Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated with very-low-dose lenalidomide and dexamethasone. A 57-year-old Japanese man was admitted to our hospital with pleural effusion, massive ascites, and leg edema. The diagnosis of POEMS syndrome was made based on the combination of the following findings: peripheral neuropathy, organomegaly, endocrinopathy, serum monoclonal protein elevation, skin changes, plasma VEGF elevation, and evidence of extravascular volume overload. Renal dysfunction induced by biopsy-proven renal involvement of POEMS syndrome was observed. Massive ascites of the patient dramatically diminished with long-time treatment of very-low-dose lenalidomide and dexamethasone. Lenalidomide seems to be a very promising therapy for POEMS syndrome presenting with extravascular volume overload such as edema, pleural effusion, and ascites. Very-low-dose lenalidomide might be effective especially for the patients with POEMS-related nephropathy. 1. Introduction POEMS syndrome is a monoclonal plasma cell disorder characterized by the presence of peripheral neuropathy and one or more of the following features: osteosclerotic myeloma, Castleman’s disease, increased serum levels of vascular endothelial growth factor (VEGF), organomegaly, endocrinopathy, edema, typical skin changes, and papilledema [1]. Patients often present with evidence of extravascular volume overload, such as edema, pleural effusion, and ascites [2]. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome who presented with massive ascites as the first manifestation, in whom the ascites dramatically diminished with long-term treatment of very-low-dose lenalidomide and dexamethasone. 2. Case Presentation A 57-year-old Japanese man was admitted to our hospital with pleural effusion, massive ascites, and leg edema in March 2010. Physical examination revealed hyperpigmentation, hemangiomas, and gynecomastia. The patient had no neurological symptoms; however, the deep tendon reflexes were absent. Chest radiography revealed cardiomegaly and bilateral pleural effusion. Computed tomography of the abdomen revealed hepatosplenomegaly and massive ascites (Figure 1(a)). Blood examination showed moderate renal dysfunction (serum creatinine 1.81？mg/dL, blood urea nitrogen 44？mg/dL). The diagnosis of