RESUMO
BACKGROUND: Variants in intracellular calcium transport genes have been associated with syndromic immunodeficiencies with a SCID phenotype. CASE REPORT: Seven-year-old girl of non-consanguineous parents, in Cartagena-Colombia. At two months of age, he presented hematochezia and was diagnosed with alimentary proctolitis without improvement with restriction to milk, wheat and eggs, and malnutrition developed. At eight months, a colon biopsy shows chronic lymphoid hyperplasia, presenting with anemia, eosinophilia, but total and specific IgE to normal foods. After four years, the Immunology Service found her asymptomatic, nutritionally recovered and without allergic sensitization, but eosinophilia and elevated calprotectin persisted, suggesting an early-onset inflammatory bowel disease. Immunoglobulins were normal, lymphocyte populations with CD3, CD4 and CD8 lymphopenia. At six years old, she presented atopic dermatitis, still had elevated calprotectin and was lymphopenic. Immunophenotyping by spectral cytometry using Cytek®cFluor®Immunoprofiling-Kit14 showed lymphopenia and CD4/CD8 inversion. Naïve CD4+ and CD8+ T lymphocytes were decreased, while T-CD8+CD45RA-CCR7- and T-CD8+CD45RA+CCR7- effector memory populations were expanded. Effector and central memory CD4+ T-lymphocytes were also increased1 (Image 1). The exome revealed a heterozygous variant in the ITPR3 gene (carrier father), c.7571G>A, p.(Arg2524His); predictors classify it as having a potential eliminating effect. CONCLUSIONS: The clinical features and immunophenotype of this candidate variant differ from others related to intracellular calcium transport. They are functional studies necessary to validate their causality. A patient with a potentially deleted variant presents an immunophenotype with CD3 lymphopenia and persistent lymphocyte activation.
ANTECEDENTES: Las variantes en genes del transporte de calcio intracelular han sido asociadas a inmunodeficiencias sindrómicas con un fenotipo IDCG. REPORTE DE CASO: Niña de siete años, de padres no consanguíneos, en Cartagena-Colombia. A los dos meses de vida, presenta hematoquecia y se diagnostica con proctolitis alimentaria sin mejoría con restricción a leche, trigo y huevo, desarrollando desnutrición. A los ocho meses, una biopsia de colon muestra hiperplasia linfoide crónica, cursa con anemia, eosinofilia, pero IgE total y específica a alimentos normales. A los cuatro años, el Servicio de Inmunología la encuentra asintomática, recuperada nutricionalmente y sin sensibilización alérgica, pero persiste eosinofilia y calprotectina elevada, sugiriendo una enfermedad inflamatoria intestinal de inicio temprano. Las inmunoglobulinas fueron normales, poblaciones linfocitarias con linfopenia CD3, CD4 y CD8. A los seis años, presenta dermatitis atópica, sigue con calprotectina elevada y linfopénica. El inmunofenotipo por citometría espectral mediante Cytek®cFluor®Immunoprofiling-Kit14, mostró linfopenia e inversión CD4/CD8. Los linfocitos T-vírgenes CD4+ y CD8+ estaban disminuidos, en cambio las poblaciones de memoria efectora T-CD8+CD45RA-CCR7- y T-CD8+CD45RA+CCR7 estaban expandidas. Los linfocitos T-CD4+ de memoria efectora y central, también estaban aumentados1 (Imagen 1). El exoma reveló una variante heterocigótica en el gen ITPR3 (padre portador), c.7571G>A, p.(Arg2524His); los predictores la clasifican como de potencial efecto deletéreo. CONCLUSIONES: La clínica y el inmunofenotipo de esta variante candidata difiere de otras relacionadas con el transporte del calcio intracelular. Son necesarios estudios funcionales para validar su causalidad. Una paciente con una variante potencialmente deletérea, presenta un inmunofenotipo con linfopenia CD3 y activación persistente de los linfocitos.
Assuntos
Imunofenotipagem , Receptores de Inositol 1,4,5-Trifosfato , Linfopenia , Humanos , Feminino , Criança , Linfopenia/genética , Linfopenia/etiologia , Receptores de Inositol 1,4,5-Trifosfato/genética , Mutação , Citometria de Fluxo , Células T de Memória/imunologiaRESUMO
Background: The consequences of SARS-CoV-2 infection in patients with primary (now called "inborn errors of immunity") or secondary immunodeficiencies is still a matter of debate. There are few reports in the literature of patients with Good's syndrome and SARS-CoV-2 infection with variable outcomes. Clinical case: A 51-year-old male with diagnosis of Good's syndrome treated with intravenous human immunoglobulin (IVIG) at a replacement dose with application every 21 days and prophylaxis for P. jirovecii with trimethoprim/ sulfamethoxazole due to profound lymphopenia at expense of T CD4+ lymphocytes who presented initially mild disease (RT-PCR+) that progressed to pneumonia with acute respiratory failure and required advanced airway management and admission to the ICU with a fatal outcome due to superinfection after 14 days hospitalized. Conclusion: It has been documented in patients with humoral immunodeficiencies a better prognosis for developing less intense cytokine release syndrome. The alteration in cellular immunity, especially lymphopenia at the expense of CD4+ T lymphocytes, may be associated with a worse prognosis as the response against viruses is compromised as well as high susceptibility to superinfection by opportunistic agents such as P. aeruginosa and Mucor sp. For this reason, we must maintain close surveillance in patients with inborn errors of immunity with cellular defects, as is the case of patients with Good's syndrome who present with COVID-19.
Introducción: las consecuencias de la infección por SARS-CoV-2 en pacientes con inmunodeficiencias primarias (ahora llamadas errores innatos de la inmunidad) o secundarias aún es un tema de debate. Existe en la literatura pocos reportes de pacientes con síndrome de Good e infección por SARS-CoV-2 con desenlaces variables. Caso clínico: paciente masculino de 51 años de edad con diagnóstico de síndrome de Good en tratamiento con inmunoglobulina humana intravenosa (IGIV) a dosis de sustitución con aplicación cada 21 días y profilaxis para P. jirovecii con trimetoprim/sulfametoxazol por linfopenia profunda a expensas de linfocitos T CD4+, que presentó infección por SARS-CoV-2 (RT-PCR+) leve, que progresó a neumonía con falla respiratoria aguda y que requirió manejo avanzado de la vía aérea e ingreso a UCI con desenlace fatal por sobreinfección luego de 14 días hospitalizado. Conclusión: se ha documentado en pacientes con inmunodeficiencias humorales mejor pronóstico por desarrollar síndrome de liberación de citocinas de menor intensidad. La alteración en la inmunidad celular, sobre todo linfopenia a expensas de linfocitos T CD4+, puede estar asociado con un peor pronóstico al verse comprometida la respuesta contra virus, así como la alta susceptibilidad a sobreinfección por agentes oportunistas como P. aeruginosa y Mucor sp. Por esta razón, debemos mantener una estrecha vigilancia en los pacientes con errores innatos de la inmunidad con defectos celulares como es el caso de los pacientes con síndrome de Good que presenten COVID-19.
Assuntos
COVID-19 , Linfopenia , Superinfecção , Timoma , Neoplasias do Timo , COVID-19/complicações , Humanos , Imunoglobulinas Intravenosas , Linfopenia/etiologia , Masculino , Pessoa de Meia-Idade , SARS-CoV-2 , Superinfecção/complicações , Superinfecção/diagnóstico , Timoma/complicações , Timoma/diagnóstico , Neoplasias do Timo/complicações , Neoplasias do Timo/diagnósticoRESUMO
Immune response dysregulation plays a key role in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pathogenesis. In this study, we evaluated immune and endothelial blood cell profiles of patients with coronavirus disease 2019 (COVID-19) to determine critical differences between those with mild, moderate, or severe COVID-19 using spectral flow cytometry. We examined a suite of immune phenotypes, including monocytes, T cells, NK cells, B cells, endothelial cells, and neutrophils, alongside surface and intracellular markers of activation. Our results showed progressive lymphopenia and depletion of T cell subsets (CD3+, CD4+, and CD8+) in patients with severe disease and a significant increase in the CD56+CD14+Ki67+IFN-γ+ monocyte population in patients with moderate and severe COVID-19 that has not been previously described. Enhanced circulating endothelial cells (CD45-CD31+CD34+CD146+), circulating endothelial progenitors (CD45-CD31+CD34+/-CD146-), and neutrophils (CD11b+CD66b+) were coevaluated for COVID-19 severity. Spearman correlation analysis demonstrated the synergism among age, obesity, and hypertension with upregulated CD56+ monocytes, endothelial cells, and decreased T cells that lead to severe outcomes of SARS-CoV-2 infection. Circulating monocytes and endothelial cells may represent important cellular markers for monitoring postacute sequelae and impacts of SARS-CoV-2 infection during convalescence and for their role in immune host defense in high-risk adults after vaccination.
Assuntos
COVID-19/imunologia , Células Endoteliais/imunologia , Monócitos/imunologia , SARS-CoV-2 , Adolescente , Adulto , Fatores Etários , Idoso , Anticorpos Antivirais/biossíntese , Anticorpos Antivirais/imunologia , Biomarcadores , Antígeno CD56/análise , COVID-19/sangue , COVID-19/epidemiologia , Criança , Comorbidade , Células Endoteliais/química , Feminino , Citometria de Fluxo , Humanos , Hipertensão/epidemiologia , Hipertensão/imunologia , Imunofenotipagem , Ativação Linfocitária , Subpopulações de Linfócitos/imunologia , Linfopenia/etiologia , Linfopenia/imunologia , Masculino , Pessoa de Meia-Idade , Monócitos/química , Neutrófilos/imunologia , Obesidade/epidemiologia , Obesidade/imunologia , Molécula-1 de Adesão Celular Endotelial a Plaquetas/análise , SARS-CoV-2/imunologia , Índice de Gravidade de Doença , Glicoproteína da Espícula de Coronavírus/imunologia , Adulto JovemRESUMO
Most persons living with HIV (PLWH) experience a significant restoration of their immunity associated with successful inhibition of viral replication after antiretroviral therapy (ART) initiation. Nevertheless, with the robust quantitative and qualitative restoration of CD4+ T-lymphocytes, a fraction of patients co-infected with tuberculosis develop immune reconstitution inflammatory syndrome (TB-IRIS), a dysregulated inflammatory response that can be associated with significant tissue damage. Several studies underscored the role of adaptive immune cells in IRIS pathogenesis, but to what degree T lymphocyte activation contributes to TB-IRIS development remains largely elusive. Here, we sought to dissect the phenotypic landscape of T lymphocyte activation in PLWH coinfected with TB inititating ART, focusing on characterization of the profiles linked to development of TB-IRIS. We confirmed previous observations demonstrating that TB-IRIS individuals display pronounced CD4+ lymphopenia prior to ART initiation. Additionally, we found an ART-induced increase in T lymphocyte activation, proliferation and cytotoxicity among TB-IRIS patients. Importantly, we demonstrate that TB-IRIS subjects display higher frequencies of cytotoxic CD8+ T lymphocytes which is not affected by ART. Moreover, These patients exhibit higher levels of activated (HLA-DR+) and profilerative (Ki-67+) CD4+ T cells after ART commencenment than their Non-IRIS counterparts. Our network analysis reveal significant negative correlations between Total CD4+ T cells counts and the frequencies of Cytotoxic CD8+ T cells in our study population which could suggest the existance of compensatory mechanisms for Mtb-infected cells elimination in the face of severe CD4+ T cell lymphopenia. We also investigated the correlation between T lymphocyte activation profiles and the abundance of several inflammatory molecules in plasma. We applied unsupervised machine learning techniques to predict and diagnose TB-IRIS before and during ART. Our analyses suggest that CD4+ T cell activation markers are good TB-IRIS predictors, whereas the combination of CD4+ and CD8+ T cells markers are better at diagnosing TB-IRIS patients during IRIS events Overall, our findings contribute to a more refined understanding of immunological mechanisms in TB-IRIS pathogenesis that may assist in new diagnostic tools and more targeted patient management.
Assuntos
Síndrome da Imunodeficiência Adquirida/imunologia , Síndrome Inflamatória da Reconstituição Imune/imunologia , Ativação Linfocitária , Subpopulações de Linfócitos T/imunologia , Tuberculose/imunologia , Síndrome da Imunodeficiência Adquirida/complicações , Síndrome da Imunodeficiência Adquirida/tratamento farmacológico , Fármacos Anti-HIV/farmacologia , Fármacos Anti-HIV/uso terapêutico , Biomarcadores , Relação CD4-CD8 , Linfócitos T CD4-Positivos/imunologia , Linfócitos T CD8-Positivos/imunologia , Citotoxicidade Imunológica , Humanos , Síndrome Inflamatória da Reconstituição Imune/sangue , Síndrome Inflamatória da Reconstituição Imune/etiologia , Imunofenotipagem , Linfopenia/etiologia , Linfopenia/imunologia , Mycobacterium tuberculosis/imunologia , Estudos Observacionais como Assunto/estatística & dados numéricos , Ensaios Clínicos Controlados Aleatórios como Assunto/estatística & dados numéricos , Estudos Retrospectivos , Tuberculose/complicaçõesRESUMO
INTRODUCTION: In April 2020, the pediatric multisystem inflammatory syndrome temporarily associated with COVID-19 (MIS-C) was described for the first time. MIS-C could have a severe course and may require critical care support. OBJECTIVE: To describe the clinical, laboratory, and management characteristics of hospitalized children who meet MIS-C criteria with severe presentation in a pediatric critical pa tient unit. PATIENTS AND METHOD: Descriptive prospective study of children with severe MIS-C mana ged by treatment phases with immunoglobulin and methylprednisolone, according to their clinical response. Epidemiological, clinical, laboratory and imaging data were obtained. Phenotypes were classified into Kawasaki and not Kawasaki, comparing their findings. RESULTS: 20 patients were analy zed, the median age was 6 years, 60% were female, and 40% presented comorbidity. SARS-CoV-2 was detected in 90% of the patients. They presented fever as the first symptom, followed by brief and early gastrointestinal symptoms (70%). 75% presented the Kawasaki phenotype. They evolved with lymphopenia, hypoalbuminemia, coagulation alterations, and elevated systemic and cardiac in flammatory parameters. 80% of the cases presented echocardiographic alterations and 90% shock that required critical care support. All the patients had a short and favorable evolution. All patients responded to the established therapy, but 40% required a second phase of treatment. There were no differences when comparing phenotypes. No deaths were reported. CONCLUSION: MIS-C is a new childhood disease whose presentation could be life-threatening. It requires early suspicion, immuno modulatory management, critical care support, and a multidisciplinary approach to obtain the best results and optimize its prognosis.
Assuntos
COVID-19 , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica , Corticosteroides/uso terapêutico , Antibacterianos/uso terapêutico , Anticoagulantes/uso terapêutico , Transtornos da Coagulação Sanguínea/etiologia , COVID-19/complicações , COVID-19/diagnóstico , COVID-19/terapia , Criança , Cuidados Críticos , Feminino , Heparina de Baixo Peso Molecular/uso terapêutico , Humanos , Hipoalbuminemia/etiologia , Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Unidades de Terapia Intensiva Pediátrica , Linfopenia/etiologia , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Estudos Prospectivos , SARS-CoV-2/isolamento & purificação , Choque/etiologia , Choque/terapia , Avaliação de Sintomas , Síndrome de Resposta Inflamatória Sistêmica/complicações , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/terapiaRESUMO
BACKGROUND: Immune cell counts in blood in severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection may be useful prognostic biomarkers of disease severity, mortality, and response to treatment. OBJECTIVES: To analyze sub-populations of lymphocytes at hospital admission in survivors and deceased from severe pneumonia due to coronavirus disease-2019 (COVID-19). METHODS: We conducted a cross-sectional study of healthcare workers confirmed with SARS-CoV-2 in convalescents (control group) and healthy controls (HC) diagnosed with severe COVID-19. Serum samples were taken at hospital admission and after recovery. Serum samples ≥ 25 days after onset of symptoms were analyzed for lymphocyte subpopulations through flow cytometry. Descriptive statistics, Kruskall-Wallis test, receiver operating characteristic curve, calculation of sensitivity, specificity, predictive values, and Kaplan-Meier analysis were performed. RESULTS: We included 337 patients: 120 HC, 127 convalescents, and 90 severe COVID-19 disease patients (50 survivors, 40 deceased). For T cells, total lymphocytes ≥ 800/µL, CD3+ ≥ 400/µL, CD4+ ≥ 180/µL, CD8+ ≥ 150/µL, B cells CD19+ ≥ 80/µL, and NK ≥ 34/µL subsets were associated with survival in severe COVID-19 disease patients. All subtypes of lymphocytes had higher concentrations in survivors than deceased, but similar between HC and convalescents. Leukocytes ≥ 10.150/µL or neutrophils ≥ 10,000/µL were associated with increased mortality. The neutrophil-to-lymphocyte ratio (NLR) ≥ 8.5 increased the probability of death in severe COVID-19 (odds ratio 11.68). CONCLUSIONS: Total lymphocytes; NLR; and levels of CD3+, CD4+, CD8+, and NK cells are useful as biomarkers of survival or mortality in severe COVID-19 disease and commonly reach normal levels in convalescents.
Assuntos
Linfócitos T CD4-Positivos/patologia , Linfócitos T CD8-Positivos/patologia , COVID-19 , Linfopenia , Neutrófilos/patologia , Biomarcadores/sangue , COVID-19/sangue , COVID-19/diagnóstico , COVID-19/mortalidade , COVID-19/terapia , Correlação de Dados , Estudos Transversais , Feminino , Humanos , Estimativa de Kaplan-Meier , Células Matadoras Naturais/patologia , Contagem de Leucócitos/métodos , Linfopenia/sangue , Linfopenia/diagnóstico , Linfopenia/etiologia , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Mortalidade , Valor Preditivo dos Testes , Avaliação de Sintomas/métodosRESUMO
BACKGROUND: Although the chemotherapy-induced depletion of circulating white blood cells (WBC) is well recognized, the impact of exclusive radiotherapy (RT) on the different subpopulations of WBC remains unexplored. This may be important for immunotherapy administrated in combination with radiation, especially in malignant tumors usually treated with RT or chemoradiotherapy (CRT), and characterized by a high mutational burden, such as endometrial (EC) or cervical cancer (CC). We aimed to evaluate the impact of RT and CRT on circulating WBC in uterine cancers and its correlation with survival. MATERIAL AND METHODS: A total of 202 consecutive patients with uterine cancers treated with RT or CRT between 2009 and 2016 in a large European center and with available basal and post-treatment blood tests were retrospectively evaluated. EC and CC patients were analyzed separately. The differences between pre- and post- treatment WBC mean values were evaluated independently in patients treated with CRT and exclusive RT. Two-sided T test for paired samples and Kaplan-Meier curves were applied for analysis (p value < 0.05, SPSS v.23). RESULTS: Among EC patients, 29 received CRT and 34 exclusive postoperative RT, while in CC cohort, 105 were treated with CRT and 34 with RT. In both cohorts, CRT affected significantly all WBC subtypes, whereas exclusive RT decreased only lymphocytes population (p = 0.000). Radiation-induced lymphopenia (RIL) had no impact on survival outcomes. CONCLUSIONS: The selective depletion of lymphocytes after RT was significant in both EC and CC. Our results are of interest for further research on RIL and for design of immunotherapy-based clinical trials.
Assuntos
Neoplasias do Endométrio/terapia , Linfopenia/etiologia , Neoplasias do Colo do Útero/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Quimiorradioterapia/efeitos adversos , Ensaios Clínicos como Assunto , Neoplasias do Endométrio/mortalidade , Feminino , Humanos , Imunoterapia , Contagem de Leucócitos , Pessoa de Meia-Idade , Neoplasias do Colo do Útero/mortalidadeRESUMO
Background We aimed to identify risk factors for early complications in systemic lupus erythematosus (SLE) patients undergoing major surgery. Methods We conducted a retrospective comparative cohort study including patients with SLE undergoing major surgery, and non-SLE patients matched 1:1. Main outcomes were development of infectious and noninfectious complications, and 30-day postoperative mortality. Results A total of 382 patients (191 SLE and 191 non-SLE) were included. Postoperative complications occurred in 82 (43%) SLE patients and 58 (30%) without SLE, ( p = 0.01). Variables associated with infectious complications in SLE patients: prednisone use (OR 1.81, 95% CI 1.13-2.90), anemia (OR 2.43, 95% CI 1.45-4.08), hypoalbuminemia (OR 2.58, 95% CI 1.55-4.30) and lymphopenia (OR 2.43, 95% CI 1.52-3.89), p < 0.05. Variables associated with noninfectious complications: anemia (OR, 1.93, 95% CI 1.03-3.64) and hypoalbuminemia (OR 2.11, 95% CI 1.16-3.86), p < 0.05. Variables associated with any complication: SLEDAI-2K (OR 1.1, 95% CI 1.01-1.20), nephritis (OR 10.08, 95% CI 1.21-83.63), aspirin use (OR 2.68, 95% CI 1.19-6.02, p = 0.01), low C3 (OR 2.00, 95% CI 1.06-3.80), anemia (OR 2.68, 95% CI 1.39-5.18), hypoalbuminemia (OR 3.49, 95% CI 1.83-6.66) and lymphopenia (OR 2.36, 95% CI 1.30-4.26), p < 0.05. More patients with SLE died (6% vs 1%, p = 0.02). Conclusions SLE patients present higher frequency of postoperative complications and mortality compared with non-SLE patients. Hypoalbuminemia, anemia, lymphopenia and aspirin use are independent risk factors.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/mortalidade , Adolescente , Adulto , Idoso , Anemia/etiologia , Aspirina/uso terapêutico , Criança , Procedimentos Cirúrgicos Eletivos/efeitos adversos , Feminino , Humanos , Modelos Logísticos , Linfopenia/etiologia , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Nefrite/etiologia , Prednisona/uso terapêutico , Estudos Retrospectivos , Fatores de Risco , Adulto JovemRESUMO
BACKGROUND: Patients with End Stage Renal Disease (ESRD), defined as those in Stage 5 of the Kidney Disease Outcome Quality Initiative (KDOQI) classification, have a number of acquired immune deficiencies secondary to the uremic stage, among them lymphopenia. In the present report, we retrospectively characterized the peripheral blood lymphocyte counts in a group of patients with ESRD and we related lymphopenia to their biochemical parameters and to the presence or absence of infections. METHODS: Medical records from 190 patients in ESRD were selected from 282 medical records of patients being treated between February 2008 and November 2012 for kidney failure at different stages. A number of variables, including lymphocyte counts, biochemical parameters and infections, were analyzed at two different time points: Before and during dialysis. RESULTS: ESRD patients analyzed had a well defined peripheral blood hematological pattern, characterized by severe chronic anemia, normal or elevated leukocyte count and normal or below normal lymphocyte count. The degree of hematological changes correlated with the depth of renal dysfunction and improved with dialysis along with the improvement of urea and creatinine values. CONCLUSIONS: Lymphopenia was present in around half ESRD patients and was associated with increased infections, but they were of the same type as those present in ESRD patients without lymphopenia. Infections were different as those commonly associated with other immune deficiency lymphopenias. The implications of these findings are discussed.
Introducción: los pacientes con insuficiencia renal crónica en etapa terminal (ERET), presentan alteraciones inmunológicas diversas que los hacen más susceptibles a infecciones. Entre las alteraciones reportadas se encuentra la linfopenia. Se han realizado pocos estudios en nuestro medio que muestren la frecuencia y características de esta alteración así como su trascendencia clínica, relacionada con las infecciones que afectan a estos pacientes. Métodos: se analizó una serie de variables, incluyendo los valores de linfocitos y la presencia de infecciones, en un grupo de 190 pacientes con ERET de febrero 2008 a noviembre 2012. Se correlacionan y comparan los valores obtenidos entre ellos en dos momentos de su evolución: antes y durante su tratamiento dialítico. Resultados: en los pacientes con ERET, se obtuvo un perfil hematológico característico, caracterizado por anemia crónica severa, leucocitos totales normales o por arriba de lo normal y linfocitos normales o por debajo de lo normal (linfopenia). El grado de alteración hematológica correlacionó con el grado de afección renal y se corrigió en la medida que se corrigieron las alteraciones bioquímicas relacionadas con la ERET mediante diálisis peritoneal. Conclusiones: la linfopenia se encontró en cerca de la mitad de los pacientes con ERET y se asoció con el incremento de infecciones; el tipo de infecciones fue similar a lo observado en pacientes sin linfopenia y diferente al observado en pacientes con inmunodeficiencias primarias o adquiridas que afectan a los linfocitos.
Assuntos
Falência Renal Crônica/complicações , Linfopenia/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Incidência , Falência Renal Crônica/imunologia , Falência Renal Crônica/terapia , Contagem de Linfócitos , Linfopenia/diagnóstico , Linfopenia/epidemiologia , Linfopenia/imunologia , Masculino , Pessoa de Meia-Idade , Diálise Renal , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Congenital cardiac anomalies are associated with immunologic perturbations. Surgical thymectomy, thoracic duct manipulation, and protein- losing enteropathy (PLE), a condition related to stressed Fontan hemodynamics, presumably contribute to low peripheral absolute lymphocyte counts (ALCs) and quantitative immunoglobulins. Clinical significance of lymphopenia and hypogammaglobulinemia in single-ventricle survivors requires additional study. OBJECTIVE: Although immunologic laboratory anomalies are common in this population, we hypothesize that clinically significant immunodeficiency requiring intervention is rarely required. METHODS: A retrospective chart review of the immunologic parameters of patients enrolled in the Single Ventricle Survivorship Program (SVSP) at the Children's Hospital of Philadelphia was performed. RESULTS: The age range of the 178 SVSP patients was 3 to 26 years, with a median of 10.8 years. Most of the SVSP patients had some degree of lymphopenia. In the non-PLE group, the range of ALCs varied from 530 to 5322 cells/µL, with 17 patients without PLE maintaining an ALC of less than 1000 cells/µL. Among those with PLE, the median ALC and the IgG level were lower (672 cells/µL and 200 mg/dL, respectively) than in those without (1610 cells/µL and 868 mg/dL, respectively). Despite lymphopenia in the majority, few were severely clinically affected: 24% had delayed clearance of cutaneous viral infections, 63% had atopy, and 1 died of EBV-associated Hodgkin lymphoma. Immunoglobulin replacement was clinically indicated for 3 patients, 1 of whom had common variable immunodeficiency. Four patients with normal splenic function were treated with daily antibiotic prophylaxis. CONCLUSIONS: Patients with repaired single-ventricle physiology often demonstrate T-cell lymphopenia and hypogammaglobulinemia. A significant portion of patients without PLE also have lymphopenia. The most common clinical manifestation was delayed clearance of cutaneous viral infections, but significant systemic opportunistic infections were not seen despite laboratory abnormalities and lack of antimicrobial prophylaxis.