RESUMO
CASE PRESENTATION: A 71-year-old man with history of gastroesophageal reflux disease, chronic sinusitis, arthritis, hypothyroidism, and anemia of chronic disease initially sought treatment with a recurrent left pleural effusion along with other abnormal lung findings on chest CT scan. Before his referral, he was being managed for 3 years at his local hospital for waxing and waning fevers, fatigue, productive cough, chills, and night sweats. He did not report any hemoptysis or chest pain, but reported weight loss of 13 kgs in 15 months. During those 3 years, he was treated with multiple courses of antibiotics and steroids with temporary relief of symptoms. At that time, his chronic sinusitis was suspected to be the cause of his symptoms and he underwent balloon sinuplasty. He was receiving daily sublingual immunotherapy for inhaled respiratory allergens for the previous year after showing positive test results for 17 inhaled allergens. The patient had no other known immunologic workup before our evaluation.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Pulmão/diagnóstico por imagem , Granulomatose Linfomatoide/diagnóstico , Idoso , Broncoscopia , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Empiema/fisiopatologia , Infecções por Vírus Epstein-Barr , Febre/fisiopatologia , Humanos , Leucocitose/fisiopatologia , Pulmão/patologia , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/fisiopatologia , Granulomatose Linfomatoide/virologia , Masculino , Prednisona/uso terapêutico , Rituximab/uso terapêutico , Tomografia Computadorizada por Raios X , Vincristina/uso terapêuticoRESUMO
Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease. The atypical lymphoid cells directly accumulate within affected tissues and clinically present in the form of infiltrative lesions. It is usually a progressive disorder that virtually always involves the lung and characteristically presents as bilateral pulmonary nodules. Other commonly affected organ systems include the skin, central nervous system, and kidneys. The rareness of LYG in conjunction with its nonspecific presentation contributes to delays in diagnosis in many situations. Pathologically, it is characterized by the presence of an angiocentric and angiodestructive accumulation of varying numbers of T cells with varying numbers of atypical clonal EBV-positive B cells in a polymorphous inflammatory background. It can be subclassified using a grading system based on the number of EBV-positive large B-cell malignant cells, which is critical in selecting appropriate management strategies. Lower-grade LYG occasionally undergoes spontaneous remission and is best managed with strategies designed to enhance the host's underlying immune system, whereas high-grade LYG is best managed by combination chemoimmunotherapy but has inferior outcomes. Lymphomatoid granulomatosis can lead to progressive pulmonary failure, central nervous system disease, or progression to overt EBV-positive lymphoma without appropriate recognition and management. Improvements in the modern understanding of the biology of LYG, particularly the precise role of EBV in its pathogenesis, offer promise in the development of improved management strategies.
Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Herpesvirus Humano 4/patogenicidade , Linfoma não Hodgkin , Granulomatose Linfomatoide , Linfócitos B/patologia , Linfócitos B/virologia , Sistema Nervoso Central/patologia , Humanos , Pulmão/patologia , Linfoma não Hodgkin/patologia , Granulomatose Linfomatoide/diagnóstico , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/fisiopatologia , Granulomatose Linfomatoide/virologia , Rituximab , Pele/patologia , Linfócitos T/patologia , Linfócitos T/virologiaRESUMO
Pulmonary lymphomatoid granulomatosis is a rare diagnosis that frequently presents with a constellation of seemingly unrelated signs and symptoms. It can present with bilateral pulmonary nodules, subcutaneous skin nodules, renal nodules, and peripheral neuropathy. Its protean manifestation, both clinically and radiologically, may delay a definitive diagnosis. We present the case of a patient who had thoracotomy twice and waited for nearly a year to get the final diagnosis because of the presence of a variety of seemingly unrelated symptoms.
Assuntos
Neoplasias Pulmonares/diagnóstico por imagem , Granulomatose Linfomatoide/diagnóstico por imagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Diagnóstico Diferencial , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pulmonares/fisiopatologia , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/fisiopatologia , Masculino , Pessoa de Meia-Idade , Testes de Função Respiratória , Tomografia Computadorizada por Raios XAssuntos
Granulomatose Linfomatoide , Doenças do Sistema Nervoso , Adulto , Evolução Fatal , Humanos , Granulomatose Linfomatoide/complicações , Granulomatose Linfomatoide/patologia , Granulomatose Linfomatoide/fisiopatologia , Masculino , Doenças do Sistema Nervoso/etiologia , Doenças do Sistema Nervoso/patologia , Doenças do Sistema Nervoso/fisiopatologiaRESUMO
OBJECTIVE: To report the successful treatment of a patient with lymphomatoid granulomatosis (LYG), a rare Epstein-Barr virus-positive lymphoproliferative disorder, using rituximab (anti-CD20 monoclonal antibody). The prognosis for LYG has been reported to be poor, and no satisfactory treatment has been established. Because central nervous system (CNS) involvement of LYG has been known to show poor prognosis, the establishment of an effective treatment for CNS LYG with mild adverse effects is desired. DESIGN: Case report. SETTING: University hospital. PATIENT: A 48-year-old Japanese man presenting with slowly progressive spastic paraparesis diagnosed as LYG involving the CNS and lungs. INTERVENTIONS: The patient was treated with rituximab (375 mg/m2, once weekly for 1 month) alone. Main Outcome Measure Improvement of the lesions on imaging. RESULTS: The neurological signs resolved and the lesions in the CNS and lungs were mostly diminished after the rituximab monotherapy without any adverse effects. The patient stayed in remission for 18 months. CONCLUSION: Rituximab monotherapy was effective in treating the patient; hence, rituximab should be considered as the initial treatment against LYG involving the CNS.
Assuntos
Anticorpos Monoclonais/administração & dosagem , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias Pulmonares/tratamento farmacológico , Granulomatose Linfomatoide/tratamento farmacológico , Anticorpos Monoclonais Murinos , Encéfalo/efeitos dos fármacos , Encéfalo/patologia , Encéfalo/fisiopatologia , Sistema Nervoso Central/efeitos dos fármacos , Sistema Nervoso Central/patologia , Sistema Nervoso Central/fisiopatologia , Neoplasias do Sistema Nervoso Central/patologia , Neoplasias do Sistema Nervoso Central/fisiopatologia , Infecções por Vírus Epstein-Barr/complicações , Humanos , Fatores Imunológicos/administração & dosagem , Pulmão/efeitos dos fármacos , Pulmão/patologia , Pulmão/fisiopatologia , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/fisiopatologia , Linfócitos/imunologia , Linfócitos/patologia , Linfócitos/virologia , Granulomatose Linfomatoide/patologia , Granulomatose Linfomatoide/fisiopatologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Paraparesia Espástica/tratamento farmacológico , Paraparesia Espástica/etiologia , Paraparesia Espástica/fisiopatologia , Indução de Remissão , Rituximab , Medula Espinal/efeitos dos fármacos , Medula Espinal/patologia , Medula Espinal/fisiopatologia , Resultado do TratamentoAssuntos
Implantes de Mama/efeitos adversos , Febre/etiologia , Granuloma de Células Gigantes/induzido quimicamente , Linfonodos/patologia , Granulomatose Linfomatoide/induzido quimicamente , Silicones/toxicidade , Axila/patologia , Doxiciclina/uso terapêutico , Feminino , Granuloma de Células Gigantes/tratamento farmacológico , Granuloma de Células Gigantes/fisiopatologia , Humanos , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/fisiopatologia , Pessoa de Meia-Idade , Fatores de TempoRESUMO
Lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose chemotherapy and autologous peripheral stem cell transplantation for multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose clinical course following ABMT is complicated by fevers, in the absence of an identifiable infectious process.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Pneumopatias/etiologia , Granulomatose Linfomatoide/etiologia , Mieloma Múltiplo/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Terapia Combinada/efeitos adversos , Feminino , Infecções por Herpesviridae/etiologia , Herpesvirus Humano 4/isolamento & purificação , Humanos , Pneumopatias/fisiopatologia , Granulomatose Linfomatoide/fisiopatologia , Pessoa de Meia-Idade , Transplante Autólogo , Infecções Tumorais por Vírus/etiologiaRESUMO
PURPOSE: To evaluate the perfusion magnetic resonance (MR) imaging characteristics of cerebral toxoplasmosis and lymphoma in patients with acquired immunodeficiency syndrome (AIDS). MATERIALS AND METHODS: Perfusion MR imaging was performed prospectively in 13 patients with AIDS who had contrast material-enhancing focal brain lesions (six with active lymphoma, five with toxoplasmosis, one with treated lymphoma in remission, and one with toxoplasmosis plus lymphomatoid granulomatosis). Regional cerebral blood volume (rCBV) was determined by using dynamic echo-planar MR imaging during bolus injection of a gadolinium chelate. RESULTS: The rCBV was decreased (44% +/- 24 [standard deviation] of rCBV in the contralateral regions) throughout the toxoplasmosis lesions and in the surrounding edema of both lesion types, whereas all active lymphomas displayed areas of increased rCBV (258% +/- 99). These differences were significant (P < .005). CONCLUSION: Reduced rCBV i toxoplasmosis lesions is probably due to a lack of vasculature within the abscess; increased rCBV in lymphomas is probably due to hypervascularity in foci of active tumor growth; and decreased rCBV in the edema is probably due to vasoconstriction associated with increased interstitial pressure. Perfusion MR imaging is a rapid, noninvasive tool that may allow differentiation between cerebral lymphoma and toxoplasmosis in patients with AIDS.
Assuntos
Infecções Oportunistas Relacionadas com a AIDS/diagnóstico , Neoplasias Encefálicas/diagnóstico , Encéfalo/irrigação sanguínea , Linfoma Relacionado a AIDS/diagnóstico , Imageamento por Ressonância Magnética , Toxoplasmose Cerebral/diagnóstico , Infecções Oportunistas Relacionadas com a AIDS/fisiopatologia , Adulto , Velocidade do Fluxo Sanguíneo/fisiologia , Edema Encefálico/diagnóstico , Edema Encefálico/fisiopatologia , Neoplasias Encefálicas/irrigação sanguínea , Imagem Ecoplanar , Feminino , Humanos , Aumento da Imagem , Processamento de Imagem Assistida por Computador , Linfoma Relacionado a AIDS/fisiopatologia , Granulomatose Linfomatoide/diagnóstico , Granulomatose Linfomatoide/fisiopatologia , Masculino , Pessoa de Meia-Idade , Sensibilidade e Especificidade , Toxoplasmose Cerebral/fisiopatologia , Resistência Vascular/fisiologiaAssuntos
Doenças do Sistema Nervoso Central/etiologia , Vasculite/complicações , Síndrome de Behçet/fisiopatologia , Síndrome de Churg-Strauss/fisiopatologia , Diagnóstico Diferencial , Arterite de Células Gigantes/fisiopatologia , Granulomatose com Poliangiite/fisiopatologia , Humanos , Granulomatose Linfomatoide/fisiopatologia , Poliarterite Nodosa/fisiopatologia , Arterite de Takayasu/fisiopatologia , Vasculite/classificação , Vasculite/diagnóstico , Vasculite/fisiopatologia , Vasculite Leucocitoclástica Cutânea/fisiopatologiaRESUMO
We describe cases of severe odynophagia, extensive oral ulcerations, and bowel perforation in patients with human immunodeficiency virus infection that were caused by lymphomatoid granulomatosis. Such presentations in human immunodeficiency virus-infected individuals are usually ascribed to other causes and may be incorrectly treated on an empiric basis. In addition, deep tissue specimens obtained at the margin of ulcerative lesions are often necessary for definitive diagnosis. We review our limited treatment experience with zidovudine, interferon alfa, and H2 blockers in our patients. Based on the markedly increased frequency in which lymphomatoid granulomatosis is being diagnosed at our institution in the post-human immunodeficiency virus era, we postulated an association between these two entities.
Assuntos
Síndrome da Imunodeficiência Adquirida/complicações , Doenças do Colo/complicações , Granulomatose Linfomatoide/complicações , Doenças da Boca/complicações , Infecções Oportunistas/complicações , Síndrome da Imunodeficiência Adquirida/tratamento farmacológico , Adulto , Doenças do Colo/patologia , Doenças do Colo/cirurgia , Feminino , Humanos , Interferon Tipo I/administração & dosagem , Cetoconazol/uso terapêutico , Granulomatose Linfomatoide/fisiopatologia , Granulomatose Linfomatoide/terapia , Masculino , Doenças da Boca/fisiopatologia , Doenças da Boca/terapia , Infecções Oportunistas/tratamento farmacológico , Infecções Oportunistas/patologia , Proteínas Recombinantes , Zidovudina/uso terapêuticoAssuntos
Pneumopatias , Vasculite , Crioglobulinemia/imunologia , Humanos , Vasculite por IgA/imunologia , Imunidade Celular , Pneumopatias/classificação , Pneumopatias/etiologia , Pneumopatias/imunologia , Pneumopatias/fisiopatologia , Pneumopatias/terapia , Granulomatose Linfomatoide/fisiopatologia , Poliarterite Nodosa/fisiopatologia , Síndrome , Vasculite/classificação , Vasculite/etiologia , Vasculite/imunologia , Vasculite/fisiopatologia , Vasculite/terapiaRESUMO
A variety of neurological complications may occur in the various connective tissue and "collagen-vascular" diseases. Most of these complications are due to vasculitis affecting various sites in the central or peripheral nervous system. While the evidence for definitive vasculitis in SLE is not strong, small vessel damage usually is present in anatomic sites which correlate well with clinical features. Although patients with rheumatoid arthritis also may have vasculitis, neurological complications are usually related to nerve compression by rheumatoid nodules or the arthritic process itself. Considerable controversy exists regarding the accuracy of various diagnostic tests. While corticosteroids are the mainstay of therapy for these conditions, there are no definitive studies proving their efficacy.