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1.
Rev. bras. anal. clin ; 53(3): 245-251, 20210930. graf, tab
Article in Portuguese | LILACS | ID: biblio-1368582

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Aged , Young Adult , Osmotic Fragility , Anemia, Hemolytic , Hemolysis
2.
Arch. argent. pediatr ; 119(4): e326-e329, agosto 2021. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1281753

ABSTRACT

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.


Subject(s)
Humans , Female , Infant , Vitamin B 12 Deficiency/complications , Thrombotic Microangiopathies/diagnosis , Vitamin B 12 Deficiency/therapy , Anemia, Hemolytic/blood
3.
Arq. bras. med. vet. zootec. (Online) ; 73(2): 335-342, Mar.-Apr. 2021. tab, ilus
Article in English | LILACS, VETINDEX | ID: biblio-1248935

ABSTRACT

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)


Subject(s)
Animals , Dogs , Immunosuppressive Agents/therapeutic use , Anemia, Hemolytic/complications , Anemia, Hemolytic/veterinary , Mycophenolic Acid/analysis , Mycophenolic Acid/adverse effects , Thrombelastography/veterinary , Ehrlichia canis , Erythrocyte Count/veterinary , Hemostasis
4.
Med. lab ; 25(2): 485-499, 2021. tabs
Article in Spanish | LILACS | ID: biblio-1293234

ABSTRACT

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


Subject(s)
Thrombotic Microangiopathies , Purpura, Thrombotic Thrombocytopenic , Thrombocytopenia , Atypical Hemolytic Uremic Syndrome , Hemolytic-Uremic Syndrome , Anemia, Hemolytic
5.
Med. lab ; 25(2): 535-546, 2021. tabs, graf
Article in Spanish | LILACS | ID: biblio-1342897

ABSTRACT

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


Subject(s)
Thrombotic Microangiopathies , Thrombocytopenia , Shiga Toxin , Diarrhea , Escherichia coli , Acute Kidney Injury , Hemolytic-Uremic Syndrome , Anemia, Hemolytic
6.
Article in Chinese | WPRIM | ID: wpr-879888

ABSTRACT

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


Subject(s)
ADAM Proteins/genetics , ADAMTS13 Protein , Anemia, Hemolytic , Autoantibodies , Child , Child, Preschool , Humans , Infant, Newborn , Male , Mutation , Purpura, Thrombotic Thrombocytopenic
7.
Acta bioquím. clín. latinoam ; 54(4): 437-453, jul. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1149033

ABSTRACT

Resumen El sistema del complemento juega un papel central en la inmunidad innata, es una línea de defensa contra patógenos y participa en la homeostasis. La activación anormal del complemento contribuye al desarrollo de patologías de variable severidad, tanto inmunológicas y hematológicas como renales. Entre ellas, las microangiopatías trombóticas (MAT) representan un grupo de enfermedades raras con manifestaciones clínicas comunes caracterizadas por anemia hemolítica no inmune, trombocitopenia y daño de órgano(s) blanco. Si bien la clasificación de las MAT sigue siendo desafiante y no ha sido internacionalmente estandarizada, la descripción de entidades asociadas a anomalías del complemento fue comprobada con la eficiencia de la terapia anticomplemento en los pacientes. Las herramientas de diagnóstico desarrolladas en las últimas décadas son esenciales actualmente para diferenciar las MAT más características del grupo; esto es, la púrpura trombótica trombocitopénica (PTT) y el síndrome urémico hemolítico (SUH). En el presente trabajo se presenta una revisión del funcionamiento del sistema del complemento en condiciones fisiológicas, para poder explicar luego cuáles son las alteraciones del sistema implicadas en el desarrollo de las MAT y describir las herramientas disponibles para detectarlas en el laboratorio.


Abstract The complement system plays a crucial role in the innate immune response, being the first-line defense against pathogens and regulating homeostasis. Uncontrolled complement activation can cause immunologic, hematologic as well as renal syndromes of variable severity. Among them, thrombotic microangiopathies (TMA) represent a group of rare diseases characterised by similar clinical manifestations such as microangiopathic hemolytic anemia (MAHA), peripheral thrombocytopenia and organ injury. Although TMA classification is still challenging and no international consensus has been reached, complement-associated disorders have been described thanks to the efficiency of anti-complement therapy in patients. Diagnostic tools developed in the last decades are essential to differentiate the two most well characterized TMA: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). This review will describe how the complement system works in physiological conditions in order to explain how complement abnormalities are involved in TMA, and finally how to detect those anomalies using laboratory tests.


Resumo O sistema do complemento desempenha um papel central na imunidade inata, sendo uma linha de defesa contra patógenos e participando da homeostase. A ativação anormal do complemento contribui para o desenvolvimento de patologias de gravidade variável, como imunológicas, hematológicas e renais. Entre elas, as microangiopatias trombóticas (MAT) representam um grupo de doenças raras com manifestações clínicas comuns caracterizadas por anemia hemolítica não imune, trombocitopenia e lesão de órgão(s) alvo. Embora a classificação das MAT continue sendo desafiadora e não tenha sido padronizada internacionalmente, a descrição de entidades associadas a anomalias do complemento foi comprovada com a eficiência da terapia anticomplemento nos pacientes. As ferramentas de diagnóstico desenvolvidas nas últimas décadas são atualmente essenciais para diferenciar as MAT mais características do grupo, que são a púrpura trombocitopênica trombótica (PTT) e a síndrome hemolítica urêmica atípica (SHU). Neste trabalho, é apresentada uma revisão do funcionamento do sistema de complemento em condições fisiológicas, a fim de explicar posteriormente quais são as alterações do sistema compreendidas no desenvolvimento das MAT, e descrever as ferramentas disponíveis para detectá-las em laboratório.


Subject(s)
Humans , Biomarkers/analysis , Complement Activation/physiology , Thrombotic Microangiopathies/diagnosis , Thrombocytopenia/diagnosis , Atypical Hemolytic Uremic Syndrome/diagnosis , Homeostasis , Anemia, Hemolytic/diagnosis
8.
Arch. argent. pediatr ; 118(3): e305-e308, jun. 2020. tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1116994

ABSTRACT

El síndrome urémico hemolítico asociado a Streptococcus pneumoniae (SUH-Sp) se define como anemia hemolítica microangiopática, plaquetopenia y lesión renal aguda, en un paciente con infección invasiva por Streptococcus pneumoniae (Sp). Varón de 2 años, con neumonía con derrame pleural por Sp aislado en hemocultivos y líquido pleural. A las 72 h, presentó palidez, decaimiento, quejido respiratorio y oliguria. En el análisis de laboratorio se encontró anemia, plaquetopenia, aumento de la urea, la creatinina y la lactato deshidrogenasa en sangre; coombs directa +; esquistocitos en frotis; fibrinógeno; coagulograma normal; dímero D aumentado. Orina con proteinuria y hematuria. En Terapia Intensiva requirió asistencia respiratoria mecánica y transfusión con glóbulos rojos lavados; se recuperó progresivamente. El Instituto Malbrán informó serotipo 38 de Sp. Es el primer paciente comunicado con este serotipo


Streptococcus pneumoniae associated hemolytic uremic syndrome (Sp-HUS) is defined as microangiopathic hemolytic anemia, thrombocytopenia and acute renal injury, in a patient with Streptococcus pneumoniae (Sp) invasive infection. A 2-year-old boy was admitted with pneumonia and empyema. Sp was isolated from blood and pleural fluid cultures. After 72 h, the patient showed paleness, asthenia, respiratory whining and oliguria. Laboratory showed anemia, low platelets, increased blood urea, creatirnina, lactate dehdrogenase, direct Coombs +, schistocytes, fibrinogen, normal coagulogram and increased D-dimer. Proteinuria and hematuria were detected in urine. Mechanical ventilatory assistance and transfusions of washed red blood cells were required. The patient recovered progressively. Sp serotype 38 was isolated in the National Reference Laboratory "Malbran". This is the first report associated to this serotype


Subject(s)
Humans , Male , Child, Preschool , Hemolytic-Uremic Syndrome , Pneumonia , Respiratory Insufficiency , Streptococcus pneumoniae , Renal Insufficiency , Anemia, Hemolytic
9.
Acta méd. colomb ; 45(1): 40-43, Jan.-Mar. 2020. tab
Article in English | LILACS, COLNAL | ID: biblio-1124069

ABSTRACT

Abstract Wilson's disease is a rare genetic disorder that affects the excretion capacity of copper. Its distribution is worldwide, with an estimated prevalence in 30 cases per million habitants. Although the most frequent symptoms are those of hepatic and neuropsychiatric origin, hemolytic anemia with negative Coombs may be the only manifestation of the disease and its presentation usually precedes for months to clinically evident liver disease or neurological manifestations. The case of a young patient with negative Coombs hemolytic anemia and an alkaline phosphatase / total bilirubin ratio <4 and AST / ALT> 2.2 is presented, establishing Wilson's disease as a diagnosis. (Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1459).


Resumen La enfermedad de Wilson es un raro trastorno genético que afecta la capacidad de excreción del cobre. Su distribución es mundial, con una prevalencia estimada en 30 casos por millón de habitantes. Aunque los síntomas más frecuentes son los de origen hepático y neuropsiquiatricos, la anemia hemolítica con Coombs negativo puede ser la única manifestación de la enfermedad y su presentación suele preceder por meses a la enfermedad hepática clínicamente evidente o las manifestaciones neurológicas. Se presenta el caso de una paciente joven con anemia hemolítica Coombs negativo y relación fosfatasa alcalina/bilirrubina total <4 y AST/ALT >2,2, en quien terminó por establecerse como diagnóstico una enfermedad de Wilson.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1459).


Subject(s)
Humans , Adolescent , Anemia, Hemolytic , Coombs Test , Hemolysis , Hepatolenticular Degeneration
11.
Article in English | WPRIM | ID: wpr-811412

ABSTRACT

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.


Subject(s)
Anemia, Hemolytic , Anemia, Hemolytic, Autoimmune , Biopsy , Giant Cells , Hepatitis , Hepatocytes , Humans , Infant , Inflammation , Jaundice , Liver , Liver Diseases , Male , Pallor , Physical Examination , Rare Diseases , Recurrence , Rituximab , Splenomegaly
12.
J. bras. nefrol ; 41(4): 580-584, Out.-Dec. 2019. graf
Article in English | LILACS | ID: biblio-1056602

ABSTRACT

Abstract In kidney biopsies reviews, scleroderma renal crisis (SRC) is characterized by vascular endothelial injuries, C4d deposits on peritubular vessels, and acute and chronic injuries coexisting on the same biopsy. The clinical signs of thrombotic microangiopathy (TMA) are described in systemic sclerosis (SSc), nevertheless, it has not been related to acute injuries described on kidney biopsies. We report a case of SRC in a patient with scleroderma-dermatomyositis overlap syndrome, which also showed clinical and histopathological data of TMA. On fundus examination, a severe acute hypertensive retinopathy was found. The kidney biopsy showed severe endothelial damage with widening of mucoid cells at the level of the intima, focal concentric proliferation on most small arterioles, and C3, C4d, and IgM deposits along the capillary walls. The genetic study of complement only showed the presence of membrane cofactor protein (MCP) risk haplotypes, without other genetic complement disorders. We understand that in a patient with TMA and SSc, the kidney damage would be fundamentally endothelial and of an acute type; moreover, we would observe clear evidence of complement activation. Once further studies correlate clinical-analytical data with anatomopathological studies, it is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation.


Resumo Nas revisões de biópsias renais, a crise renal esclerodérmica (CRE) é caracterizada por lesões endoteliais vasculares, depósitos de C4d em vasos peritubulares e lesões agudas e crônicas que coexistem na mesma biópsia. Os sinais clínicos de microangiopatia trombótica (MAT) são descritos na esclerose sistêmica (ES); no entanto, não foram relacionados às lesões agudas descritas nas biópsias renais. Relatamos um caso de CRE em um paciente com síndrome de superposição de esclerodermia-dermatomiosite, que também apresentou dados clínicos e histopatológicos de MAT. No exame de fundo do olho, foi encontrada uma retinopatia hipertensiva aguda grave. A biópsia renal mostrou lesão endotelial grave com alargamento das células mucoides ao nível da íntima, proliferação concêntrica focal na maioria das pequenas arteríolas e depósitos de C3, C4d e IgM ao longo das paredes dos capilares. O estudo genético do complemento mostrou apenas a presença de haplótipos de risco da proteína cofator de membrana (PCM), sem outros distúrbios genéticos do complemento. Entendemos que em um paciente com MAT e ES, o dano renal seria fundamentalmente endotelial e do tipo agudo; além disso, observaríamos evidências claras de ativação do complemento. Uma vez que novos estudos correlacionam dados clínico-analíticos com estudos anatomopatológicos, é provável que sejamos forçados a redefinir o conceito de CRE, enfocando a relação entre dano endotelial agudo e ativação do complemento.


Subject(s)
Humans , Male , Middle Aged , Raynaud Disease/complications , Vision Disorders/etiology , Acute Kidney Injury/etiology , Kidney/blood supply , Capillaries/metabolism , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Immunohistochemistry , Papilledema/pathology , Dermatomyositis/complications , Dermatomyositis/immunology , Hypertensive Retinopathy/diagnosis , Hypertensive Retinopathy/pathology , Hypertensive Retinopathy/drug therapy , Acute Kidney Injury/diagnosis , Anemia, Hemolytic/diagnosis , Anemia, Hemolytic/etiology , Kidney/pathology , Kidney/diagnostic imaging
13.
Biosci. j. (Online) ; 35(6): 1979-1984, nov./dec. 2019. ilus, tab
Article in English | LILACS | ID: biblio-1049178

ABSTRACT

G6PD deficiency is associated with erythrocyte deficiency in the X-chromosome enzyme. It causes a hematologic syndrome called hemolytic anemia that connects G6PD deficiency with X-linked condition. In the Middle East, including Saudi Arabia, G6PD deficiency is the most dominant genetic blood disorders. It results in higher rates of mortality and morbidity due to its incurable long-lasting nature and prevalence of physical and psychological incapacities. In this study, an attempt was made to evaluate the prevalence of G6PD deficiency among the Saudi population in Riyadh city. A cross-sectional retrospective study was conducted at King Saud University Medical City in Riyadh, Saudi Arabia. The population of the study comprised randomly chosen males and females who visited the hospital from January 2017 to January 2018. Statistical analyses were performed using SPSS, and descriptive analysis was used to find the frequency of G6PD-deficient patients. Out of the 209 patients, 62.2% were males (n=130) and 37.8% were females (n=79). Twenty males and 6 females were found to have G6PD deficiency, with the male to female ratio being 1:3. Out of the total 130 male participants, 20 patients were found to be enzyme deficient and 6 patients of 79 female patients were found to be G6PD deficient. There were 38.4% (n=10) patients with G6PD level <4 units/gram hemoglobin, 26.9% (n=7) patients had G6PD levels of 4.1­7.0 units/gram hemoglobin, and 34.6% (n=9) patients had >7 units/gram hemoglobin. Among the G6PD patients, 23.07% patients were severely anemic, and 5 (19.2%) patients were reported to have high bilirubin. The present study revealed the G6PD prevalence to be 12.4% among the Saudi population; this value is significantly higher than that found in France, Spain, India, and Singapore. In the Saudi population, males are more vulnerable to G6PD-deficient than females. Hence, attention should be paid to G6PD-deficient patients while prescribing antimalarial medication. Such patients may be advised to avoid certain foods to minimize the risk of having hemolytic episodes.


A deficiência de G6PD está associada à deficiência de eritrócitos na enzima do cromossomo X. Causa uma síndrome hematológica chamada anemia hemolítica que conecta a deficiência de G6PD à condição ligada ao X. No Oriente Médio, incluindo a Arábia Saudita, a deficiência de G6PD é o distúrbio genético do sangue mais dominante. Isso resulta em maiores taxas de mortalidade e morbidade devido à sua natureza incurável e duradoura e à prevalência de incapacidades físicas e psicológicas. Neste estudo, foi feita uma tentativa de avaliar a prevalência de deficiência de G6PD entre a população saudita na cidade de Riade. Um estudo retrospectivo transversal foi realizado na cidade médica da Universidade King Saud, em Riade, na Arábia Saudita. A população do estudo compreendeu homens e mulheres escolhidos aleatoriamente que visitaram o hospital entre janeiro de 2017 e janeiro de 2018. As análises estatísticas foram realizadas com o SPSS e a análise descritiva foi utilizada para determinar a frequência de pacientes com deficiência de G6PD. Dos 209 pacientes, 62,2% eram do sexo masculino (n = 130) e 37,8% eram do sexo feminino (n = 79). Verificou-se que 20 homens e 6 mulheres apresentavam deficiência de G6PD, sendo a proporção homem/mulher de 1:3. Do total de 130 participantes do sexo masculino, 20 pacientes apresentaram deficiência de enzima e 6 de 79 pacientes do sexo feminino apresentaram deficiência de G6PD. Havia 38,4% (n = 10) pacientes com nível de G6PD < 4 unidades/grama de hemoglobina, 26,9% (n = 7) pacientes tinham níveis de G6PD de 4,1-7,0 unidades/grama de hemoglobina e 34,6% (n = 9) pacientes tinham > 7 unidades/grama de hemoglobina. Entre os pacientes com G6PD, 23,07% eram severamente anêmicos e cinco (19,2%) pacientes relataram ter alta bilirrubina. O presente estudo revelou que a prevalência de G6PD é de 12,4% na população saudita; esse valor é significativamente maior que o encontrado na França, Espanha, Índia e Cingapura. Na população saudita, os homens são mais vulneráveis à deficiência de G6PD do que as mulheres. Portanto, deve-se prestar atenção aos pacientes com deficiência de G6PD durante a prescrição de medicamentos antimaláricos. Esses pacientes podem ser aconselhados a evitar certos alimentos para minimizar o risco de episódios hemolíticos.


Subject(s)
Glucosephosphate Dehydrogenase , Hemolysis , Anemia, Hemolytic
14.
Rev. bras. anal. clin ; 51(3): 253-256, 20190930.
Article in Portuguese | LILACS | ID: biblio-1047664

ABSTRACT

Um dos quadros clínicos mais comumente encontrado em pacientes internados em serviços de referência é a anemia. Entretanto, ela não é caracterizada como uma doença e sim como consequência de um evento ou de uma patologia. Dentre as diversas manifestações existentes pode-se citar a anemia hemolítica (AH) autoimune, que consiste em um grupo de doenças cuja característica comum é a presença de autoanticorpos, os quais se ligam aos eritrócitos e diminuem o tempo de sobrevida dessas células, sendo um dos eventos autoimunes mais comuns associados a esta condição. Dentre os casos graves está a Síndrome Hematofagocítica ou Linfohistiocitose (SHF), que é uma síndrome rara e potencialmente fatal. Na última década, a SHF tem adquirido notoriedade devido ao aumento da sua incidência. Porém, nem sempre quadro anêmico está relacionado a somente uma causa. Sendo assim, o presente estudo descreverá um caso de anemia hemolítica em uma criança atendida em um hospital universitário, onde se pode apontar mais de uma causa para o quadro hemolítico.


The most commonly clinical event encouterd in patients admitted requently in the hospital are anemia. However, this condition is not characterize as a disease but as consequence of an event or a pathology. Among the anemia we can emphasize autoimmune hemolytic (AH), that consist in a group of diseases whose common feature is the presence of autoantibodies. This tend to bind to erythrocytes and decrease the survival time of these cells. That mechanism is the one of the most common autoimmune events associated with this condition. Between the serious cases is the the Hematophagocytic Syndrome or Lymphohistiocytosis (SHF), which is a rare and potentially fatal syndrome. In the last decade, this syndrome has acquired notoriety due to the increase in its incidence. However, not always an anemic event was related of only one cause. Thus, this study will describe a case of SHF and AH in a child attended at University Hospital of The West of Paraná, in this case we cleary indicated more than one cause for the hemolytic conditions.


Subject(s)
Humans , Female , Infant , Child , Lymphohistiocytosis, Hemophagocytic , Anemia, Hemolytic
15.
Rev. colomb. gastroenterol ; 34(1): 91-96, ene.-mar. 2019.
Article in Spanish | LILACS | ID: biblio-1003844

ABSTRACT

Resumen La trombosis venosa portal secundaria a esplenectomía es una patología frecuente. En pacientes llevados a este procedimiento por anemia hemolítica, varios factores de riesgo locales y sistémicos contribuyen a su presentación. Esta complicación es potencialmente mortal y ha existido gran discusión sobre las intervenciones adecuadas para su prevención y tratamiento. Frecuentemente se desconoce esta alteración. Se presenta un caso y se hace una revisión del tema.


Abstract Portal vein thrombosis is a frequently occurring pathology following splenectomy. Several local and systemic risk factors contribute to its occurrence among patients who undergo this procedure to treat hemolytic anemia. This potentially fatal complication has caused great discussion about appropriate interventions for its prevention and treatment. Frequently this alteration is undetected. We present a case and review the topic.


Subject(s)
Humans , Female , Adult , Splenectomy , Venous Thrombosis , Anemia, Hemolytic , Patients , Portal Vein , Literature
16.
Laboratory Medicine Online ; : 249-253, 2019.
Article in Korean | WPRIM | ID: wpr-760509

ABSTRACT

A 22-year old female patient with systemic lupus erythematosus presenting microangiopathic hemolytic anemia was treated with therapeutic plasma exchange 23 times. The patient's condition and laboratory findings (aspartate aminotransferase, alanine aminotransferase, ferritin, total bilirubin, and lactate dehydrogenase) did not improve despite the initial 18 therapeutic plasma exchange treatments. Thrombotic thrombocytopenic purpura was ruled out due to normal ADAMTS-13 activity test result; hemophagocytic lymphohistiocytosis was diagnosed based on fever, splenomegaly, pancytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow aspiration. The patient's condition improved rapidly upon treatment with a combination of immunosuppressants and cytotoxic agents, and more therapeutic plasma exchanges were performed five consecutive times with prolonged intervals in between. We observed that therapeutic plasma exchange treatment alone was not effective enough to treat hemophagocytic lymphohistiocytosis, unlike thrombotic thrombocytopenic purpura. Therefore, it is necessary to determine and start drug administration promptly in the treatment of hemophagocytic lymphohistiocytosis with thrombotic microangiopathy.


Subject(s)
Alanine Transaminase , Anemia, Hemolytic , Bilirubin , Bone Marrow , Cytotoxins , Female , Ferritins , Fever , Humans , Hypertriglyceridemia , Immunosuppressive Agents , Lactic Acid , Lupus Erythematosus, Systemic , Lymphohistiocytosis, Hemophagocytic , Pancytopenia , Plasma Exchange , Plasma , Purpura, Thrombotic Thrombocytopenic , Splenomegaly , Thrombotic Microangiopathies
17.
Korean Journal of Medicine ; : 303-307, 2019.
Article in Korean | WPRIM | ID: wpr-759930

ABSTRACT

Infliximab (IFX) is an anti-tumor necrosis factor (TNF) monoclonal antibody used to treat rheumatoid arthritis, ankylosing spondylitis, and Crohn's disease. Rarely, anti-TNF-induced lupus (ATIL) may occur. ATIL differs from classical drug-induced lupus. We report a 49-year-old woman who developed polyarthralgia after 2 years of IFX treatment for Crohn's disease. Based on the autoantibody profiles, ATIL was diagnosed and low-dose glucocorticoid, hydroxychloroquine, and celecoxib were prescribed. However, arthralgia and hemolytic anemia developed. Because the anti-dsDNA titers waxed and waned, she was switched to vedolizumab, a monoclonal antibody to the human lymphocyte α4β7 integrin. Six months after switching treatment, the arthralgia had improved and the anti-dsDNA antibody normalized. Here, we report a case of ATIL that resolved after switching from infliximab to vedolizumab.


Subject(s)
Anemia, Hemolytic , Arthralgia , Arthritis, Rheumatoid , Celecoxib , Crohn Disease , Female , Humans , Hydroxychloroquine , Infliximab , Lupus Erythematosus, Systemic , Lymphocytes , Middle Aged , Necrosis , Spondylitis, Ankylosing
18.
Article in Chinese | WPRIM | ID: wpr-773116

ABSTRACT

In this paper,ultra performance liquid chromatography coupled with time-of-flight mass spectrometry( UPLC-Q-TOFMS) technique was used to study the effects of steamed notoginseng on endogenous markers in plasma of rats with hemolytic anemia induced by N-acetyl phenyl hydrazine( APH). The aim was to find out the potential biomarkers and possible blood enriching mechanism of steamed notoginseng on hemolytic anemia rats. In the experiment,steamed notoginseng medicine pair( steamed notoginseng-ginseng)and compound medicines( Sanqi Yangxue Capsules) were used respectively to intervene in APH-induced hemolytic anemia model rats.Then blood routine indexes such as red blood cells( RBC),hemoglobin( Hb) and related organ indexes were determined. As compared with the blank group,the RBC and Hb levels in the model group were substantially decreased( P< 0. 01),while the liver and spleen organ indexes were increased( P< 0. 05). The results of blood routine and organ index demonstrated that the blood deficiency model was successfully established. Steamed notoginseng can significantly increase the RBC level of rats( P<0. 01),and the related indicators of each drug group had a trend of returning to normal levels,verifying the blood enriching effect of steamed notoginseng. The UPLC-Q-TOF-MS technique,principal component analysis( PCA) and partial least squares-discrimination analysis( PLS-DA) were used to analyze the metabolic profiles between the normal group and the model group. Twenty-six potential biomarkers for hemolytic anemia were screened in plasma. Nine metabolites such as retinol,L-valine,and arachidonic acid were down-regulated in the blood deficiency rats,and 17 metabolites such as protoporphyrin Ⅸ and niacinamide were up-regulated. The metabolic level of biomarkers could be changed to a normal state after rats were given with steamed notoginseng,drug pairs,and compound prescriptions. It can be speculated that steamed notoginseng may play a role of blood tonifying by improving biosynthesis of valine,leucine and isoleucine,as well as metabolic pathways such as retinol metabolism and arachidonic acid metabolism.


Subject(s)
Anemia, Hemolytic , Drug Therapy , Animals , Biomarkers , Drugs, Chinese Herbal , Pharmacology , Mass Spectrometry , Metabolome , Metabolomics , Panax notoginseng , Chemistry , Rats , Steam
19.
Article in English | WPRIM | ID: wpr-763376

ABSTRACT

BACKGROUND/AIMS: L-carnitine not only alleviates hyperammonemia and reduces muscle cramps in patients with liver cirrhosis, but also improves anemia in patients with chronic hepatitis and renal dysfunction. This study prospectively evaluated the preventative efficacy of L-carnitine supplementation against hemolytic anemia during antiviral treatment using ribavirin in patients with hepatitis C virus (HCV)-related chronic liver disease. METHODS: A total of 41 patients with chronic hepatitis were consecutively enrolled in this study. Group A (n=22) received sofosbuvir plus ribavirin for 3 months, whereas group B (n=19) was treated with sofosbuvir, ribavirin, and L-carnitine. Hemoglobin concentration changes, the effects of antiviral treatment, and the health status of patients were analyzed using short form-8 questionnaires. RESULTS: A significantly smaller decrease in hemoglobin concentration was observed in group B compared to group A at every time point. Moreover, the prescribed dose intensity of ribavirin in group B was higher than that of group A, resulting in a higher ratio of sustained virological response (SVR) 24 in group B compared with group A. The physical function of patients in group B was also significantly improved compared to group A at the end of antiviral treatment. CONCLUSIONS: L-carnitine supplementation alleviates ribavirin-induced hemolytic anemia in patients with HCV and helps relieve the physical burden of treatment with ribavirin-containing regimens. These advantages significantly increase the likelihood of achieving SVR.


Subject(s)
Anemia , Anemia, Hemolytic , Carnitine , Drug Therapy , Hepacivirus , Hepatitis C , Hepatitis C, Chronic , Hepatitis , Hepatitis, Chronic , Humans , Hyperammonemia , Liver Cirrhosis , Liver Diseases , Muscle Cramp , Prospective Studies , Ribavirin , Sofosbuvir
20.
Article in English | WPRIM | ID: wpr-763264

ABSTRACT

Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (anti-CFH-aHUS) primarily affects children aged 9–13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFH-aHUS) in Laos.


Subject(s)
Adolescent , Anemia, Hemolytic , Atypical Hemolytic Uremic Syndrome , Autoantibodies , Child , Complement C3 , Complement C4 , Complement Factor H , Complement Pathway, Alternative , Female , Humans , Immunosuppression , Kidney , Laos , Plasma , Shiga-Toxigenic Escherichia coli , Thrombocytopenia , Thrombotic Microangiopathies
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