POEMS: A Rare Cause of Low SAAG Refractory Ascites

Abstract

Purpose: Clinical Case: 67yo WF with no known liver disease presented with refractory ascites, LE edema, and acute renal failure. Six months before admission she developed peripheral neuropathy, ascites, right-sided pleural effusion and shortness of breath. Diuretic therapy led quickly to renal dysfunction requiring diuretic discontinuation and a requirement for serial therapeutic paracenteses. She underwent pleurodesis for her pleural effusion. A right heart catheterization revealed mild pulmonary hypertension but normal cardiac function. PMHx: hypothyroidism. SocHx: no alcohol. FHx: no liver disease. PE: cachectic-appearing, no jaundice or scleral icterus, no stigmata of chronic liver disease. Abd: distended, moderate ascites, NT, no palpable hepatosplenomegaly. Ext: 1+ edema to knees bilaterally. Neuro: significantly decreased lower extremity vibratory and pinprick sensation. Labs: WBC 3,500/mL, HCT 31%, Plt 224,000/mL, alb 2.6 g/dL; liver function tests were normal. Diagnostic paracentesis performed on admission revealed a SAAG of 0.4. CEA, CA 19-9, CA-125 and ascitic cytology, AFB stain, and amylase were all normal. CT of chest/abd/pelvis done to screen for malignancy and/or carcinomatosis showed hepatosplenomegaly and sclerotic bone lesions of sternum, clavicle, and iliac wings. EGD and colonoscopy were nondiagnostic, including small bowel biopsies for celiac disease and rectal biopsies for amyloid. An SPEP and IFE showed a monoclonal M spike of 0.5g/dL with lambda light chain present. Nerve conduction studies confirmed a severe diffuse demyelinating sensorimotor polyneuropathy in the lower extremities. Further testing showed low levels of FSH and LH. Severe symptomatic bradydysrhythmias with syncope in the hospital required pacemaker implantation. Given her polyneuropathy, hepatosplenomegaly, endocrinopathy, monoclonal gammapathy and sclerotic bone changes, concern for POEMS syndrome was raised. A bone marrow biopsy was consistent with multiple myeloma. She was discharged home with plans to initiate therapy for multiple myeloma. Discussion: POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. It is associated with polyneuropathy, organomegaly, endocrinopathy of various forms, monoclonal gammopathy, and skin changes. The acronym POEMS captures several dominant features of the syndrome but other important features may include elevated levels of VEGF, sclerotic bone lesions, ascites, pulmonary hypertension, and cardiac conduction disturbances. Treatment is directed at the underlying plasma cell dyscrasia. In this case, ascites was one of the main presenting features. Therefore, POEMS syndrome needs to be included in the differential diagnosis of low SAAG ascites.