A 52 year old male presented with insidious onset of abdominal distention for two months prior to presentation. His past medical history was significant for hypothyroidism and sensory motor polyneuropathy diagnosed five years ago. Physical examination revealed a cachectic male with hyperpigmentation, sclerodermoid skin changes, significant ascites and mild pedal edema. Laboratory studies revealed normal liver function tests with albumin of 4.3 mg/dl and international normalized ratio (INR) of 1.1. Hepatitis and HIV serologies were non-reactive. CA-125 and CEA levels were within normal limits. Ascitic fluid analysis revealed a serum ascites albumin gradient of 0.9 gm/dl and ascitic fluid protein of 3.2 gm/dl. Chest x-ray showed a normal sized heart with mild right pleural effusion. Transthoracic echocardiogram showed normal right and left ventricular function with ejection fraction of 62%. Computerized tomography scan of the abdomen showed normal liver, massive amount of ascites, splenomegaly and retroperitoneal lymphadenopathy. Serum protein electrophoresis showed an M spike and serum Immunofixation was consistent with IgA lambda monoclonal gammopathy. Serum VEGF and IL-6 levels were elevated. Based on the overall clinical picture and investigations, the patient was diagnosed with POEMS syndrome. The patient was started on melphalan and dexamethasone and showed significant clinical improvement in the follow up period.
POEMS syndrome is a rare paraneoplastic syndrome which is caused by an underlying plasma cell disorder.The acronym represents several of the defining features which includes Polyradiculoneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder and Skin changes. Patients also have extravascular fluid overload which most commonly manifests as peripheral edema, but pleural effusion and ascites are also common. Patients have a low serum-ascites albumin gradient in the ascitic fluid, consistent with an exudative process rather than a portal hypertension process. There is no standard treatment for POEMS syndrome and no randomized controlled trials of treatment exist in the available literature. Systemic chemotherapy with the use of melphalan plus dexamethasone has demonstrated good response rates with an acceptable toxicity profile. Ascites often responds to the administration of diuretics, but may require drainage with paracentesis. Median survival in patients of POEMS syndrome with extravascular fluid overload was determined to be 6.6 years.
This case throws light into a rare paraneoplastic syndrome associated with plasma cell disorders. The mechanism by which plasma cells cause POEMS syndrome is thought to involve elevations in vascular endothelial growth factor, interleukin 6, tumor necrosis factor-α and Interleukin 1β. Early diagnosis and a prompt multidisciplinary approach increase the likelihood of reduced long-term morbidity and mortality.
To cite this abstract:Panginikkod S, Gopalakrishnan V, Parikh MP. Can a Verse (Poem) Get Any Worse!. Abstract published at Hospital Medicine 2016, March 6-9, San Diego, Calif. Abstract 722. Journal of Hospital Medicine. 2016; 11 (suppl 1). https://www.shmabstracts.com/abstract/can-a-verse-poem-get-any-worse/. Accessed April 4, 2020.