ABSTRACT
A linfoistiocitose hematogocítica (LH) constitui uma síndrome clínico-patológica associada à febre pancitopenia severa, disfunçäo hepática e distúrbio da coagulaçäo, com hematofagocitose no nível da medula óssea e órgäos linfóides. Ocorre em pacientes imunossuprimidos, sendo induzida por infecçöes bacterianas, viróticas, fúngicas e parasitárias, bem como neoplasias, especialmente os linfomas de células T. Este trabalho relata o primeiro caso do nosso conhecimento de LH em transplante renal no Brasil, discutindo-se a etiologia, fisiopatogenia, manifestaçoös clínicas e tratamento da doença.
Subject(s)
Humans , Female , Adult , Kidney Transplantation , Histiocytosis, Non-Langerhans-Cell/virology , Histiocytosis, Non-Langerhans-Cell/pathologyABSTRACT
Familial hemophagocytic lymphohistiocytosis (FHL) is a rapidly fatal illness, usually encountered in infancy, characterized by fever, hepatosplenomegaly, pancytopenia, and central nervous system involvement. Microscopic examination of tissue shows a non-malignant lymphohistiocytic infiltrate, with prominent erythrophagocytosis. FHL is an autosomal recessive hereditary disorder but may develop secondarily to other conditions such as immunosuppression, malignancies, fat overload and certain infections. We recently experienced a case of siblings developing FHL, which may be associated with EBV infection.