RESUMO
ABSTRACT: Spinal cord sarcoidosis (SCS) is an uncommon disease with estimated incidence of 0.4% in patients with systemic sarcoidosis. Spinal cord sarcoidosis typically manifests late in the disease course and significantly contributes to patient morbidity. Therefore, early SCS diagnosis and prompt initiation of therapy are crucial. Herein, we report the case of a 51-year-old man with symptomatic SCS managed by 18 F-FDG PET/MRI, allowing diagnosis and treatment efficacy evaluation of spine involvement. We believe that the increasing clinical availability of hybrid PET/MRI devices will offer new opportunities for optimal management of patients with uncommon severe sarcoidosis localizations, such as nervous system.
Assuntos
Fluordesoxiglucose F18 , Sarcoidose , Masculino , Humanos , Pessoa de Meia-Idade , Compostos Radiofarmacêuticos/uso terapêutico , Sarcoidose/diagnóstico por imagem , Imageamento por Ressonância Magnética , Resultado do TratamentoRESUMO
The management of lymphoma in patients with primary immunodeficiency (PID) is challenging because of its poor prognosis and complex therapeutic approaches. We conducted a systematic literature review of case-reports, case-series, and cohorts indexed in MEDLINE reporting the association of lymphoma and PID. One hundred and eighty-two articles were selected out of 787. We identified 386 cases. Median age at diagnosis of PID and lymphoma was 9.5 and 12 years old, respectively. T-cell deficiencies were the main PIDs associated with lymphoma (57%). The most prevalent lymphoma was diffuse large B-cell lymphoma (33.5%). Epstein-Barr Virus-driven lymphomas were mostly observed in innate immunodeficiencies (when reported). Complete response to treatment was observed in 65.8% of the cases. Death occurred in 38.2%. Few allogenic stem cell transplantations were performed (29 cases). Our detailed analysis of the literature provides a landscape of lymphoma's occurrence in PID. Devoted studies in specific sub-groups of patients at risk are needed to develop dedicated protocols.