Multiparameter Flow Cytometry for the Identification of Neoplastic Plasma Cells in POEMS Syndrome with IgG-kappa Gammopathy: Successful Treatment Using Lenalidomide and Dexamethasone

• Published in: Internal Medicine 2019/12/01, Vol.58(23), pp.3461-3468
• Main Authors: Ikeda, Sho, Kobayashi, Takahiro, Saito, Masaya, Komatsuda, Atsushi, Ubukawa, Kumi, Kameoka, Yoshihiro, Takahashi, Naoto
• Format: Article
• Language: English
• Subjects: Aged; Biopsy; Bone marrow; Case Report; CD19 antigen; CD27 antigen; CD38 antigen; CD56 antigen; Dexamethasone; Dexamethasone - administration & dosage; Edema; Flow cytometry; Flow Cytometry - methods; Gammopathy; Glomerulonephritis; Glomerulonephritis - etiology; Hemangioma; Hemangioma - etiology; Humans; Hyperpigmentation; IgG kappa monoclonal protein; Immunoglobulin G; Immunoglobulin G - metabolism; Immunoglobulin kappa-Chains - metabolism; Immunologic Factors - administration & dosage; Internal medicine; lenalidomide; Lenalidomide - administration & dosage; Male; membranoproliferative glomerulonephritis (MPGN); multiparameter flow cytometry; Nerve conduction; Paraproteinemias - drug therapy; Pigmentation Disorders - etiology; Plasma cells; Plasma Cells - pathology; Pleural effusion; POEMS syndrome; POEMS Syndrome - diagnosis; POEMS Syndrome - drug therapy; Proliferative kidney disease; Renal failure; Renal function; Treatment Outcome; Vascular endothelial growth factor; Vascular Endothelial Growth Factor A - metabolism; Vascular Endothelial Growth Factors
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.2882-19

A 72-year-old man presented with a 6-month history of systemic edema. Hyperpigmentation, hemangioma, pleural effusion, IgG-kappa-type monoclonal protein, high vascular endothelial growth factor values, renal failure, and nerve conduction study abnormalities were also present. Multiparameter flow cytometry (MFC) showed 0.2% neoplastic plasma cells (CD38-, CD56-, and kappa-positive; CD19-, CD27-, and lambda-negative) in the bone marrow leading to POEMS syndrome. Cases involving kappa-type POEMS syndrome are extremely rare. A kidney biopsy revealed membranous proliferative glomerulonephritis-like changes in our case. Lenalidomide-dexamethasone therapy improved the renal function. Detection of neoplastic plasma cells by MFC was useful for the accurate diagnosis and treatment evaluation.