RESUMO
TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis or renal dysfunction and organomegaly) syndrome is a systemic inflammatory disease characterised by thrombocytopenia, anasarca, fever or inflammatory syndrome, reticulin myelofibrosis or renal dysfunction and organomegaly. It was first described as a subtype of idiopathic multicentric Castleman disease. Here, we report the case of a 42-year-old woman presenting with thrombocytopenia, anasarca, inflammatory syndrome, renal insufficiency, reticulin myelofibrosis at bone marrow biopsy and cervical and axillary lymph nodes. Kidney biopsy showed double contours of the glomerular basement membrane, mesangiolysis and endothelial swelling compatible with thrombotic microangiopathy (TMA) as well as with TAFRO syndrome. She was successfully treated by corticosteroids, tocilizumab and rituximab. This new case description of TAFRO syndrome underlines three features of this disease rarely described in the literature and never simultaneously in the same patient: the association to severe hypothyroidism, the presence of TMA-like lesions on kidney biopsy and the treatment by the association of steroids, tocilizumab and rituximab.
Assuntos
Anticorpos Monoclonais Humanizados/administração & dosagem , Hiperplasia do Linfonodo Gigante , Edema , Glucocorticoides/administração & dosagem , Hipotireoidismo , Rim , Rituximab/administração & dosagem , Microangiopatias Trombóticas/patologia , Adulto , Antirreumáticos/administração & dosagem , Biópsia/métodos , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/fisiopatologia , Hiperplasia do Linfonodo Gigante/terapia , Edema/diagnóstico por imagem , Edema/etiologia , Feminino , Humanos , Hipotireoidismo/diagnóstico , Hipotireoidismo/etiologia , Rim/patologia , Rim/fisiopatologia , Tomografia por Emissão de Pósitrons/métodos , Insuficiência Renal/diagnóstico , Insuficiência Renal/etiologia , Microangiopatias Trombóticas/etiologia , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Intravascular large B-cell lymphoma is a rare entity characterized by the proliferation of neoplastic lymphocytes in the lumen of small blood vessels and high mortality. Diagnosis of intravascular lymphoma is often delayed or established postmortem. Here, we report the case of a 48-year-old woman presenting hemophagocytic syndrome, with pituitary gland and neurological involvement. Diagnosis of intravascular large B-cell lymphoma was made on perisplenic vessels, while liver and bone marrow biopsy was noncontributive. This case demonstrates the importance of thorough histopathologic investigations in the setting of high suspicion.