RESUMO
PURPOSE: Gamma/delta T lymphocytes constitute a singular population due to their particular antigenic recognition and their localization inside the epithelium. Their functions are complementary to those of the alpha/beta T-cells and they are involved in the defense and regulation of the immune system. Their role in human diseases is not very well understood and the aim of our study was to analyze a population of patients with a peripheral gamma/delta T-cell lymphocytosis. METHODS: The study included 55 patients, recruited from 1997 to 2000, with a peripheral gamma/delta T lymphocytosis (defined by a proportion of gamma/delta T-cells of over 10% of total peripheral T lymphocytes). Analysis of the lymphocyte population was obtained by cytometry after peripheral blood sampling. RESULTS: Three main groups of diseases were observed: infectious diseases (viral infections and tuberculosis), inflammatory diseases (sarcoidosis and autoimmune diseases) and blood diseases (monoclonal gammopathies and hemopathies). Persistence of gamma/delta T lymphocytosis was dependent on the underlying disease (transitional when associated with an infectious disease and lasting when associated with sarcoidosis). The rest of the immunophenotyping analysis was usually normal. CONCLUSION: Our results confirm the data published in the literature concerning the role of the gamma/delta T lymphocytes in infectious, inflammatory and autoimmune diseases and neoplasias. These data are in agreement with the cytotoxic and regular functions of these lymphocytes.
Assuntos
Linfocitose/sangue , Receptores de Antígenos de Linfócitos T gama-delta , Linfócitos T , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Occupational exposure to vinyl chloride monomers is known to induce Raynaud's phenomenon, periportal fibrosis, liver angiosarcoma and scleroderma-like syndrome. We report the first case of occupational polymyositis in a 58-year-old man exposed to vinyl chloride. A dysimmune process was strongly suspected as having induced vinyl chloride disease. Our patient had an anti-histidyl-t-RNA-synthetase (Jo1) antibody, which has never to our knowledge been reported in this occupational disease.
Assuntos
Anticorpos Antinucleares/imunologia , Doenças Profissionais/induzido quimicamente , Exposição Ocupacional/efeitos adversos , Polimiosite/induzido quimicamente , Cloreto de Vinil/efeitos adversos , Anticorpos Antinucleares/análise , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Profissionais/imunologia , Polimiosite/diagnóstico , Medição de Risco , Síndrome , Fatores de TempoRESUMO
INTRODUCTION: Acute parvovirus B19 infection induces a transient inhibition of erythroid cell formation, which may induce an erythroblastopenia crisis in patients suffering from chronic hemolytic anemia. EXEGESIS: We report here an exceptional observation of acute erythroblastopenia crisis with good outcome, occurring at the same time in a mother and her son, both suffering from hereditary spherocytosis. Diagnosis of parvovirus infection is based on detection of serum parvovirus B19-specific immunoglobulin M antibodies in the mother and her son and by the positivity of parvovirus B19 DNA detected by PCR in serum in the mother. Outcome was good, with the end of the erythroblastopenia crisis obtained 7 to 10 days later, but requiring blood transfusion in the mother. CONCLUSION: Our observation is a reminder that the contagiosity of parvovirus B19 is high in household contacts and that protection of family members should rapidly be considered in hereditary spherocytosis.
Assuntos
Anemia/genética , Anemia/virologia , Eritema Infeccioso/complicações , Eritema Infeccioso/transmissão , Eritroblastos , Parvovirus B19 Humano , Esferocitose Hereditária/complicações , Doença Aguda , Adulto , Anemia/sangue , Anemia/diagnóstico , Anemia/terapia , Anticorpos Antivirais/sangue , Transfusão de Sangue , Criança , Doença Crônica , Eritema Infeccioso/sangue , Eritema Infeccioso/diagnóstico , Eritema Infeccioso/imunologia , Feminino , Humanos , Imunoglobulina M/imunologia , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Parvovirus B19 Humano/imunologia , Reação em Cadeia da PolimeraseAssuntos
Adenoma/complicações , Adenoma/diagnóstico , Hematoma/etiologia , Doenças Mandibulares/etiologia , Doenças do Mediastino/etiologia , Neoplasias das Paratireoides/complicações , Neoplasias das Paratireoides/diagnóstico , Idoso , Diagnóstico Diferencial , Humanos , Masculino , Dor/etiologia , Ruptura Espontânea , Tomografia Computadorizada por Raios XRESUMO
Polysplenia syndrome includes a group of congenital abnormalities involving the spleen, gastrointestinal tract, liver, and cardiovascular system. A case of severe lower limbs lymphedema occurring in a young woman with polysplenia, azygous continuation of the inferior vena cava, short pancreas, and preduodenal portal vein is reported. Lower limb lymphedema could represent a new clinical manifestation associated with polysplenia syndrome. Lymphedema could be the result of a primary anomaly of the lymphatic system or be induced by high pressure in the venous system or by compression of the lymphatic circulation by the hypertrophic azygous vessels in the posterior mediastinum.
Assuntos
Anormalidades Múltiplas/diagnóstico , Fígado/anormalidades , Linfedema/etiologia , Baço/anormalidades , Adulto , Sistema Cardiovascular/fisiopatologia , Doença Crônica , Diagnóstico Diferencial , Anormalidades do Sistema Digestório , Feminino , Humanos , Perna (Membro) , Mediastino/anormalidades , Veia Porta/anormalidades , Índice de Gravidade de Doença , Síndrome , Tomografia Computadorizada por Raios X , Veia Cava Inferior/anormalidadesRESUMO
INTRODUCTION: Neurological involvement is observed in 5% of cases of sarcoidosis and includes impairment of the central nervous system, the meninges, and the cranial and peripheral nerves. Besides neurological defects, cognitive impairment may be encountered ranging from isolated memory defect to dementia. EXEGESIS: We report a case of neurosarcoidosis occurring in a 40-year-old woman, a native of Reunion Island, with initial meningeal and hypophyseal involvement. Three years later, while treated with low dose prednisolone and methotrexate, she presented a paranoid state associated with cognitive impairment of frontal type and severe behavioral disturbances. After 2 years of high dose steroid treatment associated with hydroxychloroquine, her behavioral status improved, allowing social and familial reinsertion. CONCLUSION: In our observation, sarcoidosis was revealed through a central neurological impairment, with chronic meningitis, facial nerve palsy, and, finally, through psychiatric symptoms and severe behavioral disturbances. A slow favorable outcome was obtained using high dose methylprednisolone and hydroxychloroquine with total regression of behavioral disturbances but with persisting cognitive alteration.
Assuntos
Transtornos Cognitivos/etiologia , Paralisia Facial/etiologia , Meningite/etiologia , Doenças do Sistema Nervoso/etiologia , Transtornos Neurocognitivos/etiologia , Transtornos Paranoides/etiologia , Sarcoidose/etiologia , Adulto , Antirreumáticos/uso terapêutico , Transtornos Cognitivos/diagnóstico , Quimioterapia Combinada , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Imageamento por Ressonância Magnética , Metotrexato/uso terapêutico , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/tratamento farmacológico , Transtornos Paranoides/diagnóstico , Prednisolona/uso terapêutico , Prognóstico , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Resultado do TratamentoRESUMO
PURPOSE: Chronic pericarditis or recurrent pericarditis is mostly considered to be idiopathic even when up-to-date medical investigations are undertaken. The absence of aetiology and the associated inflammatory process are features of a common disease for internists. As there are only a few published reports on this disease, therapeutic options are not easily envisaged. CURRENT KNOWLEDGE AND KEY POINTS: Idiopathic pericarditis and its evolution, characterized by recurrence or chronicity, has long been diagnosed and studied. Faced with a case of acute pericarditis, no clinical or biological data can preclude evolution towards a chronic or a recurrent form. The two major complications are tamponade and constriction. Classical treatment is aspirin and nonsteroidal anti-inflammatories. Steroids have a spectacular effect but steroid dependence is frequently observed. Colchicine treatment seems to be efficacious and can be used to stop steroid therapy. There are only a few published reports on the importance of immunosuppressive drugs such as azathioprine and cyclophosphamide. FUTURE PROSPECTS AND PROJECTS: Through our own experience and literature review, we propose to consider chronic and/or recurrent pericarditis as an autonomous inflammatory disease of the pericardium. Thus, large-scale studies concerning the treatment should improve the outcome of patients.
Assuntos
Pericardite , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Inflamatórios não Esteroides/uso terapêutico , Aspirina/uso terapêutico , Doença Crônica , Colchicina/uso terapêutico , Ecocardiografia , Eletrocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pericardite/diagnóstico , Pericardite/tratamento farmacológico , Pericardite/fisiopatologia , Pericardite/cirurgia , Inibidores da Agregação Plaquetária/uso terapêutico , RecidivaRESUMO
Visceral leishmaniasis or Kala-azar is an endemic parasitic infection in Mediterranean countries. We report an interesting case occurring in a 38-year-old woman suffering from systemic lupus erythematosus and secondary antiphospholipid syndrome. In our patient visceral leishmaniasis occurred during high dose-steroids treatment mimicking a flare of lupus. As the lupus resolved under immunosuppressive treatment, a reactivation of visceral leishmaniasis was observed and was confirmed by the successive serological tests which showed crossing of leishmania and antinuclear antibody titers. Our case shows that, faced with fever occurring in lupus patients in an endemic area, visceral leishmaniasis should be searched for before intensifying immunosuppressive treatments.
Assuntos
Anticorpos Antinucleares/sangue , Anticorpos Antiprotozoários/sangue , Síndrome Antifosfolipídica/imunologia , Leishmaniose Visceral/diagnóstico , Leishmaniose Visceral/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Adulto , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/complicações , Antirreumáticos/uso terapêutico , Diagnóstico Diferencial , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Hospedeiro Imunocomprometido , Leishmaniose Visceral/complicações , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológicoRESUMO
PURPOSE: Antiphosphatidylethanolamine antibodies (aPE) are zwitterionic antiphospholipid antibodies that have been recently described in antiphospholipid syndrome. IgM is the most frequently observed isotype. We assessed the potential relationship between serum IgM levels and their presence. METHODS: Seventy-two patients (66 females, 6 males) positive for aPE of the IgM isotype were followed up for 2 years. They suffered from either an autoimmune disease or had clinical signs suggesting the existence of antiphospholipid syndrome or other immune disorders. Seventy-two control patients (58 females, 14 males) of similar age, with other immune disorders were also included in the study. For each of them, the IgM level was obtained and hyper-immunoglobulinemia M (hyper-IgM) was defined as a value upper than 2.5 g/L. Search for aPE of the IgM isotype was made using an in-house Elisa test. RESULTS: Half of the aPE-positive patients had serum hyper-IgM, whereas none of the aPE-negative patients had hyper-IgM. All IgMs were polyclonal. There was a positive correlation (r = 0.57, P = 0.001) between the level of IgM and the optical densities obtained by Elisa. The presence of a hyper-IgM did not modify the clinical manifestations (arterial and venous thromboses, recurrent fetal losses), nor the positivity of lupus anticoagulant, anticardiolipin and anti-beta-2-glycoprotein 1 antibodies. CONCLUSION: The positive correlation between the IgM level and aPE level is of value, as it suggests a possible relationship between lymphocytic activation and auto-antibodies production, which does not concern only aPE. Further studies regarding the antigenic specificity of IgM will probably provide further insights on IgM and phospholipid interactions.
Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/imunologia , Doenças Autoimunes/sangue , Isotipos de Imunoglobulinas/sangue , Imunoglobulina M/sangue , Fosfatidiletanolaminas/imunologia , Adulto , Síndrome Antifosfolipídica/sangue , Doenças Autoimunes/imunologia , Ensaio de Imunoadsorção Enzimática , Feminino , Seguimentos , Humanos , Ativação Linfocitária , Masculino , Pessoa de Meia-Idade , Valores de ReferênciaAssuntos
Antitrombinas/metabolismo , Colite Ulcerativa/complicações , Trombocitose/etiologia , Colite Ulcerativa/sangue , Colite Ulcerativa/tratamento farmacológico , Feminino , Fármacos Gastrointestinais/efeitos adversos , Fármacos Gastrointestinais/uso terapêutico , Humanos , Pessoa de Meia-Idade , Sulfassalazina/efeitos adversos , Sulfassalazina/uso terapêutico , Trombocitose/sangueRESUMO
Intracardiac thrombi have rarely been reported in patients with the antiphospholipid syndrome. We describe a new case revealed by systematic echocardiography in an asymptomatic woman who consulted for mild thrombocytopenia. Our case is characterized by a past history of migraine, fetal loss and psychiatric disturbances and by a high level of antiphospholipid antibodies (anticardiolipin, anti-beta(2)-glycoprotein I and lupus anticoagulant). Echography, in the absence of any cardiovascular symptom, showed bulky right intra-atrial thrombi requiring surgical excision. Histopathological analysis revealed the fibrino-cruoric nature of the lesion without myxoma. This case shows that, when faced with a mild thrombocytopenia associated with antiphospholipid antibodies, echocardiography may reveal a life-threatening and completely asymptomatic intracardiac thrombosis.
Assuntos
Síndrome Antifosfolipídica/complicações , Trombose Coronária/etiologia , Adulto , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/diagnóstico , Trombose Coronária/diagnóstico por imagem , Trombose Coronária/cirurgia , Transtorno Depressivo/complicações , Diagnóstico Diferencial , Ecocardiografia , Feminino , HumanosAssuntos
Injúria Renal Aguda/tratamento farmacológico , Corticosteroides/uso terapêutico , Doença Antimembrana Basal Glomerular/tratamento farmacológico , Embolia de Colesterol/tratamento farmacológico , Hemorragia/tratamento farmacológico , Injúria Renal Aguda/etiologia , Idoso , Doença Antimembrana Basal Glomerular/etiologia , Embolia de Colesterol/complicações , Hemorragia/etiologia , Humanos , Masculino , SíndromeRESUMO
The antisynthetases syndrome associates lung disease, polymyositis, Raynaud's phenomenon and frequently polyarthritis. Anti-amioacyl-tRNA synthetase antibodies are present. The most common is anti-Jol. We report a case with severe arteritis of the finger with multiple zones of skin necrosis which responded favourably to corticosteroids given in bolus and cyclophosphamid. Finger arteritis, in addition to classical Raynaud's phenomenon, may be a new manifestation of the anti-synthetases syndrome.