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2.
Intern Med ; 58(21): 3167-3171, 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31292392

RESUMO

A 44-year-old female with rheumatoid arthritis treated with methotrexate (MTX) and tocilizumab (TCZ) was admitted to our hospital with nasal pain. Nasal fiberscopy revealed septum perforation, while a membrane biopsy indicated granuloma and fibrinoid necrosis of the small artery. The patient was treated with prednisolone 30 mg/day after discontinuation of MTX and TCZ. Inguinal lymph node biopsy revealed diffuse infiltrations of atypical T-cells and Epstein-Barr virus-positive B cells. The patient was diagnosed with peripheral T-cell lymphoma due to MTX-associated lymphoproliferative disorder (MTX-LPD). We herein describe the case of a patient with nasal septum perforation due to MTX-LPD mimicking granulomatosis with polyangiitis.


Assuntos
Antirreumáticos/efeitos adversos , Artrite Reumatoide/complicações , Transtornos Linfoproliferativos/induzido quimicamente , Metotrexato/efeitos adversos , Perfuração do Septo Nasal/etiologia , Adulto , Anticorpos Monoclonais Humanizados/uso terapêutico , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Biópsia , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Humanos , Linfonodos/patologia , Transtornos Linfoproliferativos/complicações , Metotrexato/uso terapêutico , Septo Nasal/patologia
3.
Intern Med ; 58(22): 3331-3336, 2019 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-31327821

RESUMO

A 71-year-old woman being treated with methotrexate (MTX) and tacrolimus (TAC) for rheumatoid arthritis (RA) was admitted to our hospital and underwent surgery for gastric perforation and peritonitis. An endoscopic examination six days post-surgery showed an extensive ulcer in the stomach, and a biopsy revealed diffused large B-cell lymphoma. We diagnosed her with immunodeficiency-associated lymphoproliferative disorder (LPD) and discontinued the MTX and TAC. She underwent gastrectomy due to stenosis approximately two months after the first operation, but the histopathological findings of lymphoma had disappeared. LPD should be considered as a potential cause of gastric perforation during RA treatment.


Assuntos
Artrite Reumatoide/complicações , Transtornos Linfoproliferativos/complicações , Úlcera Péptica Perfurada/etiologia , Idoso , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Biópsia , Feminino , Humanos , Doença Iatrogênica , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/cirurgia , Metotrexato/uso terapêutico , Tacrolimo/uso terapêutico
4.
Vestn Otorinolaringol ; 84(2): 18-22, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31198210

RESUMO

The analysis (1998-2018) of the causes of early postoperative bleeding in 68 children with chronic lymphoproliferative syndrome and late postoperative bleeding (on the 7-10th day) in 3 children aged 3 to 17 years was performed. It was found the disorder of the platelet functional activity (adrenaline-induced platelet aggregation, adrenaline concentration - 2.5 µmol/l) in 83.8% cases (57 children). Late postoperative bleedings were developed due to the uncontrolled intake of nonsteroidal anti-inflammatory painkillers, which led to the platelet dysfunction. It has been established the decrease in the prothrombin percent by Quick in 73.5% children, and the elongation of the activated partial thromboplastin time (APTT) in the quarter of children amid the bleeding. Half of the children showed the increase of D-dimer concentration to 1.5-2 fold amid the bleeding. We offer preoperative examination: platelet count, prothrombin percent by Quick (prothrombin time), APTT and fibrinogen level. We recommend an extended hemostasis study with the detection of adhesive, aggregative and secretory functions of platelets in the children with chronic lymphoproliferative syndrome if the results of the integral screening tests differ from normal range.


Assuntos
Hemostasia , Hemostáticos , Transtornos Linfoproliferativos , Procedimentos Cirúrgicos Otorrinolaringológicos , Adolescente , Criança , Pré-Escolar , Humanos , Transtornos Linfoproliferativos/complicações , Tempo de Tromboplastina Parcial , Hemorragia Pós-Operatória/diagnóstico , Tempo de Protrombina
5.
Gan To Kagaku Ryoho ; 46(4): 736-738, 2019 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-31164519

RESUMO

A 73-year-old woman had a history of medication, including methotrexate for rheumatoid arthritis, for 5 years. She had chronic epigastralgia for 2 weeks and found to have multiple submucosal tumors on upper gastrointestinal endoscopy in another hospital. She had a strong abdominal pain thereafter and diagnosed as having gastrointestinal perforation on the basis of CT scans. Abdominal examination revealed disseminated peritonitis, and emergency laparoscopic surgery was performed on the day of admission. A 1 cm perforation of the ileum was identified, and a 5 cm mass of the mesentery near the perforation was also identified. Small bowel partial resection, including both lesions, was performed. From the intraoperative findings, methotrexate-associated lymphoproliferative disorders(MTX-LPD)was suspected, and methotrexate was discontinued after the surgery. At a later date, the pathological result from both the surgical specimen and upper gastrointestinal endoscopy was diffuse large B cell lymphoma(DLBCL). CT scan, PET-CT scan, and upper gastrointestinal endoscopy were performed 1-2 months after surgery, and no tumor was identified. Currently, 6 months after the surgery, the patient is still alive without any progression of the lymphoma.


Assuntos
Artrite Reumatoide , Perfuração Intestinal , Linfoma Difuso de Grandes Células B , Transtornos Linfoproliferativos , Metotrexato , Idoso , Artrite Reumatoide/tratamento farmacológico , Feminino , Humanos , Perfuração Intestinal/etiologia , Linfoma Difuso de Grandes Células B/complicações , Transtornos Linfoproliferativos/complicações , Metotrexato/uso terapêutico , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons
6.
Artigo em Russo | MEDLINE | ID: mdl-31156214

RESUMO

AIM: To assess the neurological and cognitive status, identify the frequency of anxiety and depression in patients with lymphoproliferative diseases, and analyze their impact on the quality of life of patients. MATERIAL AND METHODS: Fifty-eight patients, including 35 (60.34%) men and 23 (39.66%) women aged from 42 to 86 years, with a diagnosis of chronic lymphocytic leukemia (CLL) or multiple myeloma (MM) were examined. Clinical and anamnestic methods, the Montreal scale of cognitive function assessment, the Hospital Anxiety and Depression Scale (HADS), the Functional Assessment of Cancer Therapy-General (FACT-G) were administered. RESULTS: Cognitive impairment was observed in 44 (75.86%) patients. Thirty-two (56.14%) patients had no symptoms of depression, clinically diagnosed depression was observed only in 8 (14.04%). In 37 (64.91%) patients, there were no symptoms of anxiety, clinically diagnosed anxiety was revealed in 6 (10.53%). The average score on the FACT-G scale for quality of life was 62.72±23.29 with a maximum score of 108. CONCLUSION: Cognitive impairment was observed in a large number of patients. Symptoms of depression were found in less than half of the patients, and manifestations of anxiety were found in one third. The presence of affective disorders, such as anxiety and depression, reduced quality of life evaluated in all its modules.


Assuntos
Disfunção Cognitiva , Transtornos Linfoproliferativos , Qualidade de Vida , Adulto , Idoso , Idoso de 80 Anos ou mais , Ansiedade , Transtornos de Ansiedade , Depressão , Feminino , Humanos , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/psicologia , Masculino , Pessoa de Meia-Idade
7.
Arthritis Rheumatol ; 71(11): 1904-1912, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31136095

RESUMO

OBJECTIVE: Cryoglobulins are cold-precipitating immunoglobulins. Through progress in techniques, we undertook this study to update information on the biologic characteristics of cryoglobulins in a very large population. METHODS: A cohort of 13,439 patients was tested for cryoglobulins from January 2010 to December 2016. The analysis included cryoglobulin isotype, clonality, concentration, and IgM rheumatoid factor (IgM-RF) in cryoprecipitate, as well as serum complement and RF. Markers of gammopathy, viral infection, and autoimmunity were also investigated. RESULTS: Of the 13,439 patients, 1,675 (12.5%) tested positive for cryoglobulins: 155 patients (9.3%) with type I, 788 (47%) with type II, and 732 (43.7%) with type III cryoglobulins. Nine percent of patients who were retested after initially testing negative for cryoglobulins showed a positive result on a follow-up test (196 of the 2,213 retested patients). In type I cryoglobulins, IgM was more frequent but occurred at lower concentrations than IgG. Mixed cryoglobulins were found in 34.8% of the tested patients who were positive for hepatitis C virus and <5% of those who were positive for hepatitis B virus or HIV. Of the patients with anti-double-stranded DNA, anti-SSA, or anti-cyclic citrullinated peptide autoantibodies, 25.4% tested positive for mixed cryoglobulins, with type III occurring more frequently than type II. Both cryoprecipitate and serum were RF-positive in 21.6% of type II and 10.1% of type III cryoglobulins. A decrease of C4, with or without accompanying decreases of C3 and CH50, was found in 23.6% of cryoglobulin samples. CONCLUSION: Obtained with the use of modern assays, our findings from this very large collection of cryoglobulins provide an update on cryoglobulin distribution and characteristics, with minimal selection bias. Despite strict preanalytical conditions, a negative finding for the presence of cryoglobulin must be confirmed in a second sample. RF activity and complement decreases were rarely detected.


Assuntos
Anticorpos Anti-Proteína Citrulinada/imunologia , Anticorpos Antinucleares/imunologia , Proteínas do Sistema Complemento/imunologia , Crioglobulinemia/imunologia , Crioglobulinas/imunologia , Imunoglobulina M/imunologia , Fator Reumatoide/imunologia , Adulto , Idoso , Estudos de Coortes , Complemento C3/imunologia , Complemento C4/imunologia , Ensaio de Atividade Hemolítica de Complemento , Crioglobulinemia/complicações , Feminino , Infecções por HIV/complicações , Infecções por HIV/imunologia , Hepatite B/complicações , Hepatite B/imunologia , Hepatite C/complicações , Hepatite C/imunologia , Humanos , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/imunologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
8.
Medicine (Baltimore) ; 98(17): e15323, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31027102

RESUMO

RATIONALE: Indolent T-cell lymphoproliferative disease (T-LPD) of gastrointestinal tract is a rare recently described disease that seldom progresses. We report a case of T-LPD with synchronous diffuse large B-cell lymphoma (DLBCL) that cause aggravation of disease. PATIENT CONCERNS: A 46-year-old Chinese male presented with intermittent paraumbilical colic pain, bloating, and occasional diarrhea for 10 years. His condition aggravated with partial bowel obstruction recently. The patient was diagnosed as T-LPD initially based on histological result and T-cell receptor-gamma clonal gene rearrangement test. The patient was followed without chemotherapy. His condition stabilized for 1 year and then deteriorated with small intestine perforation. DIAGNOSIS: The patient was diagnosed as indolent T-LPD and DLBCL finally. INTERVENTIONS: The patient had surgery for intestine perforation and received chemotherapy for DLBCL and T-LPD afterward. OUTCOMES: At 6 months follow-up, the patient continued to have resolution of his symptoms. LESSONS: Early detection of high-grade transformation of T-LPD or the coexistence of aggressive lymphoma is essential for the patient. DLBCL may coexist in the indolent course of T-LPD. The diagnosis of T-LPD should be made cautiously in case with progressing symptoms such as intestinal obstruction.


Assuntos
Linfoma Difuso de Grandes Células B/complicações , Transtornos Linfoproliferativos/complicações , Antineoplásicos/uso terapêutico , Humanos , Perfuração Intestinal/complicações , Perfuração Intestinal/cirurgia , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Linfócitos T
9.
Clin Liver Dis ; 23(2): 293-308, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30947878

RESUMO

Hepatic abnormalities in patients with lymphoproliferative disorders are common and can occur from direct infiltration by abnormal cells, bile duct obstruction, paraneoplastic syndrome, hemophagocytic syndrome, drug-induced liver injury, opportunistic infections, and reactivation of viral hepatitis. Hepatic involvement by lymphoma is often in association with systemic disease and rarely seen as a primary hepatic lymphoma. Vanishing bile duct syndrome is a well-known complication of Hodgkin disease. Antiviral prophylaxis for hepatitis B virus (HBV) reactivation is recommended for all HBsAg+ patients undergoing chemotherapy and all resolved HBV patients undergoing rituximab therapy and stem cell transplantation.


Assuntos
Hepatopatias/etiologia , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/tratamento farmacológico , Infecções Oportunistas/complicações , Síndromes Paraneoplásicas/etiologia , Hepacivirus/fisiologia , Vírus da Hepatite B/fisiologia , Hepatite B Crônica/etiologia , Hepatite C Crônica/etiologia , Doença de Hodgkin/complicações , Humanos , Neoplasias Hepáticas/etiologia , Linfoma não Hodgkin/complicações , Transtornos Linfoproliferativos/etiologia , Transplante de Órgãos/efeitos adversos , Ativação Viral
10.
Clin J Gastroenterol ; 12(5): 434-440, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30968266

RESUMO

Chronic diarrhea is one of the major symptoms in gastroenterology. However, this may be caused by pathologic conditions for which the diagnosis is critical. Villous atrophy, as an endoscopic lesion, accompanied by chronic diarrhea can occasionally be observed in the patients with inflammatory diseases of the gastrointestinal (GI) tract. Herein, we present a case with persistent diarrhea accompanied by intestinal wall thickening without any other significant endoscopic features other than villous atrophy in the jejunum and the ileum, where we diagnosed as an indolent T cell lymphoproliferative disorder (T-LPD) of the GI tract, defined in the 2016-2017 revised World Health Organization classification, via single-balloon enteroscopy (SBE). Interestingly, we found the same lymphocyte infiltration from the distal third portion of the duodenum, where gastroscopy could not reach, via SBE, even though no endoscopic findings were observed such as villous atrophy. Since infiltrating cells in the intestinal tissues were CCR4+, mogamulizumab was administered with resulting durable symptomatic remission for more than 2 years. Patients with persistent diarrhea may have serious small intestinal disorder including not only chronic inflammatory diseases but also lymphoid neoplasmic conditions including T-LPD of GI tract.


Assuntos
Intestino Delgado/patologia , Transtornos Linfoproliferativos/diagnóstico , Enteroscopia de Balão Único/métodos , Linfócitos T/patologia , Idoso , Atrofia/etiologia , Atrofia/patologia , Biópsia , Diarreia/etiologia , Humanos , Intestino Delgado/imunologia , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/patologia , Masculino , Tomografia Computadorizada por Raios X
11.
J Am Acad Dermatol ; 81(2): 534-540, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30654082

RESUMO

BACKGROUND: Hydroa vacciniforme-like lymphoproliferative disorder (HVLLPD) is a rare Epstein-Barr virus (EBV)-related disease that is usually found in East Asians and Latin Americans. OBJECTIVE: To report the characteristics of HVLLPD in Chinese patients. METHODS: Retrospective analysis of patients with HVLLPD from a single institute. RESULTS: A total of 41 patients were enrolled. All patients presented with papulovesicular lesions, mainly distributed on sun-exposed areas, with 26 patients showing systemic symptoms. Follow-up data were available for 20 patients, 16 patients were alive, and 4 patients died. Of the 4 deceased patients, 3 had taken a serum EBV DNA test that showed high viral loads. These 3 patients also received chemotherapy. Histopathology was characterized by dense proliferation of lymphocytes in the dermis. Angiotropism or angiodestruction was found in the majority of patients, whereas prominent cellular polymorphism was noticed in only 4 patients. All patients were positive for CD3, TIA1 cytotoxic granule associated RNA binding protein, and EBV-encoded RNA in situ hybridization. LIMITATIONS: This was a retrospective study. CONCLUSIONS: HVLLPD in Chinese patients showed indolent behavior in the majority of cases, which differed from the characteristics of HVLLPD in Latin Americans. Patients with high serum EBV DNA loads had an increased risk of their disease evolving into aggressive disease. Chemotherapy should not be considered as first-line treatment for most Chinese patients.


Assuntos
DNA Viral/sangue , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4 , Transtornos Linfoproliferativos/sangue , Transtornos Linfoproliferativos/patologia , Adolescente , Adulto , Antígenos CD/metabolismo , Relação CD4-CD8 , Criança , Pré-Escolar , China , Infecções por Vírus Epstein-Barr/sangue , Feminino , Humanos , Hidroa Vaciniforme/patologia , Lactente , Linfócitos/metabolismo , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/virologia , Masculino , Estudos Retrospectivos , Carga Viral , Adulto Jovem
12.
Haemophilia ; 25(2): 252-257, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30694571

RESUMO

INTRODUCTION: Acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder caused by neutralizing antibodies against factor VIII (FVIII). Despite significant initial morbidity and mortality, most patients achieve remission with immunosuppressive therapy. AIM: Long-term follow-up data from the Quebec Reference Centre for Inhibitors (QRCI) were analysed to identify factors predictive of AHA relapse and the influence of relapse on survival. METHODS: Criteria used to define AHA were levels of FVIII <0.3 IU/mL and FVIII inhibitor titres ≥0.6 Bethesda Units (BU). Complete remission was defined as FVIII >0.5 IU/mL and/or FVIII inhibitor titres <0.6 BU while not on immunosuppression. RESULTS: Between 2000 and 2012, 111 subjects met the inclusion criteria and were followed for a median of 25.6 months. Ninety per cent of them reached remission on immunosuppression in a median time of 45 days. Fourteen patients presented one or more relapses in a median time of 13.4 months. Most relapse episodes were successfully treated. Associated lymphoproliferative syndromes (LPS) were predictive of relapse, whereas FVIII activity and inhibitor titres at initial diagnosis or immunosuppressive regimens were not. The overall survival (OS) was the same, with or without relapse. CONCLUSION: Among the recognized potential risk factors for relapse, only LPS was statistically significant. The long-term follow-up of our patients also showed that late or multiple relapses may occur, but that relapse is not associated with a worse OS. Thus, long-term follow-up is important for optimal management of AHA.


Assuntos
Hemofilia A/diagnóstico , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Coagulantes/uso terapêutico , Fator VIII/análise , Seguimentos , Hemofilia A/tratamento farmacológico , Hemofilia A/mortalidade , Humanos , Imunossupressores/uso terapêutico , Isoanticorpos/sangue , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/diagnóstico , Recidiva , Indução de Remissão , Fatores de Risco , Taxa de Sobrevida
15.
Br J Nurs ; 27(22): S20-S26, 2018 Dec 13.
Artigo em Inglês | MEDLINE | ID: mdl-30525969

RESUMO

Post-transplant lymphoproliferative disorders (PTLD) are lymphomas that may arise in organ, bone marrow or stem cell transplant recipients who are taking immunosuppressive drugs to prevent rejection of the transplant. The likelihood of developing PTLD depends on the type of transplant. PTLD is a potentially severe complication of post-transplant treatment, with an uncertain prognosis. Lymphoproliferative disorders can also occur in people taking immunosuppressants for inflammatory bowel disease. This article will explore PTLD and discuss the experience of caring for patients who developed lymphoproliferative conditions and required emergency stoma formation. The emotional and physical impact of surgery upon these patients, who have already experienced a protracted treatment journey, will be explored. Implications for practice for the lymphoma team and stoma nurse specialists involved with the care of these patients will also be considered.


Assuntos
Imunossupressores/uso terapêutico , Transtornos Linfoproliferativos/tratamento farmacológico , Transplante de Órgãos , Estomia/enfermagem , Estomas Cirúrgicos/efeitos adversos , Adulto , Doença de Crohn/complicações , Doença de Crohn/tratamento farmacológico , Feminino , Humanos , Transtornos Linfoproliferativos/complicações , Masculino , Processo de Enfermagem , Complicações Pós-Operatórias/enfermagem , Complicações Pós-Operatórias/prevenção & controle
16.
Zhonghua Er Ke Za Zhi ; 56(10): 759-764, 2018 Oct 02.
Artigo em Chinês | MEDLINE | ID: mdl-30293280

RESUMO

Objective: To summarize the clinical characteristics, virological and histopathological features, clinical outcome of Epstein-Barr virus-positive lymphoproliferative disease (EBV(+)LPD) in children. Methods: The clinical and follow-up data of 13 children histopathologically diagnosed as EBV(+)LPD in the Department of Infectious Disease of Beijing Children's Hospital between January 2011 and December 2016 were summarized. Results: Of the 13 patients, 5 were males and 8 females. The median age of disease onset was 6.0 years (range 1.3 to 15.0 years). The median duration between disease onset and diagnosis was 3 months (range 1 to 24 months). All the 13 patients had fever, 9 cases had hepatosplenomegaly and lymphoadenopathy, 4 cases had only lymphoadenopathy, 7 cases had reduced peripheral blood cells, 7 cases had lung involvement, 3 cases had central nervous system involvement, 3 cases had cardiac involvement, 3 cases had intestinal involvement, 2 cases had skin involvement and 1 case had abdominal mass. All the 13 patients underwent whole blood EBV-DNA PCR examination and the copies ranged from 1×10(8)/L to 1×10(11)/L. Pathology of lymph node confirmed 6 cases, skin pathology confirmed 2 cases, lung pathology, ileum mucosa pathology, liver pathology, abdominal mass pathology and bone marrow pathology confirmed 1 case each. Among 13 patients, 9 cases presented with EBV-positive T cell lymphoproliferative disease(EBV(+) T-LPD), 2 cases with hydroa vacciniforme (HV) and 2 cases with EBV-positive diffuse large B-cell lymphoma (EBV(+) DLBCL) . All the patients were followed up for 2 days to 65 months after discharge. Among 9 cases of EBV(+)T-LPD, 1 case died in a short time, 1 case died after evolved to T-cell lymphoma, 2 cases recovered after hematopoietic stem cell transplantation, 1 case recovered after the chemotherapy of hemophagocytic lymphohistiocytosis(HLH) 2004 protocol and 4 cases were stable now. Of 2 cases of HV patients, 1 case died after evolved to HV like lymphoma and the other still have symptoms. Among 2 cases of EBV(+) DLBCL, 1 case died shortly after discharge and the other was still stable after chemotherapy. Conclusions: Chronic recurrent fever, lymphadenopathy and hepatosplenomegaly are the most common clinical manifestations in children with EBV(+)LPD. Involvement of lung, central nervous system, intestinal tract, skin and other organs are also involved frequently. For children with chronic fever of unknown cause and accompanied by lymphadenopathy and (or) hepatosplenomegaly, EBV (+) LPD should be considered highly when the whole blood EBV-DNA load continues to increase significantly, early biopsy of the proliferative lesion should be performed to make a definite diagnosis. The prognosis of EBV (+) LPD is poor, and some evolve to lymphoma, hematopoietic stem cell transplantation is an effective way to treat this disease.


Assuntos
Infecções por Vírus Epstein-Barr , Herpesvirus Humano 4 , Transtornos Linfoproliferativos , Adolescente , Idade de Início , Criança , Pré-Escolar , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/patologia , Feminino , Humanos , Hidroa Vaciniforme , Lactente , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/patologia , Transtornos Linfoproliferativos/virologia , Masculino , Linfócitos T
19.
J Clin Immunol ; 38(5): 579-588, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29956079

RESUMO

The gastrointestinal tract is heavily populated with innate and adaptive immune cells that have an active role in preservation of mucosal homeostasis and prevention of inflammation. Inflammatory bowel diseases are thought to result from dysregulated immune function that is influenced by genetic background, environmental triggers, and microbiome changes. While most inflammatory bowel disease patients present in adolescent years or adulthood, in a minority of cases, the disease develops early in life, and in some of these young patients, a monogenic disease causing intestinal inflammation can be identified. Many of these conditions result from mutations in immune-mediated genes and can present with or without concomitant recurrent infections. In this review, we will discuss the treatment of patients with selected primary immunodeficiencies and inflammatory bowel diseases. We will focus on five conditions resulting from mutations in IL10/IL10 receptor, NADPH oxidase complex, XIAP, LRBA, and CTLA-4.


Assuntos
Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/imunologia , Doenças Inflamatórias Intestinais/etiologia , Doenças Inflamatórias Intestinais/terapia , Proteínas Adaptadoras de Transdução de Sinal/deficiência , Animais , Antígeno CTLA-4/genética , Gerenciamento Clínico , Suscetibilidade a Doenças , Doenças Genéticas Ligadas ao Cromossomo X/complicações , Doenças Genéticas Ligadas ao Cromossomo X/genética , Doenças Genéticas Ligadas ao Cromossomo X/metabolismo , Humanos , Doenças Inflamatórias Intestinais/diagnóstico , Interleucina-10/deficiência , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/metabolismo , Mutação , NADPH Oxidases/genética , Receptores de Interleucina-10/deficiência
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