RESUMO
La intoxicación por naftaleno es poco frecuente en los niños. Es causada por la ingesta, la inhalación o el contacto con la piel de sustancias que contienen naftaleno. Los pacientes suelen tener orina de color marrón oscuro, diarrea acuosa y vómito bilioso. Los signos incluyen fiebre, taquicardia, hipotensión y valores bajos en la oximetría de pulso, incluso con oxigenoterapia. Los análisis de sangre detectan anemia hemolítica, metahemoglobinemia, insuficiencia renal e hiperbilirrubinemia. Además del tratamiento sintomático, se hacen transfusiones de eritrocitos y se les administran ácido ascórbico, azul de metileno y N-acetilcisteína. En este artículo, presentamos el caso de un paciente masculino de 23 meses de edad con metahemoglobinemia y hemólisis intravascular aguda que recibió atención en la unidad de cuidados intensivos durante cinco días por intoxicación por naftaleno. Si bien la intoxicación por naftaleno es muy poco frecuente, tiene consecuencias mortales y se debe ejercer precaución con su uso y venta.
Poisoning by naphthalene is uncommon in children. It is a type of poisoning brought on by ingesting, inhaling, or coming into touch with naphthalene-containing substances on the skin. Patients typically present with an initial onset of dark brown urine, watery diarrhea, and bile vomit. The signs include fever, tachycardia, hypotension, and low pulse oximetry readings even with oxygen support. Hemolytic anemia, methemoglobinemia, renal failure, and hyperbilirubinemia are all detected in blood tests. Erythrocyte transfusion, ascorbic acid, methylene blue, and N-acetylcysteine (NAC) therapies are provided to inpatients in addition to symptomatic treatment. We present a 23-month-old male patient who developed methemoglobinemia and acute intravascular hemolysis, who was followed up in the intensive care unit for five days due to naphthalene intoxication. Although naphthalene poisoning is very rare, it should be known that it has fatal consequences, and more care should be taken in its use and sale.
Assuntos
Humanos , Masculino , Lactente , Anemia Hemolítica/diagnóstico , Metemoglobinemia/diagnóstico , Metemoglobinemia/induzido quimicamente , Ácido Ascórbico , Hemólise , NaftalenosRESUMO
La púrpura trombótica trombocitopénica es una entidad poco frecuente en pediatría, pero de alta mortalidad sin tratamiento adecuado y oportuno. Se caracteriza por presentar anemia hemolítica microangiopática asociada a signos y síntomas neurológicos, cardíacos, abdominales y menos frecuentemente renales; puede estar acompañada de fiebre. En niños, el diagnóstico se basa en los hallazgos clínicos y de laboratorio. La actividad de ADAMTS13 <10 % apoya, pero no confirma el diagnóstico y, dada la gravedad de la patología, el resultado no debe retrasar el inicio del tratamiento. Se presenta una paciente de 15 años, previamente sana, con signos neurológicos asociados a anemia hemolítica y trombocitopenia. Durante su internación, se arribó al diagnóstico de púrpura trombótica trombocitopénica adquirida.
Thrombotic thrombocytopenic purpura is a rare disease in pediatrics, but it has a high mortality if not managed in an adequate and timely manner. It is characterized by microangiopathic hemolytic anemia associated with neurological, cardiac, abdominal, and less frequently, renal signs and symptoms; it may be accompanied by fever. In children, diagnosis is based on clinical and laboratory findings. ADAMTS13 activity < 10% supports the diagnosis but does not confirm it and, given its severity, the result should not delay treatment initiation. Here we describe the case of a previously healthy 15-year-old female patient with neurological signs associated with hemolytic anemia and thrombocytopenia. During hospitalization, she was diagnosed with acquired thrombotic thrombocytopenic purpura.
Assuntos
Humanos , Feminino , Adolescente , Púrpura Trombocitopênica Trombótica/complicações , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia , Anemia Hemolítica/diagnóstico , PediatriaRESUMO
Sjögren's syndrome(SS)is a chronic autoimmune disease that affects exocrine glands, especially salivary and lacrimal glands. The main clinical manifestations are dry mouth and dry eyes, but also multi-organ and multi-system can be involved. Cold agglutinin disease(CAD)is an autoimmune disease characterized by red blood cell agglutination in the blood vessels of extremities caused by cold agglutinin at low temperature, resulting in skin microcirculation disturbance, or hemolytic anemia. Cold agglutinin disease is divided into two categories, primary cold agglutinin disease and secondary cold agglutinin disease. Primary cold agglutinin disease is characterized with cold agglutinin titer of 1 ∶4 000 or more and positive Coomb's test. However, the Coomb's test is not necessarily positive and the cold agglutinin titer is between 1 ∶32 and 1 ∶4 000 in secondary cold agglutinin disease. Here, we reported an elderly patient admitted to hospital due to fever. He was diagnosed with respiratory infection, but he showed incompletely response to the anti-infection treatment. Further laboratory tests showed the patient with positive ANA and anti-SSA antibodies. Additionally, the patient complained that he had dry mouth and dry eyes for 1 year. Schirmer test and salivate gland imaging finally confirmed the diagnosis Sjogren's syndrome. During the hospital stay, the blood clots were found in the anticoagulant tubes. Hemolytic anemia was considered as the patient had anemia with elevated reticulocytes and indirect bilirubin. In addition, further examination showed positive cold agglutination test with a titer of 1 ∶1 024, and cold agglutinin disease was an important type of cold-resistant autoimmune hemolytic anemia. Furthermore, the patient developed cyanosis after ice incubating at the tip of the nose. Hence, the patient was diagnosed as CAD and he was successfully treated with glucocorticoids instead of anti-infection treatments. Hence, the patient was diagnosed with SS combined with secondary CAD. SS combined CAD are rarely reported, and they are both autoimmune diseases. The abnormal function of B lymphocytes and the production of autoantibodies might be the common pathogenesis of them. Cold agglutinin disease can lead to severe hemolytic anemia, even life-threatening. In clinical practice, timely recognizing and dealing with CAD might promote the prognosis of the patient.
Assuntos
Masculino , Humanos , Idoso , Anemia Hemolítica Autoimune/diagnóstico , Síndrome de Sjogren/diagnóstico , Anemia Hemolítica/complicações , Síndromes do Olho Seco/complicações , AutoanticorposRESUMO
Anaemia is a global public health problem with high mortality and morbidity. It is also a common consequence of chronic kidney disease (CKD). There is a paucity of data on the actual burden of anaemia among patients on chronic haemodialysis (CHD) in Lagos, Nigeria. Objectives: To determine the prevalence and factors associated with the severity of anaemia among Nigerian patients undergoing chronic haemodialysis. Methods: This was a retrospective analysis of adult patients with end-stage renal disease (ESRD) on maintenance haemodialysis at the Lagos State University Teaching Hospital, Ikeja, Lagos. The data extracted from the clinical case files included the bio-demographic and clinical parameters, including the treatment modalities. Results: A total of 92 patients comprising 69 (75%) males and 23 (25.0%) females with the overall mean age of 48.2±14.0 years were included. Hypertension was the commonest aetiology of CKD and the average duration of haemodialysis was 16.6 months. The commonest access route for haemodialysis was a central line while 96.7% and 81.5% received erythropoietin and intravenous iron sucrose respectively. Seventy-three (79.3%) patients have had intra-dialysis blood transfusions in the past. Mild, moderate, and severe anaemia were recorded in 17%, 67%, and 16% respectively. The use of erythropoietin, iron sucrose, and increased frequency of blood transfusions correlated with the severity of anaemia. Conclusion: Anaemia is highly prevalent among patients with CKD on chronic haemodialysis. Increased frequency of blood transfusions, inadequate utilization of erythropoietin, and iron sucrose administration are associated with anaemia severity.
Assuntos
Humanos , Eritropoetina , Diálise Renal , Anemia Hemolítica , Transfusão de Sangue , Indicadores de Morbimortalidade , Saúde Pública , Insuficiência Renal Crônica , Óxido de Ferro Sacarado , Falência Renal CrônicaRESUMO
Background@#Due to the high prevalence and incidence of leprosy in the Philippines, there is a continuing need to detect and document the occurrence of dapsone-induced hemolytic anemia. @*Objective@#The aim of this study is to determine the incidence of dapsone-induced hemolytic anemia in non-glucose-6-phosphate dehydrogenase deficient leprosy patients receiving multidrug therapy (MDT) in Southern Philippines Medical Center.@*Methodology@#This is a retrospective study through chart review of leprosy patients treated with MDT regimen at Southern Philippines Medical Center from January 2016 to December 2018. The demographic profile, clinical characteristics, hemoglobin and hematocrit concentrations before and after initiation of MDT, the presence of symptoms of anemia, and the occurrence of dapsone-induced hemolytic anemia in leprosy patients were collected. The main outcome measure for this study was the incidence rate of dapsone- induced hemolytic anemia. Statistical-based analysis were used for continuous and categorical data which were summarized using means and standard deviations, and frequencies and percentages, respectively.@*Results@#There was a decrease in the mean hemoglobin and hematocrit levels noted in the majority of patients after initiation of MDT from baseline 143.46 g/dl and 0.44, respectively, to 94 g/dl and 0.28 on the third month of MDT. The incidence rate of dapsone-induced hemolytic anemia during the 3-year period was 20 cases per 100.@*Conclusion@#The relatively high incidence rate of dapsone-induced hemolytic anemia highlights the importance of frequent monitoring of hemoglobin and hematocrit concentrations in leprosy patients being treated with multidrug therapy.
Assuntos
Hanseníase , Dapsona , Anemia HemolíticaRESUMO
Thrombotic microangiopathy (TMA) is a group of highly heterogeneous, acute and severe clinicopathological syndromes, characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and ischemic injury of end organs. TMA has the characteristics of dangerous condition, multiple organ involvement and high mortality. Patients with severe TMA need to be admitted to intensive care unit (ICU) for organ function support therapy. Early and rapid evaluation, differential diagnosis, and timely and effective treatment are the key to improve the prognosis of TMA patients. Here, we review the pathophysiological changes, diagnosis differential diagnosis, and treatment of the severe TMA in adult.
Assuntos
Adulto , Humanos , Microangiopatias Trombóticas/terapia , Púrpura Trombocitopênica Trombótica/terapia , Anemia Hemolítica/terapia , Resultado do Tratamento , Diagnóstico DiferencialRESUMO
Introducción: la enfermedad hemolítica del feto y el recién nacido (EHFRN) consiste en la incompatibilidad presente entre los antígenos eritrocitarios maternos y los fetales, que desencadena en la madre una reacción inmunitaria contra los eritrocitos fetales produciendo su destrucción. La complicación más grave es la hidropesía fetal, la cual consiste en síntomas de origen hemodinámico, derivados de una falla cardíaca por la disminución en el aporte de oxígeno o por la falta de producción de albúmina. Objetivo: realizar una revisión actualizada de la EHFRN, exponiendo principalmente la hidropesía fetal como una de sus grandes complicaciones. Metodología: se realizó una revisión bibliográfica desde 2018 hasta 2021 en bases de datos tales como Science Direct, Pubmed y Medline con base en los siguientes términos MeSH: anemia hemolítica, isoinmunización Rh, eritroblastosis fetal, hidropesía fetal. Conclusión: la EHFRN es una causa frecuente de enfermedad hemolítica grave en estos pacientes, pero gracias a la Inmunoglubulina G anti-D se ha logrado prevenir la mayoría de casos de incompatibilidad Rh. Sin embargo, la hidropesía fetal presenta una alta mortalidad, lo cual hace importante promover un diagnóstico oportuno y el uso de profilaxis
Introduction: Hemolytic disease of the fetus and newborn (EHFRN) consists of the incompatibility present between maternal and fetal erythrocyte antigens, which triggers an immune reaction in the mother against fetal erythrocytes, causing their destruction. The most serious complication is hydrops fetalis, which consists of symptoms of hemodynamic origin, derived from heart failure due to the decrease in oxygen supply or the lack of albumin production. Objective: Make an updated review of the EHFRN, exposing mainly hydrops fetalis as one of its major complications. Methodology: Bibliographic review was carried out from 2018 to 2021 in databases such as Science Direct, Pubmed and Medline based on the following MeSH terms: hemolytic anemia, Rh isoimmunization, erythroblastosis fetalis, hydrops fetalis. Conclusion: EHFRN is a frequent cause of severe hemolytic disease in these patients; but thanks to the anti-D Immunoglobulin G, the majority of cases of Rh incompatibility have been prevented. However, hydrops fetalis has a high mortality rate, which makes it important to promote timely diagnosis and the use of prophylaxis
Assuntos
Humanos , Recém-Nascido , Recém-Nascido , Hidropisia Fetal , Anemia Hemolítica , Eritroblastose FetalRESUMO
Introducción: Las membranopatías son anemias hemolíticas hereditarias debidas a anomalías cualitativas o deficiencias cuantitativas de las proteínas del citoesqueleto del glóbulo rojo. Objetivo: Actualizar el diagnóstico de las membranopatías con la inclusión de las últimas recomendaciones del comité de grupos de expertos a nivel nacional e internacional. Métodos: Se realizó una revisión de la literatura en inglés y español, a través del sitio web PubMed y el motor de búsqueda Google académico, de artículos publicados en los últimos cinco años. Análisis y síntesis de la información: Las enfermedades de mayor interés clínico son: la esferocitosis, la eliptocitosis y la estomatocitosis hereditaria. Estas en general se heredan con carácter autosómico dominante pero existen formas que se transmiten con carácter recesivo, sin descartar posible mutación de novo. Para su diagnóstico se utilizan pruebas que incluyen el estudio de la morfología de los glóbulos rojos, la fragilidad osmótica, la lisis de glicerol acidificado, la criohemólisis hipertónica, la prueba de unión a la eosina-5'-maleimida por citometría de flujo, la electroforesis en gel de poliacrilamida con dodecilsulfato sódico y la ectacitometría. Conclusiones: Las membranopatías pueden sospecharse de manera preliminar teniendo en cuenta algunas alteraciones de la morfología eritrocitaria, aunque el diagnóstico se basa en estudios familiares y otros de carácter confirmatorio de la enfermedad, como los estudios moleculares. Los profesionales de la salud que atienden a pacientes jóvenes con anemia deben considerar la posibilidad de una anemia hemolítica por trastornos de la membrana eritrocitaria(AU)
ABSTRACT Introduction: Membranopathies are inherited hemolytic anemias due to qualitative abnormalities or quantitative deficiencies of red blood cell cytoskeletal proteins. Objective: to update the diagnosis of membranopathies with the inclusion of the latest recommendations from the committee of expert groups at the national and international level. Methods: A review of the literature in English and Spanish was carried out, through the PubMed website and the academic search engine Google, in articles published in the last five years. Analysis and synthesis of information: The diseases of greatest clinical interest are: spherocytocis, elliptocytosis and hereditary stomatocytosis. These are generally inherited with an autosomal dominant character but there are forms that are transmitted recessively, without ruling out a possible de novo mutation. For its diagnosis, tests are used that include the study of red blood cell morphology, osmotic fragility, acidified glycerol lysis, hypertonic cryohemolysis, eosin-5'-maleimide binding test by flow cytometry, sodium dodecyl sulfate polyacrylamide gel electrophoresis and ectacytometry. Conclusions: Membranopathies can be preliminarily suspected taking into account some alterations in erythrocyte morphology, although the diagnosis is based on family studies and others confirming the disease, such as molecular studies. Healthcare professionals caring for young patients with anemia should consider the possibility of hemolytic anemia due to red cell membrane disorders(AU)
Assuntos
Humanos , Masculino , Feminino , Fragilidade Osmótica , Pessoal de Saúde , Atenção à Saúde , Eletroforese em Gel de Poliacrilamida , Anemia Hemolítica , Citometria de FluxoRESUMO
@#<p style="text-align: justify;"><strong>Objective:</strong> Determination of the prevalence of severe hematologic manifestations among Filipino patients with systemic lupus erythematosus (SLE) and analysis of any association with organ involvement and serology.</p><p style="text-align: justify;"><strong>Methods:</strong> This cross-sectional study included SLE patients 19 years old and above seen at the UST Hospital from 2012 to 2017. Patients with severe hematologic manifestations (severe hemolytic anemia, severe thrombocytopenia, and Evans syndrome (ES)) were identified and their prevalence determined. Independent t-test was used to compare continuous variables. Categorical variables were measured using the chi-square test; odds ratios (OR) with their corresponding 95% confidence interval were calculated using the SPSS software version 21. This study has been approved by the Institutional Review Board.</p><p style="text-align: justify;"><strong>Results:</strong> Of the 253 patients (238 females, 94.07%), the mean age at diagnosis was 27.04 (SD 9.96) years. Severe hematologic involvement was noted in 12.26% (n=31); severe hemolytic anemia was the most prevalent (14, 5.53%), followed by severe thrombocytopenia (13, 5.14%) and ES (4, 1.58%). Higher prevalence of major organ involvement was observed among patients who manifest with severe hematologic disease. Severe thrombocytopenia was more likely to have cardiac involvement (OR 7.39, 95% CI 1.90 to 28.81, p=0.004). A higher prevalence of negative baseline anti-dsDNA serology was seen among patients who developed ES.</p><p style="text-align: justify;"><strong>Conclusion:</strong> Severe hematologic involvement was noted in 12.26% of Filipino SLE patients, with hemolytic anemia as the most frequently recorded. Severe thrombocytopenia was associated with cardiac involvement among these patients. Patients developing ES tend to have a negative anti-dsDNA serology.</p>
Assuntos
Lúpus Eritematoso Sistêmico , Anemia Hemolítica , TrombocitopeniaRESUMO
Abstract Thrombotic microangiopathies are disorders characterized by nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and multi-systemic failure. They are classified as thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome, and typical hemolytic uremic syndrome. The latter is associated with intestinal infections by Shiga toxin-producing bacteria. Typical hemolytic uremic syndrome in adults is an extremely rare condition, characterized by high morbidity and mortality. It has been seldom described in solid organ transplant recipients. Here is presented the case of a kidney transplant recipient who had typical hemolytic uremic syndrome with multisystem commitment, refractory to management and with a fatal outcome.
Resumo Microangiopatias trombóticas são distúrbios caracterizados por anemia hemolítica microangiopática não imune, trombocitopenia e insuficiência multissistêmica. Elas são classificadas como púrpura trombocitopênica trombótica, síndrome hemolítico-urêmica atípica e síndrome urêmica hemolítica típica. Essa última está associada a infecções intestinais por bactérias produtoras da toxina Shiga. A síndrome hemolítica urêmica típica em adultos é uma condição extremamente rara, caracterizada por alta morbimortalidade. Esta é raramente descrita em receptores de transplantes de órgãos sólidos. Apresentamos aqui o caso de um receptor de transplante renal que apresentava síndrome hemolítico-urêmica típica com comprometimento multissistêmico, refratário ao tratamento, e com desfecho fatal.
Assuntos
Humanos , Adulto , Púrpura Trombocitopênica Trombótica , Transplante de Rim , Escherichia coli Shiga Toxigênica , Síndrome Hemolítico-Urêmica Atípica , Anemia HemolíticaRESUMO
Abstract Primary atypical hemolytic-uremic syndrome is a rare disease characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction; it is related to alterations in the regulation of the alternative pathway of complement due to genetic mutations. The association with nephrotic syndrome is unusual. We present here a pediatric patient diagnosed with primary atypical hemolytic-uremic syndrome associated with nephrotic syndrome who responded to eculizumab treatment.
Resumo A síndrome hemolítico-urêmica atípica primária é uma doença rara, caracterizada por anemia hemolítica microangiopática não-imune, trombocitopenia e disfunção renal; está relacionado a alterações na regulação da via alternativa do complemento devido a mutações genéticas. A associação com a síndrome nefrótica é incomum. Apresentamos aqui um paciente pediátrico com diagnóstico de síndrome hemolítico-urêmica atípica primária associada à síndrome nefrótica que respondeu ao tratamento com eculizumab.
Assuntos
Humanos , Criança , Púrpura Trombocitopênica Trombótica , Síndrome Hemolítico-Urêmica Atípica/complicações , Anemia Hemolítica , Síndrome Nefrótica/complicações , Proteínas do Sistema ComplementoRESUMO
Objetivo: Analisar qualitativamente o teste de fragilidade osmótica (F.O.) para amostras a fresco ou após incubação a 37°C. Métodos: Foram processadas 20 amostras de sangue periférico, coletadas em duplicata com 5mL em cada tubo com heparina, de pacientes com solicitação de F.O. como exame de rotina para processamento a fresco e após incubação por 24 horas em banho-maria a 37°C, em 13 tubos com concentrações variáveis de 0,1% a 0,9% de NaCl. Resultados: Foram analisadas 20 amostras de pacientes em sua maioria do gênero feminino 17/20 (85%), com idades entre 3 meses a 75 anos, para realização do teste de F.O. A análise qualitativa dos resultados mostrou que 9/20 (45%) amostras tiveram resultado concordante entre os testes de F.O. para amostras a fresco e após incubação a 37°C. Dos resultados discordantes, 8/11 (72,7%) resultados mostram fragilidade dos eritrócitos à hemólise nas amostras a fresco e curva normal (sem hemólise) após incubação da amostra a 37°C. Outros 3/11 (22,3%) resultados apresentaram curva normal (sem hemólise) no teste com amostra à fresco e resistência à hemólise no teste com a amostra após incubação a 37°C. Com o teste de Extato de Fisher não mostrou diferença estatística (p=0,5743) para as amostras processadas a fresco ou após incubação a 37°C. Conclusão: O teste de F.O. se mostrou mais eficiente quando a amostra testada foi analisada após incubação por 24 horas a 37°C em banho-maria, contudo não houve diferença estatística para resultados processados a fresco ou após incubação a 37°C.
Objective: Qualitatively analyze the osmotic fragility test (O.F.) for samples fresh or after incubation at 37°C. Methods: Twenty peripheral blood samples were processed, collected in duplicate with 5 ml in each tube with heparin, from patients with O.F. request. as a routine examination for fresh processing and after incubation for 24 hours in a water bath at 37°C, in 13 tubes with varying concentrations of 0.1% to 0.9% NaCl. Results: Twenty samples of patients were analyzed, mostly female, 17/20 (85%), aged between 3 months to 75 years, for the O.F. test. The qualitative analysis of the results showed that only 9/20 (45%) samples had a consistent result between the F.O. tests for fresh samples and after incubation at 37°C. From the discordant results, 8/11 (72.7%) results show fragility of erythrocytes to hemolysis in fresh samples and normal curve (without hemolysis) after sample incubation at 37o C. Other 3/11 (22.3%) results showed normal curve (without hemolysis) in the test with fresh sample and resistance to hemolysis in the test with the sample after incubation at 37o C. With the Fisher Extact test showing no statistical difference (p=0.5743) for samples processed fresh or after incubation at 37°C. Conclusion: The O.F. proved to be more efficient when the tested sample was analyzed after incubation for 24h at 37°C in a water bath, however, there was no statistical difference for results processed fresh or after incubation at 37°C.
Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Fragilidade Osmótica , Anemia Hemolítica , HemóliseRESUMO
La seudomicroangiopatía trombótica o síndrome de Moschcowitz es una manifestación infrecuente del déficit de vitamina B12. Se caracteriza por anemia hemolítica con características microangiopáticas, reticulocitos e índices hematimétricos normales o con ligera megaloblastosis, asociados a manifestaciones neurológicas. La vitamina B12 está presente en alimentos proteicos de origen animal. La lactancia materna es una fuente adecuada para los niños cuando los niveles maternos son normales. Se presenta a una paciente de 16 meses que se internó por anemia hemolítica con requerimiento transfusional, plaquetopenia, mal progreso pondoestatural y retraso neuromadurativo. Durante su internación se arribó al diagnóstico de seudomicroangiopatía trombótica secundaria a déficit de vitamina B12.
Pseudo-thrombotic microangiopathy, or Moschcowitz syndrome, is a rare manifestation of vitamin B12 deficiency. It is characterized by microangiopathic hemolytic anemia, reticulocytes, and hematimetric indices that can be normal or that might present a mild megaloblastosis, and which are associated with neurological manifestations. Vitamin B12 can be found in animal-based protein foods. Breastfeeding is an adequate source of this vitamin for children, when maternal serum levels are normal. The case of a 16-month-old infant is presented. She was admitted for hemolytic anemia with transfusion requirement, thrombocytopenia, failure to thrive and developmental delay. During her hospitalization, she was diagnosed with pseudothrombotic microangiopathy caused by vitamin B12 deficiency.
Assuntos
Humanos , Feminino , Lactente , Deficiência de Vitamina B 12/complicações , Microangiopatias Trombóticas/diagnóstico , Deficiência de Vitamina B 12/terapia , Anemia Hemolítica/sangueRESUMO
This study aimed to identify, by means of thromboelastometry assessment, altered thrombotic risk in dogs with primary and secondary IMHA by E. canis infection after initiating the immunosuppressive therapy with mycophenolate mofetil. The animals' screening was based on complete blood count (CBC), biochemical and urine tests. Dogs with moderate to severe anemia (hematocrit ≤ 25%) which showed symptoms of immune-mediated hemolysis, such as spherocytosis, positive saline agglutination, bilirubinuria and/or hemoglobinuria, were included. Blood and urine samples were collected at two different moments. The first sample (M1) was collected at the time of diagnosis, when hematocrit was lower or equal to 25% before treatment with mycophenolate mofetil (Accord ®); the second sample (M2) was collected after treatment with mycophenolate mofetil, when hematocrit was greater or equal to 30%. Five out of the twelve animals selected died before the end of the study. No reduction in thrombotic risk was observed in the animals treated with mycophenolate mofetil. The animals that presented hypocoagulation at the time of diagnosis showed the worst prognosis, and their reticulocyte count displayed a better prognostic value than their erythrocytes count at the time of diagnosis.(AU)
O objetivo deste estudo foi esclarecer se há alteração do risco trombótico em cães com anemia hemolítica imunomediada primária e secundária a E.canis, avaliado por meio da tromboelastometria, após início de tratamento com micofenolato de mofetila. A seleção dos animais foi baseada na avaliação de hemograma, exame bioquímico e urinálise. Cães com anemia moderada a severa (hematócrito ≤ 25%), com sinais de hemólise imunomediada, como esferocitose, aglutinação em salina positivo, bilirrubinúria e/ ou hemoglobinúria, foram incluídos. As amostras de sangue e urina foram coletadas em dois momentos diferentes. A primeira amostra (M1) foi coletada no momento do diagnóstico, quando o hematócrito era igual ou inferior a 25%, sem fazer uso do micofenolato de mofetila (Accord®), e o segundo momento (M2), após tratamento com micofenolato de mofetila, quando o hematócrito era igual ou maior que 30%. Doze animais foram selecionados, cinco morreram antes do término do estudo. Não houve diminuição do risco trombótico entre os animais tratados com micofenolato de mofetila; os animais que apresentaram menor coagulabilidade apresentaram pior prognóstico, e a contagem de reticulócitos apresentou melhor valor prognóstico do que a contagem de hemácias no momento do diagnóstico.(AU)
Assuntos
Animais , Cães , Imunossupressores/uso terapêutico , Anemia Hemolítica/complicações , Anemia Hemolítica/veterinária , Ácido Micofenólico/análise , Ácido Micofenólico/efeitos adversos , Tromboelastografia/veterinária , Ehrlichia canis , Contagem de Eritrócitos/veterinária , HemostasiaRESUMO
RESUMEN La enfermedad hemolítica perinatal es infrecuente hoy por la prevención que de ella se hace. Sin embargo, existen casos de madres altamente sensibilizadas que desean tener un hijo, lo que obliga a que ese embarazo deseado sea controlado de manera especial y sometido a procedimientos invasivos no exentos de morbimortalidad fetal. El uso prenatal de inmunoglobulina humana en la madre puede representar una alternativa terapéutica. Se presenta un caso en que su uso impidió el desarrollo de enfermedad intrauterina y favoreció la buena evolución neonatal a pesar de que el pronóstico inicial era muy adverso.
ABSTRACT Perinatal Hemolytic Disease is uncommon today due to its prevention. However, there are cases of highly sensitized mothers who wish to have a child, that forces this desired pregnancy to be controlled in a special way and be subjected to invasive procedures not exempt from fetal morbidity and mortality. Prenatal use of human inmunoglobulin in the mother may represent a therapeutic alternative. We present a case in which its use prevented the development of intrauterine disease and favored a good neonatal evolution despite the fact that the initial prognosis was very adverse.
Assuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Adulto , Imunoglobulinas Intravenosas/administração & dosagem , Eritroblastose Fetal/prevenção & controle , Anemia Hemolítica/prevenção & controle , Cuidado Pré-Natal , Isoimunização Rh/prevenção & controle , Transfusão de Sangue IntrauterinaRESUMO
Resumen Las manifestaciones hematológicas de la infección por el VIH son frecuentes y variadas debido a su capacidad de afectar prácticamente todas las líneas celulares. Dentro de éstas, la púrpura trombocitopénica trombótica (PTT) es una de las entidades que constituyen las microangiopatías trombóticas. Se caracteriza por la presencia de trombocitopenia y anemia hemolítica microangiopática con alteración de la función renal. Actualmente, la co-existencia de estas dos entidades es poco frecuente debido a la terapia anti-retroviral de alta efectividad (TARV) Presentamos el caso de un paciente de 28 años, quien consultó por fiebre asociada a episodios de gingivorragia, palidez mucocutánea generalizada y debilidad progresiva. Los estudios evidenciaron una anemia y trombocitopenia grave. Se encontraron esquistocitos y microesferocitos en el frotis de sangre periférica con actividad de la enzima ADAMTS 13 disminuida (6,8%). Se confirmó el diagnóstico de una PTT como manifestación inicial de una infección por VIH. Se indicó manejo con plasmaféresis e inicio de TARV con buena respuesta.
Abstract Hematological manifestations for human immunodeficiency virus (HIV) infection are frequent and diverse due to its ability to affect almost all cell lines. Among these, thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathies syndromes, characterized by the presence of thrombocytopenia and microangiopathic hemolytic anemia with impaired renal function. Nowadays, the relationship between these two entities is rare given the current highly active antiretroviral therapy (HAART). We report the case of a 28-year-old patient, who presented with fever associated with gingival bleeding, generalized mucocutaneous pallor and progressive weakness. Routine investigations showed anemia and severe thrombocytopenia, schistocytes and micro spherocytes in peripheral blood smear. Required blood transfusion, with decreased ADAMTS 13 enzyme activity (6.8%). With these findings,TTP was diagnosed as the initial manifestation of the HIV infection. The patient received management with five sessions of plasmapheresis and HAART with subsequent improvement.
Assuntos
Humanos , Masculino , Adulto , Púrpura Trombocitopênica Trombótica , Infecções por HIV , Anemia Hemolítica , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia , Infecções por HIV/complicações , Infecções por HIV/tratamento farmacológico , PlasmafereseRESUMO
A boy, aged 3 years and 8 months, had recurrent thrombocytopenia with hemolytic anemia for more than 3 years. The physical examination showed no enlargement of the liver, spleen, and lymph nodes or finger deformities. Laboratory results showed a negative result of the direct antiglobulin test, normal coagulation function, and increases in bilirubin, lactate dehydrogenase and reticulocytes. The results of von Willebrand factor-cleaving protease ADAMTS13 activity assay showed extreme deficiency, and antibody assay showed negative ADAMTS13 inhibitory autoantibodies. Next-generation sequence showed compound heterozygous mutation in the
Assuntos
Criança , Pré-Escolar , Humanos , Recém-Nascido , Masculino , Proteínas ADAM/genética , Proteína ADAMTS13 , Anemia Hemolítica , Autoanticorpos , Mutação , Púrpura Trombocitopênica TrombóticaRESUMO
El síndrome hemolítico urémico (SHU) típico en adultos es una patología infrecuente. En la literatura se encuentran pocos reportes, y se ha documentado principalmente en la población pediátrica. Esta entidad se caracteriza por ser una microangiopatía trombótica (MAT) que compromete de manera característica los riñones. Es causada usualmente por la infección por Escherichia coli productora de toxina Shiga (STEC), específicamente el serotipo O157:H7. En Colombia no existen casos reportados sobre esta condición en adultos, lo cual llama la atención, pero puede deberse en parte a las dificultades en su diagnóstico, al no tenerse fácil acceso a algunas de las pruebas que orientan hacia esta enfermedad y confirman el diagnóstico. Se reporta el caso de una mujer adulta mayor colombiana, quien consultó por deposiciones diarreicas y hematoquecia, con el posterior desarrollo de trombocitopenia severa, lesión renal aguda, y evidencia de equinocitos y esquistocitos en extendido de sangre periférica, lo que llevó a sospechar una MAT. Se le solicitó FilmArray® gastrointestinal, el cual fue positivo para STEC, confirmando así el diagnóstico de un SHU típico. Se presenta también una breve revisión del tema de una entidad que requiere un diagnóstico temprano y certero que permita brindar un tratamiento eficaz y oportuno
The classic or typical hemolytic uremic syndrome (HUS) in adults is a rare disease. Few reports are found in the literature, and it has mainly been documented in the pediatric population. This condition is a form of thrombotic microangiopathy (TMA), which characteristically compromises the kidneys. It is mainly caused by infection with Shiga toxin-producing Escherichia coli (STEC), specifically the O157:H7 serotype. In Colombia there are no reports on this condition in adults, and may be due in part to difficulties in its diagnosis, as there is not easy access to some of the tests that guide towards this condition and confirm the diagnosis. The case of an elderly Colombian woman is reported, who presented diarrhea and hematochezia, and subsequently developed severe thrombocytopenia and acute kidney injury, with evidence of echinocytes and schistocytes in peripheral blood smears, which led to suspect TMA. A gastrointestinal FilmArray™ was ordered, which was positive for STEC, thus confirming the diagnosis of a typical HUS. A brief literature review is also presented, which covers general concepts of a condition that requires an early and accurate diagnosis in order to provide an effective and timely treatment
Assuntos
Microangiopatias Trombóticas , Trombocitopenia , Toxina Shiga , Diarreia , Escherichia coli , Injúria Renal Aguda , Síndrome Hemolítico-Urêmica , Anemia HemolíticaRESUMO
La microangiopatía trombótica (MAT) es un síndrome donde hay formación de microtrombos en la circulación que llevan a anemia hemolítica microangiopática (AHMA) y trombocitopenia con falla multiorgánica, debido a la isquemia de los tejidos. Las MAT pueden ser primarias sin causa subyacente asociada, como la púrpura trombocitopénica trombótica debida a deficiencia de la enzima ADAMTS13, el síndrome hemolítico urémico debido a la toxina Shiga de Escherichia coli enterohemorrágica, y la MAT producida por alteraciones en la regulación del complemento. Adicionalmente, pueden ser secundarias a enfermedades malignas, infecciosas, metabólicas, autoinmunes o inducidas por el embarazo. Estas patologías requieren diagnóstico y tratamiento oportunos debido a que tienen alta morbimortalidad y se asocian a complicaciones que incluyen enfermedad renal, alteraciones neurológicas como convulsiones, accidente cerebrovascular, coma y muerte. El tratamiento es multidisciplinario y se enfoca en el soporte hemodinámico, transfusional y en el manejo de la etiología cuando esta es identificada. La siguiente revisión pretende explicar de forma clara y precisa los aspectos generales de las MAT primarias
Thrombotic microangiopathy (TMA) is a syndrome characterized by the formation of microthrombi in the circulation leading to microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, with multiorgan failure due to tissue ischemia. TMA can be primary with no associated underlying cause, such as thrombotic thrombocytopenic purpura due to ADAMTS13 deficiency, hemolytic uremic syndrome due to the Shiga toxin from enterohemorrhagic Escherichia coli, or due to complement dysregulation. Furthermore, TMA can be secondary to malignant, infectious, metabolic or autoimmune diseases, or induced by pregnancy. These conditions require a timely diagnosis and treatment due to their associated high morbidity and mortality, and complications like renal disease, neurological disorders such as seizures, stroke, coma and death. Treatment is multidisciplinary and focuses on hemodynamic and transfusion support, and on the management of the etiology when it is identified (daily plasma exchange, eculizumab or management of underlying disease). This review aims to discuss the general aspects of primary thrombotic microangiopathies