RESUMO
A doença de Castleman (DC) é uma rara e benigna desordem linfoproliferativa descrita por Castleman como "hiperplasia hialinizante linfoide". Sua etiologia e patogênese é desconhecida, porém parece haver hiperestimulação antigênica induzida por vírus de forma crônica e mediada por interleucina 6, com alguns casos ligados ao herpes vírus 8 e vírus da imunodeficiência humana (AU)
Castleman's disease (CD) is a rare and benign lymphoproliferative disorder described by Castleman as "hyelinizing lymphoid hyperplasia". Its etiology and pathogenesis are unknown, but there appears to be antigen hyperstimulation chronically induced by virus and mediated by interleukin 6, with some cases related to herpes virus 8 and human immunodeficiency virus (AU)
Assuntos
Humanos , Feminino , Criança , Adolescente , Pessoa de Meia-Idade , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/classificação , Hiperplasia do Linfonodo Gigante/patologia , Hiperplasia do Linfonodo Gigante/terapiaRESUMO
BACKGROUND: Castleman's disease is an atypical lymphoproliferative disorder which may be compatible with paraneoplastic manifestations of POEMS syndrome. CLINICAL CASE: a 53 year old man with a history of type 2 diabetes, hypothyroidism and Addison's disease presented with numbness and weakness in limbs, dyspnea, skin hardening, Raynaud's phenomenon, weight loss and fatigue. A physical exam showed tachypnea, generalized cutaneous hyperpigmentation and skin hardening of extremities, muscle weakness, hypoesthesia and hyporeflexia. Laboratory showed hyperprolactinemia, low testosterone, hypothyroidism and Addison's disease. Electrophoresis of proteins showed polyclonal hypergammaglobulinemia. Somatosensory evoked potentials reported peripheral neuropathy and severe axonal polyneuropathy by electromyography. Chest X-rays showed bilateral reticular infiltrates and mediastinal widening. An echocardiogram displayed moderate pulmonary hypertension. Skin biopsy had no evidence of scleroderma. CT reported axillar, mediastinal and retroperitoneal nodes. The mediastinal lesion biopsy reported hyaline vascular Castleman's disease, multicentric variety. He was treated with rituximab. CONCLUSIONS: the case meet criteria for multicentric hyaline vascular Castleman's disease, POEMS variant, treated with rituximab.
Introducción: la enfermedad de Castleman es un trastorno linfoproliferativo atípico en el que pueden existir manifestaciones compatibles con síndrome POEMS. Caso clínico: hombre de 53 años de edad con antecedente de diabetes mellitus tipo 2, hipotiroidismo y enfermedad de Addison. Se iniciaron parestesias y debilidad en las extremidades y, posteriormente, disnea, endurecimiento cutáneo, fenómeno de Raynaud y pérdida de peso. Se identificó taquipnea, hiperpigmentación cutánea generalizada y extremidades con endurecimiento cutáneo, debilidad muscular, hipoestesia e hiporreflexia; así como hiperprolactinemia, testosterona baja, hipotiroidismo y enfermedad de Addison; los anticuerpos antinucleares y antiScl-70 fueron negativos. Los potenciales evocados somatosensoriales indicaron neuropatía periférica y la electromiografía, olineuropatía axonal severa. Radiografía torácica: infiltrado reticular bilateral y ensanchamiento mediastinal. Electrocardiograma: hipertensión arterial pulmonar moderada. Tomografía toracoabdominal: ganglios axilares, mediastinales y retroperitoneales. Con la biopsia se identificó enfermedad de Castleman multicéntrica hialina vascular. El paciente recibió rituximab. Conclusiones: si bien la experiencia con el rituximab aún es limitada, en el caso descrito se observó buena respuesta.
Assuntos
Hiperplasia do Linfonodo Gigante/diagnóstico , Síndrome POEMS/diagnóstico , Hiperplasia do Linfonodo Gigante/classificação , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome POEMS/classificaçãoRESUMO
A doença de Castleman é um distúrbio linfoproliferativo atípico, de etiologia desconhecida, que pode estar associada a uma série de condições clínicas, inclusive doenças de caráter autoimune e neoplasias malignas. No presente relato, uma paciente de 72 anos foi encaminhada ao serviço de cirurgia torácica do Hospital Universitário Getúlio Vargas, localizado na cidade de Manaus (AM) para a ressecção de um tumor de mediastino posterior. Três meses antes, havia sido internada em UTI com um quadro de dispneia intensa, ocasião na qual foi diagnosticada miastenia gravis. Após a ressecção da massa mediastinal, a análise histopatológica revelou doença de Castleman hialino-vascular complicada por sarcoma de células dendríticas foliculares. Até o momento da redação deste estudo, a paciente utilizava um anticolinesterásico e corticoides para o controle da miastenia gravis.
Castleman's disease is an atypical lymphoproliferative disorder of unknown etiology, which might be associated with various clinical conditions, including autoimmune diseases and malignant neoplasms. We report the case of a 72-year-old female patient who was referred to the thoracic surgery department of Getúlio Vargas University Hospital, in the city of Manaus, Brazil, for the resection of a posterior mediastinal tumor. Three months prior, the patient had been admitted to the ICU with signs of severe dyspnea, at which time she was diagnosed with myasthenia gravis. After the resection of the mediastinal tumor, the histopathological examination revealed hyaline vascular-type Castleman's disease, complicated by follicular dendritic cell sarcoma. At this writing, the patient was being treated with an anticholinesterase agent and corticosteroids for the control of myasthenia gravis.
Assuntos
Idoso , Feminino , Humanos , Sarcoma de Células Dendríticas Foliculares/complicações , Hiperplasia do Linfonodo Gigante/complicações , Miastenia Gravis/diagnóstico , Diagnóstico Diferencial , Sarcoma de Células Dendríticas Foliculares/patologia , Hiperplasia do Linfonodo Gigante/classificaçãoRESUMO
Castleman's disease is an atypical lymphoproliferative disorder of unknown etiology, which might be associated with various clinical conditions, including autoimmune diseases and malignant neoplasms. We report the case of a 72-year-old female patient who was referred to the thoracic surgery department of Getúlio Vargas University Hospital, in the city of Manaus, Brazil, for the resection of a posterior mediastinal tumor. Three months prior, the patient had been admitted to the ICU with signs of severe dyspnea, at which time she was diagnosed with myasthenia gravis. After the resection of the mediastinal tumor, the histopathological examination revealed hyaline vascular-type Castleman's disease, complicated by follicular dendritic cell sarcoma. At this writing, the patient was being treated with an anticholinesterase agent and corticosteroids for the control of myasthenia gravis.