Division of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University Hospital, Japan.
Division of Palliative Care, Tohoku Medical and Pharmaceutical University Hospital, Japan.
Intern Med. 2021;60(6):927-933. doi: 10.2169/internalmedicine.5820-20. Epub 2021 Mar 15.
Spontaneous regression is rare in patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN). An 85-year-old man presented with pancytopenia and skin lesions, and the bone marrow exhibited 79.6% CD4+, CD56+, CD123+, and TCL-1+ abnormal cells, with a normal karyotype; he was thus diagnosed with BPDCN. While being followed without chemotherapy, he was admitted due to sepsis induced by Serratia marcescens, which was successfully treated with antibiotics. Notably, his blood cell counts improved, and the skin lesions disappeared. To our knowledge, this is the first reported case of spontaneous regression of BPDCN with a decrease in tumor cells in the bone marrow following sepsis.
原发性骨外弥漫性大 B 细胞淋巴瘤伴浆母细胞分化(BPDCN)极为罕见。一位 85 岁男性因全血细胞减少和皮肤病变就诊,骨髓中存在 79.6% 的 CD4+、CD56+、CD123+和 TCL-1+异常细胞,核型正常;因此被诊断为 BPDCN。该患者未接受化疗,仅进行随访,但因黏质沙雷氏菌引起的败血症入院,经抗生素治疗后成功治愈。值得注意的是,他的血细胞计数有所改善,皮肤病变也消失了。据我们所知,这是首例报道的败血症相关 BPDCN 肿瘤细胞在骨髓中减少并自发性消退的病例。