RESUMO
Primary effusion lymphoma (PEL) is a rare and aggressive herpesvirus-8 (HHV-8) driven B cell non-Hodgkin's lymphoma (NHL) that is usually associated with human immunodeficiency virus (HIV) infection, and has a poor prognosis. PEL is comprised of two clinically distinct but pathologically similar variants: classic and extracavitary PEL. Based on retrospective series, treatment options include combined antiretroviral therapy (cART) in conjunction with chemotherapy regimens used in other forms of NHLs. Treatment outcomes with this approach are usually dismal and there is no standard of care. We present a case of a patient with HIV associated CD30+ extracavitary PEL unfit for multi-agent chemotherapy, who achieved a durable complete response with single agent brentuximab-vedotin and cART.
Assuntos
Infecções por HIV , HIV-1 , Infecções por Herpesviridae , Herpesvirus Humano 8 , Imunoconjugados/administração & dosagem , Linfoma de Efusão Primária , Adulto , Antirretrovirais/administração & dosagem , Brentuximab Vedotin , Infecções por HIV/diagnóstico por imagem , Infecções por HIV/tratamento farmacológico , Infecções por HIV/virologia , Infecções por Herpesviridae/diagnóstico por imagem , Infecções por Herpesviridae/tratamento farmacológico , Infecções por Herpesviridae/virologia , Humanos , Linfoma de Efusão Primária/diagnóstico por imagem , Linfoma de Efusão Primária/tratamento farmacológico , Linfoma de Efusão Primária/virologia , MasculinoRESUMO
An unusual case of aplastic anemia presenting in association with lymphoplasmacytic lymphoma and paroxysmal nocturnal hemoglobinuria is discussed. An insult to the hematological stem cell compartment may result in multiple pathological entities, potentially influencing our approach to the treatment of hematological clonal disorders.
