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1.
Medicine (Baltimore) ; 99(3): e18856, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32011506

RESUMO

RATIONALE: Primary biliary cholangitis (PBC) is a rare autoimmune cholestatic liver disease. It is often associated with extrahepatic autoimmune disorders. However, the concurrence of PBC and Sjögren syndrome (SS) with the subsequent onset of autoimmune hemolytic anemia (AIHA) is extremely rare. PATIENT CONCERNS: This study investigated a 60-year-old woman admitted to our hospital with complaints of xerostomia for 5 years, pruritus for 3 years, and abnormal liver function for 3 months. DIAGNOSES: The patient was suffering from typical clinical PBC and SS, and developed decompensated liver cirrhosis after 32 months of ursodeoxycholic acid (UDCA) therapy. In May 2018, she was readmitted to the hospital with a high fever of 39 °C, coughing, and sever fatigue without remission after 3 days of cephalosporin antibiotic therapy. During the clinical course of PBC, her antimitochondrial antibodies (AMA) titers fluctuated from 1:1000 to negative and then to weakly positive, determined by indirect immunofluorescence (IIF), immunoblotting, and enzyme-linked immunosorbent assay (ELISA) based on recombinant mitochondrial antigens; furthermore, her titers of anti-gp210, an antinuclear antibody (ANA), increased sharply. Laboratory tests and imaging were performed to diagnose PBC and SS in September 2015. However, she was subsequently diagnosed with AIHA after 32 months of UDCA therapy based on the identification of pancytopenia, increased reticulocyte (RET) count, and a positive result from the direct Coombs test. INTERVENTIONS: UDCA, hepatic protectant, albumin infusion, chest drainage, rational antibiotic use, diuretics, and methylprednisolone were used to treat the patient. OUTCOMES: Liver cirrhosis was complicated by the development of AIHA, which became severe at 42 months of follow-up. LESSONS: This is the first case report showing a patient with comorbid PBC and SS, as well as the sequential development of AIHA with decreased AMA and increased anti-gp210 titers; this may have been due to immunodeficiency. These findings stress the importance of the serological screening of ANA profile, as well as repeated measurement of ANA and AMA to track PBC progression and prognosis.


Assuntos
Anemia Hemolítica Autoimune/imunologia , Autoanticorpos/imunologia , Colangite/imunologia , Mitocôndrias/imunologia , Complexo de Proteínas Formadoras de Poros Nucleares/imunologia , Síndrome de Sjogren/imunologia , Anemia Hemolítica Autoimune/terapia , Autoanticorpos/sangue , Colangite/terapia , Terapia Combinada , Feminino , Humanos , Cirrose Hepática Biliar/imunologia , Cirrose Hepática Biliar/terapia , Testes de Função Hepática , Pessoa de Meia-Idade , Complexo de Proteínas Formadoras de Poros Nucleares/sangue , Síndrome de Sjogren/terapia
2.
Rev Port Cir Cardiotorac Vasc ; 26(3): 223-224, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31734976

RESUMO

Cold agglutinins (CA) are autoantibodies whose clinical significance depends upon titer and thermal amplitude. Patients, which undergo cardio-pulmonary bypass and especially hypothermic cardioplegia myocardial protection, represent a challenge regarding operative management, as tissue temperature should be maintained above the threshold of agglutination. We report on a case in which the presence of CA was discovered during elective aortic valve replacement surgery, and managed with normothermic cardiopulmonary bypass and continuous retrograde warm blood cardioplegia administration.


Assuntos
Anemia Hemolítica Autoimune/complicações , Ponte Cardiopulmonar/métodos , Parada Cardíaca Induzida/métodos , Doenças das Valvas Cardíacas/cirurgia , Hipotermia Induzida/efeitos adversos , Anemia Hemolítica Autoimune/imunologia , Valva Aórtica/cirurgia , Autoanticorpos/efeitos adversos , Ponte Cardiopulmonar/efeitos adversos , Crioglobulinas/efeitos adversos , Procedimentos Cirúrgicos Eletivos , Parada Cardíaca Induzida/efeitos adversos , Doenças das Valvas Cardíacas/complicações , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Humanos
3.
Hematology ; 24(1): 588-595, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31392938

RESUMO

Objective: To explore the activity of B subsets from Autoimmune Hemolytic Anemia/Evans syndrome (AIHA/ES) patients. Methods: The expression of Bruton's tyrosine kinase (BTK) and phosphorylated BTK (p-BTK) on CD5+CD19+B and CD5-CD19+B lymphocytes were detected using flow cytometry in AIHA/ES patients with different disease states, healthy controls (HCs) and chronic lymphocytic leukemia (CLL) patients. The correlations of expressed BTK and p-BTK with clinical variables were analyzed. Results: Thirty six AIHA/ES patients (16 hemolytic, 20 remission), 11 CLL patients, and 15 HCs were enrolled. The expression levels of BTK and p-BTK on CD5+B lymphocytes in AIHA/ES patients were higher than those in HCs and CLL patients. The latter two groups had no significant difference, and were positively correlated with the quantity of IgE. The ratio of p-BTK to BTK on CD5+B lymphocytes of the hemolytic and remission groups was obviously higher than that on CD5-B lymphocytes (74.62 ± 6.42% and 29.63 ± 10.19%, respectively; P = 0.001 versus 77.95 ± 9.57% and 26.29 ± 6.86%, respectively; P = 0.006). The ratio of p-BTK to BTK on CD5+B lymphocytes (54.89 ± 9.56%) and CD5-B lymphocytes (30.86 ± 12.47%) did not differ significantly in HCs (P = 0.109). BTK did not differ significantly between CD5+ and CD5-B lymphocytes in AIHA/ES, but p-BTK on CD5+B lymphocytes was significantly higher than that on CD5-B lymphocytes in AIHA/ES patients. Conclusions: CD5+B lymphocytes are the major B subtype that is activated in AIHA/ES patients and it positively correlates with IgE.


Assuntos
Tirosina Quinase da Agamaglobulinemia/metabolismo , Anemia Hemolítica Autoimune/imunologia , Linfócitos B/metabolismo , Trombocitopenia/imunologia , Adulto , Idoso , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
4.
Transfus Clin Biol ; 26(3): 152-154, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31277985

RESUMO

The complement is a key player of the innate immune response. It provides defense mechanisms that are not specific, but very efficient at neutralizing any invader, accounting for 4% of the proteins in the peripheral blood. Nevertheless, there is a dark side to the complement system, as it may activate its machinery against healthy cells such as peripheral blood red blood cells and platelets resulting in undesired hemolysis and thrombocytopenia, respectively. Understanding and identifying the role of complement in these settings allow physicians to adjust their diagnostic and therapeutic modalities accordingly. The role of complement in the pathophysiology and management of autoimmune hemolytic anemia and of alloimmune-mediated thrombocytopenia is under investigation and discussed.


Assuntos
Anemia Hemolítica Autoimune/imunologia , Proteínas do Sistema Complemento/imunologia , Imunidade Inata/imunologia , Transfusão de Plaquetas , Anemia Hemolítica Autoimune/terapia , Especificidade de Anticorpos , Antígenos de Plaquetas Humanas/imunologia , Soro Antilinfocitário/sangue , Soro Antilinfocitário/imunologia , Autoanticorpos/sangue , Autoanticorpos/imunologia , Humanos , Imunoglobulina G/imunologia , Imunoglobulina M/imunologia
5.
Blood ; 134(1): 9-21, 2019 07 04.
Artigo em Inglês | MEDLINE | ID: mdl-30940614

RESUMO

Evans syndrome (ES) is a rare severe autoimmune disorder characterized by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. In most cases, the underlying cause is unknown. We sought to identify genetic defects in pediatric ES (pES), based on a hypothesis of strong genetic determinism. In a national, prospective cohort of 203 patients with early-onset ES (median [range] age at last follow-up: 16.3 years ([1.2-41.0 years]) initiated in 2004, 80 nonselected consecutive individuals underwent genetic testing. The clinical data were analyzed as a function of the genetic findings. Fifty-two patients (65%) received a genetic diagnosis (the M+ group): 49 carried germline mutations and 3 carried somatic variants. Thirty-two (40%) had pathogenic mutations in 1 of 9 genes known to be involved in primary immunodeficiencies (TNFRSF6, CTLA4, STAT3, PIK3CD, CBL, ADAR1, LRBA, RAG1, and KRAS), whereas 20 patients (25%) carried probable pathogenic variants in 16 genes that had not previously been reported in the context of autoimmune disease. Lastly, no genetic abnormalities were found in the remaining 28 patients (35%, the M- group). The M+ group displayed more severe disease than the M- group, with a greater frequency of additional immunopathologic manifestations and a greater median number of lines of treatment. Six patients (all from the M+ group) died during the study. In conclusion, pES was potentially genetically determined in at least 65% of cases. Systematic, wide-ranging genetic screening should be offered in pES; the genetic findings have prognostic significance and may guide the choice of a targeted treatment.


Assuntos
Anemia Hemolítica Autoimune/genética , Anemia Hemolítica Autoimune/imunologia , Trombocitopenia/genética , Trombocitopenia/imunologia , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Masculino , Mutação , Adulto Jovem
6.
Hum Pathol ; 83: 193-198, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30063906

RESUMO

Cancer patients occasionally have anemia with high mean corpuscular volume in addition to iron deficiency anemia. Secondary autoimmune hemolytic anemia (AIHA) following cancer is also observed with low frequency. To date, no causal mechanisms for these disease states have been reported. Here, we present the case of an 80-year-old woman with AIHA that was resistant to prednisolone. Further examinations revealed primary adenocarcinoma of the sigmoid colon and primary squamous cell carcinoma in the right lung. After resections of these tumors, her anemia partially improved until a colon cancer-derived metastatic tumor was detected in the left lung. Immunoprecipitation of erythrocyte membrane proteins with an autoantibody followed by mass spectrometry/Western blotting identified band 3 as the target of the autoantibody. Immunohistochemical analysis revealed ectopic expression of band 3 in the colon adenocarcinoma. To our knowledge, this is the first report that identifies the cause in a case of anemia without bleeding in a cancer patient and that defines a mechanism underlying secondary AIHA following cancer progression.


Assuntos
Adenocarcinoma/complicações , Anemia Hemolítica Autoimune/imunologia , Proteína 1 de Troca de Ânion do Eritrócito/imunologia , Neoplasias do Colo/complicações , Expressão Ectópica do Gene/imunologia , Adenocarcinoma/patologia , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Autoantígenos/imunologia , Carcinoma de Células Escamosas/patologia , Neoplasias do Colo/patologia , Feminino , Humanos , Neoplasias Pulmonares/patologia , Neoplasias Primárias Múltiplas/patologia
7.
Clin Immunol ; 199: 44-46, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30543928

RESUMO

Relapsing Evans syndrome (ES) and systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome (APS) is very rare association. Coexistence of these syndromes is potentially fatal and require high-dose combined immunosuppressive therapy. We describe a case of successful use of Bortezomib and plasma exchange in a patient with ES and APS refractory to standard therapy. Thirty-two-year-old male who presented episodes of relapsing hemolytic anemia, pancytopenia and multiple thrombosis with positive direct and indirect antiglobulin test result, lupus anticoagulant and medium titer of anti-beta-2-glycoprotein 1 and anti-cardiolipin antibodies was diagnosed with ES and SLE with secondary APS. High-dose therapy by steroids and Cyclosporin A were started with temporary improvement. There was also no stable improvement with Rituximab and Cyclophosphamide. Bortezomib in combination with cyclosporine A and plasma exchange was introduced. He had stable improvement in hematological parameters with no evidence of relapse of hemolytic crisis or thrombosis during a follow-up for 1 year.


Assuntos
Anemia Hemolítica Autoimune/terapia , Síndrome Antifosfolipídica/terapia , Bortezomib/uso terapêutico , Lúpus Eritematoso Sistêmico/terapia , Troca Plasmática , Trombocitopenia/terapia , Adulto , Anemia Hemolítica Autoimune/imunologia , Síndrome Antifosfolipídica/imunologia , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Recidiva , Trombocitopenia/imunologia , beta 2-Glicoproteína I/imunologia
8.
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
9.
Clin Adv Hematol Oncol ; 16(10): 670-676, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30543597

RESUMO

Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP) have been associated with B-cell lymphoproliferative disorders. Here, we review the epidemiology, pathogenesis, diagnosis, and treatment of these autoimmune disorders, specifically in the setting of B-cell malignancies. AIHA and ITP are classically associated with chronic lymphocytic leukemia (CLL) but have also been reported in plasmacytic and lymphoproliferative disorders. AIHA includes both warm AIHA and cold agglutinin disease, the latter of which is strongly associated with Waldenström macroglobulinemia. The pathogenesis of these cytopenias varies with the underlying disease, but malignant cells serving as antigen-presenting cells to T lymphocytes, with the generation of autoreactive lymphocytes, may be involved. The diagnosis requires the presence of hemolysis and a positive direct antiglobulin test result. In a minority of cases, the direct antiglobulin test result is negative, and more specialized testing may be required. Data on the prognostic effect of these comorbidities are conflicting, and the prognosis may vary depending on when in the B-cell malignant process the cytopenia(s) develops. The treatment of AIHA and ITP in the setting of B-cell lymphoproliferative disorders often involves treatment of the underlying disorder, although in some cases of CLL, treatment of the underlying disorder is not indicated, and management is similar to that for idiopathic AIHA or ITP.


Assuntos
Anemia Hemolítica Autoimune , Linfócitos B , Leucemia Linfocítica Crônica de Células B , Púrpura Trombocitopênica Idiopática , Macroglobulinemia de Waldenstrom , Anemia Hemolítica Autoimune/epidemiologia , Anemia Hemolítica Autoimune/imunologia , Anemia Hemolítica Autoimune/patologia , Anemia Hemolítica Autoimune/terapia , Linfócitos B/imunologia , Linfócitos B/patologia , Comorbidade , Humanos , Leucemia Linfocítica Crônica de Células B/epidemiologia , Leucemia Linfocítica Crônica de Células B/imunologia , Leucemia Linfocítica Crônica de Células B/patologia , Leucemia Linfocítica Crônica de Células B/terapia , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/imunologia , Púrpura Trombocitopênica Idiopática/patologia , Púrpura Trombocitopênica Idiopática/terapia , Macroglobulinemia de Waldenstrom/epidemiologia , Macroglobulinemia de Waldenstrom/imunologia , Macroglobulinemia de Waldenstrom/patologia , Macroglobulinemia de Waldenstrom/terapia
10.
Medicine (Baltimore) ; 97(40): e12716, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30290676

RESUMO

RATIONALE: Relapsing polychondritis (RP) is a rare autoimmune-related disease and may be associated with other autoimmune diseases. PATIENT CONCERNS: Here we reported a case of RP patients with mixed-type AIHA. The patient was diagnosed with RP in March 2008 which was treated and the patient was in stable condition. Laboratory data revealed progressive decrease in hemoglobin during her hospitalization due to pulmonary infection in 2016. Positive Coombs' test and moderate titer of anti-cold agglutinin was detected. DIAGNOSIS: Mixed-type AIHA was diagnosed as a comorbidity in this case given the circumstance that her RP was stable and low-dose oral corticosteroids was enough to maintain remission. INTERVENTIONS: The patient was treated with intravenous immunoglobulin and steroids. OUTCOMES: The patient's body temperature dropped and hemoglobin levels rose in 2 weeks. LESSONS: Reports of RP patients with autoimmune hemolytic anemia (AIHA) are extremely rare and cases with the mixed-type AIHA has not been reported. Here we describe a case of RP with mixed-type AIHA which was considered as a comorbidity rather than a complication.


Assuntos
Anemia Hemolítica Autoimune/imunologia , Policondrite Recidivante/imunologia , Feminino , Humanos , Pessoa de Meia-Idade
11.
Clin Immunol ; 197: 219-223, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30368009

RESUMO

Early onset multisystem autoimmunity is commonly the defining feature of IPEX. Recurrent sinopulmonary infections and CVID-like phenotype were not previously recognized as a presentation in IPEX. Herein, we describe three extended family members with IPEX. In addition to autoimmunity, all three had a CVID-like presentation consisting of recurrent sinopulmonary infections, hypogammaglobulinemia and B-cell class switching defect. In vitro studies have shown that the B cell class switching defect is not B cell intrinsic. Additionally, a marked increase in circulating T follicular helper (cTFH) cells with high IFN-γ and IL-17 secretion on stimulation was noted in our patients. The dysregulated cTFH cells could contribute to a decreased B cell class switching. However, the exact mechanism of how expanded and dysregulated cTFH lead to B cell class switching defect and hypogammaglobulinemia in our patients is not clear. Our study could extend the clinical spectrum of IPEX to include a CVID-like presentation.


Assuntos
Agamaglobulinemia/imunologia , Autoimunidade/imunologia , Linfócitos B/imunologia , Diabetes Mellitus Tipo 1/congênito , Diarreia/imunologia , Doenças Genéticas Ligadas ao Cromossomo X/imunologia , Doenças do Sistema Imunitário/congênito , Linfócitos T Auxiliares-Indutores/imunologia , Adulto , Agamaglobulinemia/terapia , Anemia Hemolítica Autoimune/imunologia , Diabetes Mellitus Tipo 1/genética , Diabetes Mellitus Tipo 1/imunologia , Diabetes Mellitus Tipo 1/terapia , Diarreia/genética , Diarreia/terapia , Eczema/imunologia , Família , Feminino , Fatores de Transcrição Forkhead/genética , Doenças Genéticas Ligadas ao Cromossomo X/genética , Doenças Genéticas Ligadas ao Cromossomo X/terapia , Heterozigoto , Humanos , Doenças do Sistema Imunitário/genética , Doenças do Sistema Imunitário/imunologia , Doenças do Sistema Imunitário/terapia , Switching de Imunoglobulina/imunologia , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Enteropatias/imunologia , Masculino , Pessoa de Meia-Idade , Linhagem , Pneumonia/imunologia , Recidiva , Sinusite/imunologia , Adulto Jovem
12.
Zhonghua Nei Ke Za Zhi ; 57(9): 656-660, 2018 Sep 01.
Artigo em Chinês | MEDLINE | ID: mdl-30180450

RESUMO

Objective: To investigate the significant of peripheral CD(4)(+) CD(69)(+) T lymphocytes in patients with autoimmune hemolytic anemia (AIHA)/Evans syndrome (ES). Methods: In this study peripheral blood samples from 32 patients with AIHA/ES (15 hemolytic episode patients, 17 remission patients) and 13 healthy controls were collected. Patients with AIHA/ES were recruited in Tianjin Medical University General Hospital from October 2015 to May 2016. The percentages of CD(69)(+) T lymphocytes were analyzed by flow cytometry. The expression of CD(69) mRNA in CD(4)(+) T lymphocytes which was sorted from peripheral blood by magnetic activated cell sorting (MACS) was detected using real-time PCR. Soluable CD(69) was measured by ELISA. Results: In hemolytic episode patients, the ratio of CD(3)(+)CD(69)(+)/CD(3)(+)T lymphocytes [(3.08±1.48)%] was significantly higher than that in healthy controls [(1.28±0.83)%, P<0.01] and in remission group[(1.96±1.33)%, P<0.05]. The absolute count of CD(3)(+)CD(69)(+)T lymphocytes in hemolytic episode group [(2.94±1.81)×10(7)/L] was higher than that in healthy controls [(1.48±1.42)×10(7)/L, P<0.05]. The ratio of CD(3)(+)CD(4)(+)CD(69)(+)/CD(3)(+)CD(4)(+)T cells in hemolytic episode group [(2.16±1.56)%] was significantly higher than that in remission group [(1.16±0.62)%, P<0.05] and healthy controls[(0.94±0.78)%, P<0.05]. The quantity of CD(3)(+)CD(4)(+)CD(69)(+)T lymphocytes in hemolytic episode group[(1.04±0.98)×10(7)/L] was higher than in healthy controls [(0.44±0.38)×10(7)/L, P<0.05]. The ratio of CD(3)(+)CD(8)(+)CD(69)(+)/CD(3)(+)CD(8)(+)T lymphocyte in hemolytic episode group [(4.87±2.56)%] was significantly higher than that in healthy controls[(1.83±1.27)%, P<0.01]. The quantity of CD(3)(+)CD(8)(+)CD(69)(+)T lymphocytes in three groups did not show significant difference. The ratio of CD(3)(+)CD(4)(+)CD(69)(+)/CD(3)(+)CD(4)(+) T lymphocytes in hemolytic episode group was negatively correlated with hemoglobin (Hb) (P<0.01) , positively correlated with the percentage of reticulocytes (Ret%) (P=0.01) total bilirubin(TBil), indirect bilirubin(IBil) (P<0.01) and not correlated with absolute reticulocytes count, lactic dehydrogenase (LDH), complement 3(C3), complement 4 (C4). The ratio of CD(3)(+)CD(4)(+)CD(69)(+)/CD(3)(+)CD(4)(+)T lymphocytes in remission group was negatively correlated with Hb (P<0.05). In hemolytic episode patients CD(69) mRNA (32.26±35.11) was significantly higher than that in remission group(6.05±5.87) (P<0.05) and healthy controls (1.76±1.85)(P<0.01). CD(69) mRNA in remission group was significantly higher than healthy controls (P<0.05). Serum CD(69) in hemolytic episode patients [(494.21±16.06) ng/L] was significantly higher than that in healthy controls [(441.39±104.6) ng/L, P<0.05]. Conclusion: Our findings suggest that the proportion of CD(4)(+)CD(69)(+) T lymphocytes increase in AIHA/ES patients, which is correlated with the severity of disease.


Assuntos
Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/imunologia , Linfócitos T CD8-Positivos/imunologia , Anemia Hemolítica Autoimune/patologia , Linfócitos T CD8-Positivos/citologia , Citometria de Fluxo , Humanos , Contagem de Linfócitos , RNA Mensageiro , Trombocitopenia
13.
Vnitr Lek ; 64(5): 514-519, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30193520

RESUMO

Autoimmune hemolytic anemia (AIHA) is caused by auto-antibodies directed against self red blood cell (RBC) surface antigens. A consequence may be an intravascular hemolysis mediated by activated complement or extravascular hemolysis caused by destruction of complex of RBC with autoantibody in spleen and liver. The basic classification subdivides AIHA in primary/idiopathic and secondary with known underlying disease. A classification according to the thermal range of antibody recognizes warm AIHA, cold aglutinin disease (CAD), mixed AIHA and paroxysmal cold hemoglobinuria. Pathogenesis of AIHA consists of a defective antigen presentation to immunocompetent cells, insufficient process of T-lymphocyte tolerance to autoantigens and induction of autoantibody production by B-lymphocytes. For the diagnosis of AIHA are essential direct and indirect antiglobulin tests. The first-line therapy for warm AIHA is still administration of corticosteroids. For non-responding patients, second-line treatment includes rituximab or splenectomy. Combination of other immunosuppressive drugs represents a third-line treatment for resistant/relapsing patients. Rituximab is a treatment of choice for patients with CAD. Key words: anemia hemolytic - autoimmunity - corticosteroids - diagnosis - pathogenesis - rituximab - splenectomy - treatment.


Assuntos
Anemia Hemolítica Autoimune , Corticosteroides , Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/tratamento farmacológico , Anemia Hemolítica Autoimune/imunologia , Autoanticorpos , Humanos , Rituximab/uso terapêutico , Esplenectomia
15.
Curr Opin Hematol ; 25(6): 473-481, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30169458

RESUMO

PURPOSE OF REVIEW: Pathogenic autoantibodies directed against red blood cells (RBCs) may lead to autoimmune hemolytic anemia (AIHA), a severe and sometimes fatal disease. Much of what is known about the etiology and pathogenesis of AIHA has been learned from observations made in human patients and murine models, but many questions remain; importantly, it is still unclear why some people generate RBC-specific autoantibodies. The combination of technological advancements applied to existing models and the development of new AIHA murine models will continue to provide considerable insight into the initiation of AIHA and provide a platform for the design of more effective therapies. RECENT FINDINGS: Advancements in well described murine models of AIHA show that reticulocytes are preferentially targeted by anti-RBC autoantibodies and an increase in oxidative stress may trigger autoantibody production. Additionally, a new murine model of erythrocyte autoreactivity demonstrates that T cell tolerance is the stopgap for autoimmunity. Moreover, unlike many self-antigens, data suggest that RBC self-antigens are not presented in the thymus thereby escaping the scrutiny of T cell central tolerance mechanisms and placing emphasis on peripheral tolerance instead. Information gained from this new model provide novel insight into how the immune system responds to RBC autoantigens and provides a tractable platform to discover new therapies for AIHA. SUMMARY: Murine models of AIHA have provided significant understanding into the risk factors for AIHA. The application of new technologies and models of erythrocyte autoreactivity is a pathway with the potential to elucidate how tolerance to RBC autoantigens is established, maintained, and broken down.


Assuntos
Anemia Hemolítica Autoimune/imunologia , Modelos Animais de Doenças , Anemia Hemolítica Autoimune/tratamento farmacológico , Animais , Eritrócitos/imunologia , Linfócitos T/imunologia
16.
Iran J Kidney Dis ; 12(4): 243-246, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-30087220

RESUMO

Awareness of the uncommon associated clinical manifestations of immunoglobulin G4 (IgG4)-related kidney disease is essential for the early diagnosis and effective treatment of patients. To the best of our knowledge, there have been few reports of patients with IgG4-related kidney disease associated with autoimmune hemolytic anemia. We here report a rare case of IgG4-related kidney disease associated with autoimmune hemolytic anemia. A 70-year-old man with kidney dysfunction and severe anemia had been diagnosed with chronic kidney disease and treated without any improvement. On admission, he had a high serum creatinine level, low hemoglobin level, positive direct Coombs test, and mild proteinuria. Serum IgG and IgG4 were elevated. Kidney biopsy showed marked infiltration of IgG4-positive plasma cells and storiform fibrosis in the interstitial compartment, which confirmed the diagnosis of IgG4-related kidney disease. Corticosteroid therapy was initiated, and subsequently, the kidney dysfunction and anemia dramatically improved.


Assuntos
Anemia Hemolítica Autoimune/imunologia , Doenças Autoimunes/imunologia , Autoimunidade , Imunoglobulina G/imunologia , Nefropatias/imunologia , Rim/imunologia , Corticosteroides/uso terapêutico , Idoso , Anemia Hemolítica Autoimune/diagnóstico , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Biomarcadores/sangue , Biópsia , Humanos , Imunoglobulina G/sangue , Imuno-Histoquímica , Rim/efeitos dos fármacos , Rim/patologia , Nefropatias/complicações , Nefropatias/diagnóstico , Nefropatias/tratamento farmacológico , Masculino , Resultado do Tratamento
17.
Transfus Med Rev ; 32(4): 213-219, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30097223

RESUMO

Alloimmunization to red blood cell (RBC) antigens represents a challenge for physicians caring for women of child bearing potential. Exposure to non-self RBC antigens may occur during transfusion or pregnancy leading to the development of antibodies. If a subsequent fetus bears that antigen, maternal antibodies may attack the fetal red blood cells causing red cell destruction and clinically significant hemolytic disease of the fetus and newborn (HDFN). In the most severe cases, HDFN may result in intrauterine fetal demise due to high output cardiac failure, effusions and ascites, known as "hydrops fetalis". This article reviews strategies for management and prevention of RBC alloimmunization in women of child bearing potential.


Assuntos
Anemia Hemolítica Autoimune/imunologia , Eritroblastose Fetal/imunologia , Eritrócitos/imunologia , Isoanticorpos/sangue , Complicações Hematológicas na Gravidez , Sistema do Grupo Sanguíneo Rh-Hr/imunologia , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/epidemiologia , Transfusão de Sangue , Eritroblastose Fetal/sangue , Eritroblastose Fetal/epidemiologia , Feminino , Humanos , Gravidez
18.
Clin Lab ; 64(6): 1075-1078, 2018 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-29945320

RESUMO

BACKGROUND: Cold agglutinin disease is a very rare condition associated with agglutination of erythrocytes in cold environment usually due to IgM type antibodies. Other than hemolytic anemias, it may interfere with routine hemogram tests due to miscalculation of red blood cell count (RBC) and other hemogram parameters calculated with involvement of RBC. Awareness of the condition is important to overcome laboratory errors. METHODS: We studied a peripheral blood smear and repeated the hemogram test at 37°C to establish the diagnosis of cold agglutinin disease. RESULTS: Initial hemogram test results of the fifty-eight year-old man was as follows: RBC: 1.34 M/µL, hemoglobin (Hb): 12.4 g/dL, hematocrit (Htc): 11.8%, mean corpuscular hemoglobin (MCH): 92.4 pg, and mean corpuscular hemoglobin concentration (MCHC): 105 gr/dL. Despite the standard indirect Coombs test being negative, repeated tests at room temperature was 4+. We suspected cold agglutinin disease and repeated the hemogram test using the Bain-Marie method at 37°C and the test results showed RBC: 3.4 M/µL, hemoglobin: 12.6 g/dL, hematocrit: 30.2%, MCH: 31.7 pg, and MCHC: 41.8 g/dL. CONCLUSIONS: Inappropriate hemogram results may be a sign of underlying cold agglutinin disease. Hemolytic anemia not always accompanies the disease; however, cold exposure may trigger erythrocyte agglutination in vitro and may cause erratic laboratory results.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica/diagnóstico , Temperatura Baixa , Eritrócitos/metabolismo , Aglutinação/imunologia , Anemia Hemolítica/sangue , Anemia Hemolítica/imunologia , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/imunologia , Teste de Coombs , Contagem de Eritrócitos , Índices de Eritrócitos , Eritrócitos/imunologia , Testes Hematológicos , Hemoglobinas/metabolismo , Humanos , Imunoglobulina M/sangue , Imunoglobulina M/imunologia , Masculino , Pessoa de Meia-Idade
19.
J Immunol ; 201(1): 31-40, 2018 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-29743314

RESUMO

CD8 T cells can play both a protective and pathogenic role in inflammation and autoimmune development. Recent studies have highlighted the ability of CD8 T cells to function as T follicular helper (Tfh) cells in the germinal center in the context of infection. However, whether this phenomenon occurs in autoimmunity and contributes to autoimmune pathogenesis is largely unexplored. In this study, we show that CD8 T cells acquire a CD4 Tfh profile in the absence of functional regulatory T cells in both the IL-2-deficient and scurfy mouse models. Depletion of CD8 T cells mitigates autoimmune pathogenesis in IL-2-deficient mice. CD8 T cells express the B cell follicle-localizing chemokine receptor CXCR5, a principal Tfh transcription factor Bcl6, and the Tfh effector cytokine IL-21. CD8 T cells localize to the B cell follicle, express B cell costimulatory proteins, and promote B cell differentiation and Ab isotype class switching. These data reveal a novel contribution of autoreactive CD8 T cells to autoimmune disease, in part, through CD4 follicular-like differentiation and functionality.


Assuntos
Anemia Hemolítica Autoimune/imunologia , Anemia Hemolítica Autoimune/patologia , Linfócitos T CD8-Positivos/imunologia , Switching de Imunoglobulina/imunologia , Linfócitos T Auxiliares-Indutores/imunologia , Animais , Autoimunidade/imunologia , Linfócitos B/citologia , Linfócitos B/imunologia , Diferenciação Celular/imunologia , Eritrócitos/imunologia , Feminino , Interleucina-2/genética , Interleucinas/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Knockout , Proteínas Proto-Oncogênicas c-bcl-6/metabolismo , Receptores CXCR5/metabolismo
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