Swan D, Murphy M, Elhassadi E
Haematology Department, University Hospital Waterford, Regional Cancer Center South East, University College Cork, Cork, Ireland.
Pathology Department, University Hospital Waterford, Regional Cancer Center South East, University College Cork, Cork, Ireland.
Case Rep Hematol. 2017;2017:8152610. doi: 10.1155/2017/8152610. Epub 2017 Sep 13.
We describe an unusual case of a biclonal nodal plasma cell dyscrasia, presenting with a vasculitic rash, end-organ damage, and cytopenias. Serum protein electrophoresis demonstrated a biclonal kappa-restricted paraprotein, with a negative skeletal survey and no bone marrow disease. Fluorodeoxyglucose-PET-CT (FDG-PET-CT) revealed nodal involvement, which was not appreciable clinically, and facilitated biopsy, confirming the diagnosis of a nodal plasmacytoma. Complete biochemical response and resolution of the vasculitic rash were achieved with bortezomib-based therapy.
我们描述了一例罕见的双克隆性淋巴结浆细胞发育异常病例,表现为血管炎性皮疹、终末器官损害和血细胞减少。血清蛋白电泳显示双克隆性κ轻链限制的副蛋白,骨骼检查阴性且无骨髓疾病。氟脱氧葡萄糖-PET-CT(FDG-PET-CT)显示有淋巴结受累,临床上未发现,有助于活检,确诊为淋巴结浆细胞瘤。基于硼替佐米的治疗实现了完全生化缓解和血管炎性皮疹消退。