SAT-205 POEMS: A Medical Odyssey

Abstract

Introduction: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) syndrome is a rare disorder with poorly understood pathogenesis. The incidence of endocrinopathy associated with POEMS syndrome has been recognized more frequently in the last two decades, ranging between 67-84%. The cause of endocrine dysfunction associated with the syndrome is not known, but has been described to include hypogonadism, hypothyroidism, abnormalities of glucose metabolism, hyperprolactinemia, gynecomastia in men, hyperestrogenemia, calcium abnormalities and adrenal insufficiency (AI).Case: A 38 y/o Hispanic male was initially referred to nephrology with complaint of leg swelling. Subsequently, the patient was referred to endocrinology for abnormal thyroid function studies and ongoing fatigue. Patient had prior medical history of recently diagnosed hypothyroidism treated with levothyroxine 100 mcg daily. The most bothersome complaint was pitting edema in the lower extremities with associated pain. He also endorsed right shoulder pain exacerbated by use of the right arm. He reported unintentional weight loss of about 30 pounds in the past 2-3 years, denying night sweats. He did endorse feeling fatigued, skin darkening, and erectile dysfunction. On exam patient was afebrile, BP 120/71 mm/Hg, HR 87 bpm, no goiter, no gynecomastia, skin hyperpigmentation, darkening creases of the palms, pitting edema in lower extremities. Work up showed abnormal lambda monoclonal bands in plasma and monoclonal lambda free light chain in urine. Bone survey showed lytic lesion in right scapula. [prolactin 15.6 ng/ml (normal (NL)= 0-25), free testosterone 23.1 pg/ml (NL= 35.0-155.0), total testosterone 205 ng/dl (NL= 250-1100), LH 8.2 MIU/ml (NL= 1.2-8.6), FSH 4.3 MIU/ml (NL=1.3-19.3), TSH 17.8 uIU/ml (NL= 0.45-5.33), free T4 0.62 ng/dl (NL= 0.58-1.64), free T3 3.05 pg/ml (NL=2.5-3.9), TPO antibody (AB) negative, IGF-1 41 ng/ml (NL=53-331), AM cortisol 8.2 ug/dl (NL=6.7-22.6), ACTH 99 pg/ml (NL=6-50), aldosterone 3 ng/dl (NL ≤28), renin activity 2.78 ng/ml/h (NL=0.25-5.82), 21-hydroxylase AB negative, 17-hydroxyprogesterone 54 ng/dl (NL=42-196), DHEA-S 88 mcg/dl (NL=106-464)]. ACTH stimulation test (250 mcg) was performed with basal cortisol 8.0 ug/dl (NL= 5.0- 21.0), basal ACTH 93 pg/ml (NL=6-50), 30min cortisol 9.7 ug/dl (NL=13.0-30.0), 60min cortisol 10.2 ug/dl (NL=14.0-36.0). Pt was diagnosed with primary AI and started on hydrocortisone. CT abdomen and pelvis with contrast showed normal adrenal glands.Discussion: Physicians should be cognizant of a unifying diagnosis in a syndrome of such a broad presentation. Endocrinopathies vary in extent of dysfunction and may fluctuate during the course of POEMS syndrome. Multidisciplinary management as well as regular reassessment of endocrine function is integral in managing this complex disease.