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1.
BMJ Case Rep ; 12(3)2019 Mar 31.
Artigo em Inglês | MEDLINE | ID: mdl-30936343

RESUMO

Common variable immunodeficiency syndrome (CVID) is a heterogeneous disorder characterised by diminished levels of IgG, IgA and/or IgM, and recurrent bacterial infections. Sinopulmonary infections are most commonly reported followed by gastrointestinal (GI) infections. GI tract represents the largest immune organ with abundance of lymphoid cells, its involvement can manifest variably ranging from asymptomatic involvement to florid symptoms and signs. Diffuse nodular lymphoid hyperplasia (DNLH) of the GI tract is characterised by numerous small polypoid nodules of variable size in the small intestine, large intestine or both. It is commonly seen in association to immunodeficiency states such as CVID, IgA deficiency and chronic infections due to Giardia lamblia and Helicobacter pylori and cryptosporidiosis. Repetitive antigenic stimulation leads to lymphoid hyperplasia. We herein describe a case of DNLH of the intestine and another case of duodenal cytomegalovirus (CMV) infection associated with CVID.


Assuntos
Imunodeficiência de Variável Comum/virologia , Infecções por Citomegalovirus/complicações , Diarreia/virologia , Duodeno/patologia , Hiperplasia/virologia , Intestino Delgado/patologia , Transtornos Linfoproliferativos/virologia , Adulto , Antivirais/uso terapêutico , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/fisiopatologia , Infecções por Citomegalovirus/fisiopatologia , Duodeno/virologia , Endoscopia do Sistema Digestório , Ganciclovir/uso terapêutico , Humanos , Hiperplasia/tratamento farmacológico , Hiperplasia/fisiopatologia , Imunoglobulinas Intravenosas/uso terapêutico , Intestino Delgado/virologia , Transtornos Linfoproliferativos/tratamento farmacológico , Transtornos Linfoproliferativos/fisiopatologia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
2.
Int J Immunopathol Pharmacol ; 33: 2058738419843381, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30968712

RESUMO

Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency diseases characterized by hypogammaglobulinemia and dysfunctional immune response to invading pathogens. Previous studies have indicated that CVID is associated with microbial translocation and systemic myeloid cell activation. The goal of this study was to determine whether patients with CVID display elevated systemic levels of markers of granulocyte activation and whether the levels are further influenced by intravenous immunoglobulin (IVIg) infusions. The plasma levels of granulocyte activation markers elastase and myeloperoxidase were determined using enzyme-linked immunosorbent assay (ELISA) in 46 CVID patients and 44 healthy controls. All CVID patients were in a stable state with no apparent acute infection. In addition, granulocyte activation markers' plasma levels in 24 CVID patients were determined prior to and 1 h following IVIg administration. Neutrophil elastase and myeloperoxidase plasma levels were significantly higher in CVID patients than in healthy controls. Systemic elastase levels were further increased following IVIg administration. In vitro stimulation of 13 CVID patients' whole blood using IVIg in a therapeutically relevant dose for 2 h resulted in a significant increase in plasma elastase levels compared to unstimulated blood. The data presented here indicate that CVID is associated with chronic granulocytic activation which is further exacerbated by administering IVIg. Increased myeloperoxidase and elastase levels may contribute to associated comorbidities in CVID patients.


Assuntos
Imunodeficiência de Variável Comum/sangue , Imunodeficiência de Variável Comum/diagnóstico , Elastase de Leucócito/sangue , Peroxidase/sangue , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Imunodeficiência de Variável Comum/tratamento farmacológico , Feminino , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Masculino , Pessoa de Meia-Idade , Adulto Jovem
4.
BMJ Case Rep ; 20182018 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-29559491

RESUMO

Common variable immunodeficiency (CVID) refers to a group of disorders where differentiation and maturation of B cells into plasma cells are affected, leading to decreased or defective immunoglobulin production and subsequent immunodeficiency. Symptoms may present at any age between 5 and 72 years, although more severe forms often manifest earlier in life. Milder forms may not be detected. We present an intriguing case of a 69-year-old man presenting with recurrent pneumonia caused by a rare organism Staphylococcus lugdunensis, eventually determined to be caused by CVID. The patient had a good clinical outcome after receiving immunoglobulin replacement therapy.


Assuntos
Imunodeficiência de Variável Comum/diagnóstico , Pneumonia Estafilocócica/etiologia , Idade de Início , Idoso , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/tratamento farmacológico , Farmacorresistência Bacteriana , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Pulmão/diagnóstico por imagem , Masculino , Pneumonia Estafilocócica/sangue , Recidiva , Staphylococcus lugdunensis/isolamento & purificação , Vancomicina/uso terapêutico
5.
Int J Mol Sci ; 19(2)2018 Feb 02.
Artigo em Inglês | MEDLINE | ID: mdl-29393912

RESUMO

Common variable immunodeficiency (CVID) is an immunodeficiency disorder with a high incidence of gastrointestinal manifestations and an increased risk of gastric carcinoma and lymphoma. This review discusses the latest advancements into the immunological, clinical and diagnostic aspects of gastric malignancies in patients with CVID. The exact molecular pathways underlying the relationships between CVID and gastric malignancies remain poorly understood. These include genetics, immune dysregulation and chronic infections by Helicobacter pylori. Further studies are needed to better stratify the risk for cancer in these patients, to elaborate surveillance programs aimed at preventing these complications, and to develop new and more effective therapeutic approaches.


Assuntos
Adenocarcinoma/patologia , Imunodeficiência de Variável Comum/patologia , Imunoglobulinas/deficiência , Linfoma/patologia , Neoplasias Gástricas/patologia , Adenocarcinoma/diagnóstico , Adenocarcinoma/tratamento farmacológico , Adenocarcinoma/imunologia , Corticosteroides/uso terapêutico , Antineoplásicos/uso terapêutico , Linfócitos B/efeitos dos fármacos , Linfócitos B/imunologia , Linfócitos B/patologia , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/imunologia , Árvores de Decisões , Gerenciamento Clínico , Detecção Precoce de Câncer , Helicobacter pylori/patogenicidade , Helicobacter pylori/fisiologia , Humanos , Imunoglobulinas/uso terapêutico , Linfoma/diagnóstico , Linfoma/tratamento farmacológico , Linfoma/imunologia , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/tratamento farmacológico , Neoplasias Gástricas/imunologia , Linfócitos T/efeitos dos fármacos , Linfócitos T/imunologia , Linfócitos T/patologia
6.
Curr Probl Diagn Radiol ; 47(4): 282-284, 2018 Jul - Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28583689

RESUMO

Common variable immunodeficiency is the most common primary immunodeficiency and consists of impaired immunoglobulin production causing recurrent sinopulmonary infections. The most common cause of mortality for this disorder, however, is from the development of malignancy and autoimmune disorders. One common entity that develops is a systemic granulomatous and lymphoproliferative disorder that can cause an interstitial lung disease more formally referred to as granulomatous-lymphocytic interstitial lung disease (GL-ILD). We discuss a case of a 25-year-old woman with common variable immunodeficiency and GL-ILD and review the literature to summarize the most common radiological findings to raise the suspicion for GL-ILD on high-resolution computed tomography and delineate this from infection and other mimickers. We will also review key histopathological characteristics for diagnosis and the clinical approach and treatment options for this rare disease.


Assuntos
Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/diagnóstico por imagem , Imunodeficiência de Variável Comum/tratamento farmacológico , Granuloma do Sistema Respiratório/diagnóstico por imagem , Granuloma do Sistema Respiratório/etiologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Tomografia Computadorizada por Raios X/métodos , Adulto , Biópsia , Diagnóstico Diferencial , Feminino , Granuloma do Sistema Respiratório/tratamento farmacológico , Humanos , Doenças Pulmonares Intersticiais/tratamento farmacológico
8.
Intern Med ; 56(21): 2899-2902, 2017 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-28924106

RESUMO

Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and nodules on the bilateral lung fields, systemic lymphadenopathy, and splenomegaly. A surgical lung biopsy specimen provided the final diagnosis of lymphoproliferative disease in CVID, which was grouped under the term granulomatous lymphocytic interstitial lung disease. Interestingly, the lung lesions of this case resolved immediately after the initiation of intravenous immunoglobulin monotherapy.


Assuntos
Imunodeficiência de Variável Comum/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , Doenças Pulmonares Intersticiais/dietoterapia , Adulto , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/diagnóstico , Feminino , Humanos , Pulmão/patologia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Tomografia Computadorizada por Raios X/métodos
10.
J Clin Immunol ; 37(8): 790-800, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28956255

RESUMO

INTRODUCTION: LPS-responsive beige-like anchor protein (LRBA) deficiency is a primary immunodeficiency categorized as common variable immunodeficiency associated with autoimmune manifestations and inflammatory bowel diseases; however, the clinical spectrum has been extended. Here, we present our cohort of Turkish LRBA-deficient patients from a single center, demonstrating a diversity of clinical manifestations. METHOD: Seven affected individuals from five families were assessed retrospectively in this study. RESULTS: Of the seven patients with LRBA deficiency, four had homozygous, and two had compound heterozygous mutations. One patient remained disease free until the last follow-up (age 17 years). The most common clinical manifestations of the six symptomatic patients were organomegaly (6/6), autoimmunity (6/6), and chronic diarrhea (5/6). Recurrent infectious episodes were observed in three patients. None of the patients had hypogammaglobulinemia at presentation. B cell subpopulation analysis revealed low numbers of switched-memory B cell numbers in two of the four tested patients. During the disease course, three of the patients died, two of them underwent successful hematopoietic stem cell transplantation (HSCT) from matched sibling donors, and one is under abatacept therapy. CONCLUSION: LRBA defects should always be kept in mind as a differential diagnosis for patients with autoimmune disease affecting multiple organs, chronic diarrhea, and organomegalies. In our experience, early HSCT is a life-saving therapeutic strategy.


Assuntos
Abatacepte/uso terapêutico , Proteínas Adaptadoras de Transdução de Sinal/genética , Doenças Autoimunes/genética , Imunodeficiência de Variável Comum/genética , Imunossupressores/uso terapêutico , Doenças Inflamatórias Intestinais/genética , Mutação/genética , Adolescente , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Criança , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/tratamento farmacológico , Intervalo Livre de Doença , Evolução Fatal , Feminino , Homozigoto , Humanos , Lactente , Doenças Inflamatórias Intestinais/diagnóstico , Doenças Inflamatórias Intestinais/tratamento farmacológico , Masculino , Sepse , Turquia
11.
Immunol Cell Biol ; 95(9): 775-788, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28611475

RESUMO

Inherited mutations in lipopolysaccharide-responsive beige-like anchor (LRBA) cause a recessive human immune dysregulation syndrome with memory B-cell and antibody deficiency (common variable immunodeficiency), inflammatory bowel disease, enlarged spleen and lymph nodes, accumulation of activated T cells and multiple autoimmune diseases. To understand the pathogenesis of the syndrome, C57BL/6 mice carrying a homozygous truncating mutation in Lrba were produced using CRISPR/Cas9-mediated gene targeting. These mice revealed that LRBA has a critical, cell-autonomous role in promoting cytotoxic T-lymphocyte antigen-4 (CTLA-4) accumulation within CD4 effector T cells and FOXP3+ T-regulatory cells (Tregs). In young mice, or in chimeric mice where only half of the T cells are LRBA deficient, low CTLA-4 was the only detectable abnormality in Tregs, whereas in old mice FOXP3 was also decreased. Low CTLA-4 did not translate into increased CD86 on B cells unless the LRBA-deficient mice were immunised, and neither immunisation nor chronic lymphocytic choriomeningitis virus infection precipitated immune dysregulation. LRBA deficiency did not alter antigen-specific B-cell activation, germinal centre (GC) formation, isotype switching or affinity maturation. Paradoxically, CD86 was decreased on GC B cells in LRBA-deficient mice, pointing to compensatory mechanisms for controlling CD86 in the face of low CTLA-4. These results add to the experimental rationale for treating LRBA deficiency with the CTLA4-Ig fusion protein, Abatacept, and pose questions about the limitations of laboratory experiments in mice to reproduce human disease in natura.


Assuntos
Proteínas Adaptadoras de Transdução de Sinal/metabolismo , Antígeno CTLA-4/metabolismo , Imunodeficiência de Variável Comum/imunologia , Coriomeningite Linfocítica/imunologia , Vírus da Coriomeningite Linfocítica/imunologia , Linfócitos T Auxiliares-Indutores/imunologia , Linfócitos T Reguladores/imunologia , Abatacepte/uso terapêutico , Proteínas Adaptadoras de Transdução de Sinal/genética , Animais , Antígeno B7-2/metabolismo , Antígeno CTLA-4/genética , Células Cultivadas , Repetições Palindrômicas Curtas Agrupadas e Regularmente Espaçadas , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/genética , Fatores de Transcrição Forkhead/metabolismo , Humanos , Imunossupressores/uso terapêutico , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout
12.
BMJ Case Rep ; 20172017 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-28551598

RESUMO

A 57-year-old woman with frequent respiratory infections was initially diagnosed with IgG subclass deficiency based on low levels of IgG subclasses 2 and 3. Three years later, she progressed to having IgA deficiency as well. With a normal total IgG level, she does not meet criteria for common variable immunodeficiency (CVID). This may represent a variant of CVID. This also highlights the importance of immunoglobulin subclass estimation in patients where immunodeficiency is suspected clinically. She is being treated with rotational antibiotics the first 10 days of every month, monthly intravenous immunoglobulin (IVIG) infusion and osteopathic manipulation one to two times per month. On this regimen, although she has had several viral respiratory infections, she has avoided further hospitalisation for more than 1 year.


Assuntos
Imunodeficiência de Variável Comum/classificação , Imunodeficiência de Variável Comum/imunologia , Antibacterianos/uso terapêutico , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/tratamento farmacológico , Diagnóstico Diferencial , Feminino , Humanos , Deficiência de IgA , Deficiência de IgG , Imunoglobulinas Intravenosas/uso terapêutico , Pessoa de Meia-Idade , Infecções Respiratórias/tratamento farmacológico , Infecções Respiratórias/etiologia , Infecções Respiratórias/imunologia , Resultado do Tratamento
13.
J Allergy Clin Immunol Pract ; 5(4): 938-945, 2017 Jul - Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28351785

RESUMO

A proportion of people living with common variable immunodeficiency disorders develop granulomatous-lymphocytic interstitial lung disease (GLILD). We aimed to develop a consensus statement on the definition, diagnosis, and management of GLILD. All UK specialist centers were contacted and relevant physicians were invited to take part in a 3-round online Delphi process. Responses were graded as Strongly Agree, Tend to Agree, Neither Agree nor Disagree, Tend to Disagree, and Strongly Disagree, scored +1, +0.5, 0, -0.5, and -1, respectively. Agreement was defined as greater than or equal to 80% consensus. Scores are reported as mean ± SD. There was 100% agreement (score, 0.92 ± 0.19) for the following definition: "GLILD is a distinct clinico-radio-pathological ILD occurring in patients with [common variable immunodeficiency disorders], associated with a lymphocytic infiltrate and/or granuloma in the lung, and in whom other conditions have been considered and where possible excluded." There was consensus that the workup of suspected GLILD requires chest computed tomography (CT) (0.98 ± 0.01), lung function tests (eg, gas transfer, 0.94 ± 0.17), bronchoscopy to exclude infection (0.63 ± 0.50), and lung biopsy (0.58 ± 0.40). There was no consensus on whether expectant management following optimization of immunoglobulin therapy was acceptable: 67% agreed, 25% disagreed, score 0.38 ± 0.59; 90% agreed that when treatment was required, first-line treatment should be with corticosteroids alone (score, 0.55 ± 0.51).


Assuntos
Imunodeficiência de Variável Comum , Granuloma , Doenças Pulmonares Intersticiais , Instituições de Caridade , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/diagnóstico por imagem , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/patologia , Consenso , Granuloma/diagnóstico , Granuloma/diagnóstico por imagem , Granuloma/tratamento farmacológico , Granuloma/patologia , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/patologia , Sociedades Médicas , Reino Unido
14.
Clin Exp Rheumatol ; 35(2): 327-329, 2017 Mar-Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28134088

RESUMO

Lipopolysaccharide-responsive, beige-like anchor protein (LRBA) deficiency causes common variable immunodeficiency (CVID) disorders and autoimmunity. LRBA deficiency has become a clinically variable syndrome with a wide spectrum of clinical manifestations. We report a patient with LRBA deficiency associated chronic non-erosive arthritis. This report highlights the spectrum of arthritis in such patients and the potential causative role of LRBA gene in juvenile arthritis.


Assuntos
Proteínas Adaptadoras de Transdução de Sinal/genética , Artrite Juvenil/genética , Imunodeficiência de Variável Comum/genética , Mutação , Abatacepte/administração & dosagem , Proteínas Adaptadoras de Transdução de Sinal/deficiência , Proteínas Adaptadoras de Transdução de Sinal/imunologia , Adolescente , Artrite Juvenil/diagnóstico , Artrite Juvenil/tratamento farmacológico , Artrite Juvenil/imunologia , Artrografia , Doença Crônica , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/imunologia , Análise Mutacional de DNA , Predisposição Genética para Doença , Humanos , Imunossupressores/administração & dosagem , Articulação do Joelho/diagnóstico por imagem , Imagem por Ressonância Magnética , Masculino , Fenótipo , Resultado do Tratamento
16.
Clin Exp Immunol ; 187(1): 138-145, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27896807

RESUMO

Common variable immunodeficiency (CVID) is the most common severe adult primary immunodeficiency and is characterized by a failure to produce antibodies leading to recurrent predominantly sinopulmonary infections. Improvements in the prevention and treatment of infection with immunoglobulin replacement and antibiotics have resulted in malignancy, autoimmune, inflammatory and lymphoproliferative disorders emerging as major clinical challenges in the management of patients who have CVID. In a proportion of CVID patients, inflammation manifests as granulomas that frequently involve the lungs, lymph nodes, spleen and liver and may affect almost any organ. Granulomatous lymphocytic interstitial lung disease (GLILD) is associated with a worse outcome. Its underlying pathogenic mechanisms are poorly understood and there is limited evidence to inform how best to monitor, treat or select patients to treat. We describe the use of combined 2-[(18)F]-fluoro-2-deoxy-d-glucose positron emission tomography and computed tomography (FDG PET-CT) scanning for the assessment and monitoring of response to treatment in a patient with GLILD. This enabled a synergistic combination of functional and anatomical imaging in GLILD and demonstrated a widespread and high level of metabolic activity in the lungs and lymph nodes. Following treatment with rituximab and mycophenolate there was almost complete resolution of the previously identified high metabolic activity alongside significant normalization in lymph node size and lung architecture. The results support the view that GLILD represents one facet of a multi-systemic metabolically highly active lymphoproliferative disorder and suggests potential utility of this imaging modality in this subset of patients with CVID.


Assuntos
Imunodeficiência de Variável Comum/diagnóstico por imagem , Granuloma do Sistema Respiratório/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Linfócitos/imunologia , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada por Raios X/métodos , Adulto , Imunodeficiência de Variável Comum/tratamento farmacológico , Feminino , Fluordesoxiglucose F18 , Granuloma do Sistema Respiratório/tratamento farmacológico , Humanos , Pulmão/patologia , Doenças Pulmonares Intersticiais/tratamento farmacológico , Pessoa de Meia-Idade , Ácido Micofenólico/uso terapêutico , Rituximab/uso terapêutico , Resultado do Tratamento
18.
Eur Ann Allergy Clin Immunol ; 48(5): 197-201, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27608477

RESUMO

Purpose. To report early onset steroid induced posterior subcapsular cataract in a case of common variable immunodeficiency. Methods. Case report. Results. Here we report a 14-yearold male of steroid induced bilateral posterior subcapsular cataract in a common variable immunodeficiency patient with damaging mutations in Glutathione reductase gene, leading to hypersensitivity of patient to glucocorticoid (GC) products. Conclusions. In order to reduce the ocular side effects of the GCs there are some advisements, including a complete history, regular examination, GC should be prescribed in minimal dosage and minimal course, and as possible GC-sparing drugs should always be considered.


Assuntos
Catarata/induzido quimicamente , Imunodeficiência de Variável Comum/tratamento farmacológico , Glucocorticoides/efeitos adversos , Cápsula Posterior do Cristalino/efeitos dos fármacos , Prednisona/efeitos adversos , Adolescente , Catarata/diagnóstico , Catarata/genética , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/imunologia , Análise Mutacional de DNA , Predisposição Genética para Doença , Glutationa Redutase/genética , Humanos , Masculino , Mutação , Fenótipo , Cápsula Posterior do Cristalino/patologia , Fatores de Risco , Fatores de Tempo
19.
J Investig Allergol Clin Immunol ; 26(4): 233-43, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27374799

RESUMO

Common variable immunodeficiency (CVID) is the most common clinical primary immunodeficiency. It is characterized by a defect in B-cell differentiation to plasma and memory B cells. Moreover, numerous T-cell abnormalities have been reported and include decreased T-cell count and proliferative response, increased T-cell activation and apoptosis, and abnormalities in cytokine production. The aims of this review are to describe phenotypic and functional defects in T cells in CVID patients and to review the literature with respect to the effects of immunoglobulin replacement on the T-cell component in CVID patients.


Assuntos
Imunodeficiência de Variável Comum/imunologia , Linfócitos T/imunologia , Linfócitos T CD4-Positivos/imunologia , Imunodeficiência de Variável Comum/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Ativação Linfocitária , Linfócitos T Reguladores/imunologia
20.
Medicine (Baltimore) ; 95(30): e4304, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27472706

RESUMO

Common variable immunodeficiency (CVID) is defined by low levels of IgG and IgA, but perturbations in T cells are also commonly found. However, there is limited information on γδ T cells in CVID patients. Newly diagnosed CVID patients (n = 15) were enrolled before and after intravenous IgG (IVIg) replacement therapy. Cryopreserved peripheral blood mononuclear cells were then used to study γδ T cells and CVID patients were compared to healthy controls (n = 22). The frequency and absolute count of Vδ1 γδ T cells was found to be increased in CVID (median 0.60% vs 2.64%, P <0.01 and 7.5 vs 39, P <0.01 respectively), while they were decreased for Vδ2 γδ T cells (median, 2.36% vs 0.74%, P <0.01 and 37.8 vs 13.9, P <0.01 respectively) resulting in an inversion of the Vδ1 to Vδ2 ratio (0.24 vs 1.4, P <0.001). Markers of immune activation were elevated on all subsets of γδ T cells, and HLA-DR expression was associated with an expansion of Vδ1 γδ T cells (r = 0.73, P = 0.003). Elevated PD-1 expression was found only on Vδ2 γδ T cells (median 1.15% vs 3.08%, P <0.001) and was associated with the decrease of Vδ2 γδ T cells (r = -0.67, P = 0.007). IVIg had no effect on the frequency of Vδ1 and Vδ2 γδ T cells or HLA-DR expression, but alleviated CD38 expression on Vδ1 γδ T cells (median MFI 965 vs 736, P <0.05). These findings suggest that immunological perturbations of γδ T cells are a general feature associated with CVID and are only partially reversed by IVIg therapy.


Assuntos
Imunodeficiência de Variável Comum/imunologia , Subpopulações de Linfócitos T/imunologia , Adolescente , Adulto , Estudos de Casos e Controles , Criança , Imunodeficiência de Variável Comum/tratamento farmacológico , Feminino , Citometria de Fluxo , Humanos , Imunoglobulina A/imunologia , Imunoglobulina G/imunologia , Imunoglobulina G/uso terapêutico , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
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