de Goes Vitor Abreu, Cortez Anita Cassoli, Morbeck Diogo Lago, D'Almeida Costa Felipe, da Silveira Talita Bueno
Albert Einstein Israeli Faculty of Health Sciences, São Paulo, SP, Brazil.
A.C.Camargo Cancer Center, São Paulo, SP, Brazil.
Hematol Transfus Cell Ther. 2024 Dec;46 Suppl 6(Suppl 6):S316-S321. doi: 10.1016/j.htct.2024.04.119. Epub 2024 Jul 23.
Primary effusion lymphoma (PEL) is an aggressive and rare type of diffuse large B-cell lymphoma (DLBL) that commonly presents itself as pleural, pericardial or peritoneal effusion without lymph node or extranodal involvement in immunosuppressed patients, such as HIV-positive or transplanted receptors. On rare occasions, it may be found in solid sites without effusion, in an immunophenotypically and morphologically similar neoplasm well-known as extracavitary PEL (EC-PEL). Both PEL and EC-PEL are associated with extremely poor prognosis. Due to the rarity of these entities, ther e are no gold standard treatments . Here we discuss the role of autologous bone marrow transplant (auto-BMT) in the treatment of these patients as well as report the case of a young HIV-positive male diagnosed with both PEL and EC-PEL, who underwent a salvage therapy with auto-BMT and achieved complete and sustained remission eight years after the diagnosis.
原发性渗出性淋巴瘤（PEL）是一种侵袭性且罕见的弥漫性大B细胞淋巴瘤（DLBL），通常表现为胸腔、心包或腹腔积液，在免疫抑制患者（如HIV阳性或移植受体）中无淋巴结或结外受累。极少数情况下，它可在无积液的实体部位发现，是一种免疫表型和形态学相似的肿瘤，即腔外PEL（EC-PEL）。PEL和EC-PEL均与极差的预后相关。由于这些实体罕见，尚无金标准治疗方法。在此，我们讨论自体骨髓移植（auto-BMT）在这些患者治疗中的作用，并报告一例年轻的HIV阳性男性病例，该患者被诊断为同时患有PEL和EC-PEL，接受了auto-BMT挽救治疗，并在诊断后八年实现了完全且持续的缓解。
