Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 770
Filtrar
4.
Cell Physiol Biochem ; 53(3): 453-464, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31448885

RESUMO

BACKGROUND/AIMS: Eryptosis, the suicidal death of red blood cells (RBCs), is characterized by phosphatidylserine (PS) exposure at the cell surface. It can be catalysed by a variety of abnormal conditions and diseases. Until now, the many questions surrounding the physiology and pathophysiology of eryptosis have not been sufficiently answered. Recently, we demonstrated IgM and IgA autoantibodies (aab) to induce PS exposure on circulating RBCs of patients with autoimmune haemolytic anaemia (AIHA). However, it remained unclear how these aab lead to eryptosis. METHODS: Serum and plasma samples from patients with clinically relevant AIHA of cold type were used to induce eryptosis in O RBCs. Serum containing fresh complement from healthy donors, antibodies to complement component, and complement factor depleted sera were added to examine the influence of the complement on PS-exposure. RBC bound annexin V PE were analysed by flow cytometry. RESULTS: Eryptosis related to IgM aab was found to be dependent on complement activation and could be effectively inhibited by EDTA, serum heat inactivation and anti-C5. PS exposure increased with sequential activation of the sublytic terminal complement components C5b6, C5b-7 and was most significant at the C5b-8 stage. A decrease was observed following the formation of the lytic membrane attack complex C5b-9, either because of lysis of eryptotic RBCs or because of inhibition of eryptosis by C9. CONCLUSION: Our findings reflect new aspects on RBC destruction in AIHA as well the impact of the terminal complement complexes on the RBC membrane. The striking differences to nucleated cell apoptosis may even have physiological meaning of RBC acting as a buffer of the complement system.


Assuntos
Anemia Hemolítica Autoimune/patologia , Autoanticorpos/farmacologia , Proteínas do Sistema Complemento/metabolismo , Eriptose/efeitos dos fármacos , Imunoglobulina M/imunologia , Anemia Hemolítica Autoimune/sangue , Ativação do Complemento/efeitos dos fármacos , Complemento C5/metabolismo , Ácido Edético/farmacologia , Eritrócitos/citologia , Eritrócitos/efeitos dos fármacos , Eritrócitos/metabolismo , Humanos , Fosfatidilserinas/farmacologia
6.
Lupus ; 28(8): 986-994, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31246559

RESUMO

OBJECTIVE: We aimed to study the usefulness of serum soluble CD163 (sCD163) as a biomarker for macrophage activation syndrome (MAS) associated with systemic lupus erythematosus (SLE). METHODS: Serum sCD163 levels were retrospectively measured by enzyme-linked immunosorbent assay for SLE patients associated with MAS (SLE-MAS), lupus nephritis (LN), or autoimmune hemolytic anemia (AIHA) and/or immune thrombocytopenia (ITP) and healthy controls (HCs). Posttreatment samples were also evaluated in the available SLE-MAS patients. The associations between serum sCD163 levels and clinical information were statistically analyzed. RESULTS: The serum sCD163 levels in SLE-MAS, LN and SLE-AIHA/ITP groups were significantly higher than those in HCs (n = 17, 29, 13, and 68, respectively; p < 0.01 for all comparisons). In addition, the serum sCD163 levels in the SLE-MAS group were even higher than those in the LN and SLE-AIHA/ITP groups (p < 0.01 for both comparisons). Serum sCD163 levels were correlated with the SLE Disease Activity Index 2000 scores (r = 0.53), whereas they were not correlated with the serum ferritin levels. With the determined cut-off value, the sensitivity and specificity of serum sCD163 for the diagnosis of SLE-MAS were 59% and 86%, respectively. Retesting showed that the serum sCD163 levels decreased significantly following treatment in parallel with disease amelioration in the SLE-MAS group (p < 0.01). CONCLUSIONS: The present study suggests the usefulness of serum sCD163 as a diagnostic and disease-activity biomarker for SLE-associated MAS. Serum sCD163 might also have a different role as a biomarker for SLE-associated MAS than serum ferritin does.


Assuntos
Antígenos CD/sangue , Antígenos de Diferenciação Mielomonocítica/sangue , Lúpus Eritematoso Sistêmico/complicações , Síndrome de Ativação Macrofágica/sangue , Receptores de Superfície Celular/sangue , Adulto , Anemia Hemolítica Autoimune/sangue , Biomarcadores/sangue , Estudos de Casos e Controles , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Lúpus Eritematoso Sistêmico/sangue , Nefrite Lúpica/sangue , Síndrome de Ativação Macrofágica/diagnóstico , Macrófagos/metabolismo , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/sangue , Curva ROC , Estudos Retrospectivos
7.
BMJ Case Rep ; 12(5)2019 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-31118169

RESUMO

A 46-year-old man was admitted to the emergency department with fever and pleuritic thoracic pain. Six weeks prior to admission, the patient had undergone cardiac surgery. The ECG showed diffuse ST segment elevation and PR segment depression. The blood tests revealed increased inflammatory markers and negative myocardial necrosis markers. Pericardial and left-sided pleural effusion were noted. Sterile blood cultures were negative. Hence, the hypothesis of Dressler's syndrome was established. The patient improved clinically and analytically with a short course of anti-inflammatory therapy and was discharged with colchicine and acetylsalicylic acid. A thoracic radiography performed 2 months after showed complete remission of pleural effusion.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/fisiopatologia , Derrame Pericárdico/etiologia , Assistência ao Convalescente , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/tratamento farmacológico , Anti-Inflamatórios/uso terapêutico , Diagnóstico Diferencial , Eletrocardiografia , Humanos , Masculino , Pessoa de Meia-Idade , Infarto do Miocárdio/metabolismo , Derrame Pericárdico/diagnóstico por imagem , Pericardite/diagnóstico por imagem , Pericardite/etiologia , Resultado do Tratamento
8.
J Clin Lab Anal ; 33(4): e22844, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30945356

RESUMO

BACKGROUND: There is currently no single index for the diagnostic screening of hereditary spherocytosis (HS). However, hematology analyzers are widely used in hospital laboratories because of their highly automated performance and quality control procedure, and detection of some blood cell parameters may be useful for the early screening of HS. METHODS: We investigated the values of blood cell parameters for the screening and differential diagnosis of HS. We performed a descriptive study of 482 samples (67 cases of HS, 59 cases of G6PD deficiency, 57 cases of AIHA, 199 cases of thalassemia, and 100 cases of healthy controls) that were run on Beckman Coulter LH780 Hematology Analyzer. RESULTS: HS was characterized by increased MCHC, decreased MRV, MSCV-MCV < 0, and increased Ret with no concomitant increase in IRF. The areas under the ROC curves were MSCV-MCV (0.97; 95% CI 0.95-1.0) > MRV (0.94; 95% CI 0.91-0.97) > MCHC (0.92; 95% CI 0.88-0.97) > Ret/IRF (0.77; 95% CI 0.7-0.84). MSCV-MCV ≤ 0.6 fl was valuable parameter for the diagnostic screening of HS, with a sensitivity of 95.5% and specificity of 94.9%. CONCLUSION: These indices have high reference values for differentiating HS from thalassemia, AIHA, and G6PD deficiency.


Assuntos
Índices de Eritrócitos , Esferocitose Hereditária/sangue , Adolescente , Adulto , Anemia Hemolítica Autoimune/sangue , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Curva ROC , Reticulócitos/patologia , Sensibilidade e Especificidade , Esferocitose Hereditária/diagnóstico , Talassemia/sangue
10.
Am J Hematol ; 94(5): 563-574, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30790338

RESUMO

Immune checkpoint inhibitors (ICPis) are a novel class of immunotherapeutic agents that have revolutionized the treatment of cancer; however, these drugs can also cause a unique spectrum of autoimmune toxicity. Autoimmune hemolytic anemia (AIHA) is a rare, but often severe, complication of ICPis. We identified 14 patients from nine institutions across the United States who developed ICPi-AIHA. The median interval from ICPi initiation to development of AIHA was 55 days (interquartile range [IQR], 22-110 days). Results from the direct antiglobulin test (DAT) were available for 13 of 14 patients: 8 patients (62%) had a positive DAT and 5 (38%) had a negative DAT. The median pretreatment and nadir hemoglobin concentrations were 11.8 g/dL (IQR, 10.2-12.9 g/dL) and 6.3 g/dL (IQR, 6.1-8.0 g/dL), respectively. Four patients (29%) had a preexisting lymphoproliferative disorder, and two (14%) had a positive DAT prior to initiation of ICPi therapy. All patients were treated with glucocorticoids, with three requiring additional immunosuppressive therapy. Complete and partial recoveries of hemoglobin were achieved in 12 (86%) and 2 (14%) patients, respectively. Seven patients (50%) were rechallenged with ICPis, and one (14%) developed recurrent AIHA. Clinical and laboratory features of ICPi-AIHA were similar in DAT positive and negative patients. ICPi-AIHA shares many clinical features with primary AIHA; however, a unique aspect of ICPi-AIHA is a high incidence of DAT negativity. Glucocorticoids are an effective first-line treatment in the majority of patients with ICPi-AIHA, and most patients who are rechallenged with an ICPi do not appear to develop recurrence of AIHA.


Assuntos
Anemia Hemolítica Autoimune , Hemoglobinas/metabolismo , Imunossupressão , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/terapia , Feminino , Glucocorticoides , Humanos , Masculino , Pessoa de Meia-Idade
11.
J Nucl Med Technol ; 47(2): 175-176, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30700537

RESUMO

A 99mTc-red blood cell (RBC)-labeled multigated acquisition is a procedure in which the patient's RBCs are radiolabeled and imaged with electrocardiography-gated cardiac scintigraphy to assess the heart's pumping efficiency. Cold agglutinin disease, or cold antibody autoimmune hemolytic anemia, is a rare form of autoimmune hemolytic anemia in which the body's immune system attacks and destroys its own RBCs. This case addresses an altered biodistribution pattern of radiolabeled RBCs in the presence of suspected cold agglutinin disease observed during a multigated acquisition.


Assuntos
Anemia Hemolítica Autoimune/metabolismo , Eritrócitos/metabolismo , Compostos de Organotecnécio/metabolismo , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Distribuição Tecidual
12.
Am J Hematol ; 94(4): 461-466, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30663792

RESUMO

Immune thrombocytopenia (ITP) is the most common autoimmune cytopenia in children. Approximately, 25% of patients develop chronic disease, which may be unpredictable and challenging to treat. It is not currently possible to predict at the time of presentation which patients will have chronic disease or will experience symptoms requiring second-line therapy defined as treatment beyond corticosteroids, intravenous immunoglobulin, or Rh immune globulin. A multi-institutional retrospective review of 311 pediatric patients with ITP was performed with the goal of identifying clinical characteristics associated with disease course. In a cohort of 216 patients tested and for whom disease status was known, a positive direct antiglobulin test (DAT) was associated with chronic ITP vs spontaneous resolution of disease (29.2% vs 8.1%, P < 0.001) as well as the need for treatment with second line agents (38.5% vs 11.4%, P < 0.001) in 241 patients. Controlling for the effect of Evans syndrome, defined as having two immune cytopenias, a positive DAT was independently associated with chronic ITP (OR = 2.7, 95% CI: 1.0-7.2, P = 0.041) and use of second-line agents (OR: 3.6, 95% CI: 1.7-7.7, P = 0.001) by multivariate logistic regression model. These findings demonstrate an association with positive DAT and chronic disease, as well as refractory disease requiring second-line agents.


Assuntos
Corticosteroides/administração & dosagem , Anemia Hemolítica Autoimune , Teste de Coombs , Imunoglobulinas Intravenosas/administração & dosagem , Púrpura Trombocitopênica Idiopática , Imunoglobulina rho(D)/administração & dosagem , Trombocitopenia , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/tratamento farmacológico , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , Masculino , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Trombocitopenia/sangue , Trombocitopenia/tratamento farmacológico
15.
Intern Med ; 58(7): 999-1002, 2019 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-30568122

RESUMO

A 79-year-old man with Sjögren's syndrome and systemic lupus erythematosus developed acute impaired consciousness and hemolytic anemia. The patient's red blood cells agglutinated spontaneously at 25-37°C. The treatment of red blood cells with 2-mercaptoethanol resulted in the loss of spontaneous agglutination. A diagnosis of IgM-mediated warm autoimmune hemolytic anemia was made. The patient received steroid pulse and plasma exchange therapies. Rituximab was also administered. However, the patient died from multiple organ failure at six days from the symptom onset. The clinical progress of the patient and autopsy findings suggested that complement activation might have been associated with the pathology.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Anticorpos Anti-Idiotípicos/imunologia , Imunoglobulina M/imunologia , Lúpus Eritematoso Sistêmico/complicações , Idoso , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/imunologia , Anticorpos Anti-Idiotípicos/sangue , Autopsia , Evolução Fatal , Humanos , Imunoglobulina M/sangue , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino
16.
Hematology Am Soc Hematol Educ Program ; 2018(1): 382-389, 2018 11 30.
Artigo em Inglês | MEDLINE | ID: mdl-30504336

RESUMO

The diagnosis of autoimmune hemolytic anemia (AIHA) can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis and then determine the immune nature of hemolysis with the direct anti-globulin test. Once alternative causes for these findings have been excluded, AIHA is established, and the clinician must search for secondary causes, as well as identify the type of AIHA. Rituximab is now the preferred second-line treatment for primary warm AIHA and first-line treatment for primary cold agglutinin disease (CAD), either as monotherapy or combined with bendamustine. Complement inhibitors have shown utility in stabilizing AIHA patients with acute severe hemolysis. Future prospects are discussed and include the C1s inhibitor BIVV009 (sutimlimab) that is now entering phase 3 studies for CAD.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/tratamento farmacológico , Inativadores do Complemento/uso terapêutico , Rituximab/uso terapêutico , Anemia Hemolítica Autoimune/sangue , Ensaios Clínicos Fase III como Assunto , Humanos
18.
PLoS One ; 13(11): e0207218, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30408135

RESUMO

Thrombotic manifestations are a hallmark of many auto-immune diseases (AID), specially of warm autoimmune hemolytic anemia (wAIHA), as 15 to 33% of adults with wAIHA experience venous thromboembolic events (VTE). However, beyond the presence of positive antiphospholipid antibodies and splenectomy, risk factors for developing a VTE during wAIHA have not been clearly identified. The aim of this retrospective study was to characterize VTEs during wAIHA and to identify risk factors for VTE. Forty-eight patients with wAIHA were included, among whom 26 (54%) had secondary wAIHA. Eleven (23%) patients presented at least one VTE, that occurred during an active phase of the disease for 10/11 patients (90%). The frequency of VTE was not different between primary and secondary AIHA (23.7 vs. 19.2%; p = 0.5). The Padua prediction score based on traditional risk factors was not different between patients with and without VTE. On multivariate analysis, total bilirubin ≥ 40 µmol/L [odds ratio (OR) = 7.4; p = 0.02] and leucocyte count above 7x10(9)/L (OR = 15.7; p = 0.02) were significantly associated with a higher risk of thrombosis. Antiphospholipid antibodies were screened in 9 out the 11 patients who presented a VTE and were negative. Thus, the frequency of VTE is high (23%) during wAIHA and VTE preferentially occur within the first weeks of diagnosis. As no clinically relevant predictive factors of VTE could be identified, the systematic use of a prophylactic anticoagulation should be recommended in case of active hemolysis and its maintenance after hospital discharge should be considered. The benefit of a systematic screening for VTE and its procedure remain to be determined.


Assuntos
Anemia Hemolítica Autoimune/complicações , Tromboembolia Venosa/etiologia , Adulto , Idoso , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/terapia , Anticoagulantes/uso terapêutico , Bilirrubina/sangue , Feminino , Humanos , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Retrospectivos , Fatores de Risco , Tromboembolia Venosa/sangue , Tromboembolia Venosa/prevenção & controle
19.
BMJ Case Rep ; 20182018 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-30413455

RESUMO

Donath-Landsteiner haemolytic anaemia (DLHA), also known as paroxysmal cold haemoglobinuria, is a very rare and difficult condition to diagnose as well as treat. Here, we present a case of a 55-year-old Hispanic woman who presented with severe intravascular haemolytic anaemia in the setting of a viral illness 2 weeks prior to presentation. Direct antiglobulin testing revealed mixed results: positive for either complement, IgG or both on various occasions which led to a battery of tests including the Donath-Landsteiner antibody testing which turned out positive establishing the diagnosis of DLHA. She was initially treated unsuccessfully with supportive care in the form of packed red blood cell transfusions and steroids as well as rituximab for about 4 weeks but her condition improved on cyclophosphamide, and she is on the road to recovery after 10 weeks of hospital stay.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/tratamento farmacológico , Anemia Hemolítica Autoimune/sangue , Ciclofosfamida/uso terapêutico , Diagnóstico Diferencial , Feminino , Humanos , Imunossupressores/uso terapêutico , Pessoa de Meia-Idade
20.
Transfusion ; 58(12): 2777-2781, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30291762

RESUMO

BACKGROUND: Evans syndrome is a rare autoimmune disorder that is defined by the simultaneous or sequential presence of two or more cytopenias without an obvious underlying precipitating cause. Evans syndrome usually follows a chronic relapsing and remitting course and is quite rare, making it difficult to evaluate in clinical studies. CASE REPORT: A 66-year-old male patient with a 17-year history of Evans syndrome presented with fulminant autoimmune hemolytic anemia (AIHA). He presented with a markedly elevated C-reactive protein (CRP; 46 mg/L [normal, 0-5 mg/L]) before onset of a decrease in hemoglobin. He required the transfusion of 20 units of red blood cells while awaiting response to aggressive immunosuppressive therapy including high-dose corticosteroids, intravenous immunoglobin therapy, and rituximab. He achieved a complete hematologic response. RESULTS: His postdischarge course was complicated by acute cholecystitis requiring laparoscopic cholecystectomy. In addition, his transfusional iron overload requiring 16 phlebotomies to reduce his ferritin level from 4933 µg/L to 326 µg/L, with phlebotomies ongoing every 2 weeks to achieve a ferritin level of less than 100 µg/L. CONCLUSION: Neither transfusional iron overload nor acute cholecystitis are well-recognized complications of a severe episode of AIHA. An elevated CRP has been recently recognized as an important prognostic marker in patients with immune thrombocytopenic purpura and this case suggests a need to evaluate its utility in AIHA.


Assuntos
Corticosteroides/administração & dosagem , Anemia Hemolítica Autoimune , Colecistite , Transfusão de Eritrócitos , Imunoglobulinas Intravenosas/administração & dosagem , Sobrecarga de Ferro , Rituximab/administração & dosagem , Trombocitopenia , Reação Transfusional , Idoso , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/complicações , Anemia Hemolítica Autoimune/terapia , Colecistite/sangue , Colecistite/complicações , Colecistite/patologia , Colecistite/terapia , Gangrena , Humanos , Sobrecarga de Ferro/sangue , Sobrecarga de Ferro/tratamento farmacológico , Sobrecarga de Ferro/etiologia , Sobrecarga de Ferro/patologia , Masculino , Trombocitopenia/sangue , Trombocitopenia/complicações , Trombocitopenia/terapia , Reação Transfusional/sangue , Reação Transfusional/tratamento farmacológico
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA