SUN-544 A Not-So-Poetic Case of Abdominal Distension

Introduction: POEMS syndrome is a rare disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. The cause of POEMS syndrome is unknown, although chronic overproduction of proinflammatory and other cytokines is thought to be a contributing factor. Diagnosis requires thorough evaluation as well as high clinical suspicion. Case Presentation: A 44 year old man presents to emergency department due to abdominal swelling and dyspnea for one week. He does endorse diffuse joint pain, fatigue and peripheral neuropathy. He denies constipation, diarrhea, fever and chills. His vital signs on presentation are significant for hypotension with BP of 94/63 mmHg and tachycardia at 108 bpm, otherwise unremarkable. His physical exam is significant for a chronically ill appearing male, with a distended abdomen, non-tender to palpation, with decreased bowel sounds throughout, and splenomegaly but no fluid wave. He has bilateral pitting edema to the ankles. His presenting labs are significant for WBCs 12.7 10^3/uL Hemoglobin 12.3 gm/dL, hematocrit 36.3%, platelets 816 10^3/uL, Na 139, K 4.1, Co2 of 21, Cr. 1.19, glucose 74 mg/dl. CT of the abdomen and pelvis shows large volume ascites, hepatosplenomegaly, lymphadenopathy and an osteosclerotic lesion of T10. Paracentesis is consistent with portal hypertension with no evidence of infection. Echocardiogram is consistent with pulmonary hypertension. Patient has continued multidisciplinary evaluation for his ascites, thrombocytosis, weight loss, lymphadenopathy, organomegaly, and osteosclerotic lesion. Vascular endothelial growth factor is elevated at 264 pg/mL. Kappa and lamba light chains are both elevated at 99.5 mg/L and 92.6 mg/L, respectively. Bone marrow biopsy demonstrates increased plasma cells that rim lymphoid aggregates with lambda predominance suggestive of POEMS syndrome. TSH is found to be 12.33 ulU/mL (0.5-5.0), FT4 0.52 ng/dL (0.6-1.15), free T3 of 1.1 (2.4-4.2). Cosyntropin stimulation test reveals a baseline cortisol of 7.2 mg/dl and inadequate peak cortisol of 15.1 mcg/dL. Patient is started on steroids followed by thyroid replacement therapy. Further evaluation of endocrinopathy reveals LH 14.3 miu/ml (2-12.0), FSH 20.2 miu/ml (1.5-15), testosterone 376 ng/dl (300-890), SHBG 29 nmol/l (11-80), free testosterone 74 pg/ml (47-244), prolactin 14 ng/ml (1-18). Brain MRI is normal. He is then started on chemotherapy followed by bone marrow transplant. Discussion: Endocrinopathies are central feature of POEMS. Degree resolution of endocrinopathies following successful therapy of the disease is unknown. Patients on hormonal replacement therapy who receive systemic treatment of the plasma cell disorder, should receive ongoing evaluation of endocrine abnormalities to prevent drug overdoses.