. Eles foram divididos em dois grupos de acordo com a classificação de biópsia renal do Estudo Internacional de Doenças Renais em Crianças. O desfecho renal, parâmetros clínicos e laboratoriais, protocolos de tratamento e desfechos foram comparados entre os grupos. Foi avaliado o acompanhamento de pacientes de curto e longo prazo. Resultados De 72 pacientes, 47 eram homens (65,3%) e 44 (61,1%) tinham ≤10 anos de idade. A razão neutrófilo-linfócito foi encontrada mais alta em pacientes com envolvimento escrotal (p=0,042). O desfecho desfavorável de curto prazo foi significativamente maior em pacientes com envolvimento escrotal (p=0,038). Constatou-se que pacientes com hipertensão e diminuição da depuração de creatinina apresentaram desfechos mais desfavoráveis no acompanhamento de longo prazo (p=0,029, p=0,040). Conclusão A ciclosporina-A e a ciclofosfamida podem ser eficazes em pacientes com NPHS não responsivos a esteroides. Pacientes com envolvimento escrotal, diminuição da depuração de creatinina e hipertensão devem ser monitorados de perto para sequelas de NPHS.

The paradox of thrombotic thrombocytopenic purpura and the TACO challenge!

Thrombotic thrombocytopenic purpura (TTP) is a rare medical emergency characterized by the pentad of microangiopathic hemolytic anemia, thrombocytopenia, fever, renal failure, and neurological dysfunction. TTP is an infrequent condition and is a thrombotic microangiopathy. TTP is essentially a clinical diagnosis. As untreated TTP has a high mortality, diagnosis is usually presumptive and prompt treatment with plasma exchange is highly beneficial and reduces mortality significantly. Therapeutic plasma exchange with fresh frozen plasma is the standard treatment of choice for TTP. Transfusion-associated reactions may occur in some patients further complicating the disease picture and prolonging hospital stay and recovery. Transfusion-associated circulatory overload and transfusion-associated acute lung injury are the leading cause of transfusion-related mortality. We present here the diagnostic and therapeutic challenges that we faced with a young male patient who presented with fever, jaundice, and seizures.

Reference range for ADAMTS13 antigen, activity and anti-ADAMTS13 antibody in the healthy adult Singapore population

INTRODUCTION@#ADAMTS13 (a disintegrin-like and metalloproteinase with a thrombospondin Type 1 motif, member 13) plays a fundamental role in the regulation of haemostasis and thrombosis. Its deficiency leads to an accumulation of ultra-large von Willebrand multimers, inducing spontaneous platelet aggregation, thrombosis in the microvasculature, and thrombotic thrombocytopenic purpura (TTP), a condition with 90% mortality when left untreated. Prompt quantification of ADAMTS13 antigen, activity and autoantibody plays a crucial role in the diagnosis and management of TTP and can help differentiate it from other thrombotic microangiopathies (TMAs). Reference ranges for ADAMTS13 are generally derived from Caucasian patients. Given that polymorphism in the ADAMTS13 gene can be associated with variable ADAMTS13 levels, we aimed to establish the first reference range in Singapore and provide a crucial laboratory test for institutions here and elsewhere.@*METHODS@#150 healthy voluntary donors (75 men, 75 women) aged 21-60 years, with an ethnic mix mirroring Singapore's population profile, were recruited. ADAMTS13 antigen, activity and autoantibody levels were measured using the fluorescence resonance energy transfer-vWF73 and enzyme-linked immunosorbent assay methodologies.@*RESULTS@#Levels (activity 0.65-1.79 IU/mL, antigen 0.36-1.17 IU/mL, autoantibody 1.4-12.5 U/mL) were not statistically different between the genders and various age groups.@*CONCLUSION@#TTP and TMAs are encountered in a wide range of specialties. The availability of new assays in Singapore will aid clinicians in the timely management of these conditions. Standardising reference ranges established for Singapore against World Health Organization standards allows harmonisation of measurements between laboratories and for future research collaborations.