Introduction: POEMS syndrome is a rare multisystemic disorder including polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder and skin changes. We aim to describe the course of the endocrine disease in the context of this paraneoplastic syndrome.
Methods: 69 patients with POEMS have been under the MDT care in our hospital. Data is currently available for 32 patients who attended the joint haematological/neurological/endocrine clinic from 06/1999 to 01/2017. All patients had regular endocrine screening including pituitary, thyroid and calcium metabolism. Median and interquartile range and mean±S.D. were used for quantitative variables and percentage for qualitative variables.
Results: Seventy-two patients were male. The age at diagnosis was 49.4(38.259.8) years and the median follow-up was 4.6(27.4) years. At diagnosis, all patients presented with polyneuropathy, 25% had hyperhidrosis and 50% endocrinopathy. From those with endocrinopathy, 36.7% had thyroid disease, 26.7% hypogonadism, 3.3% type 2 diabetes mellitus (2DM) and 6.5% Addison disease. None of them had parathyroid disorders. 60% had more than one endocrinopathy at diagnosis. During surveillance 90.3% of patients developed endocrinopathy. 68.8% of patients had hyperprolactinemia with a fold increase 1.8±0.99 above the upper limit of the normal. Hyperprolactinaemia was transient in 72.7%. One patient with hyperprolactinaemia had an empty sella on MRI. 59.4% had hypothyroidism (37.5% clinical, 21.9% subclinical). This was transient in 27.8% of patients. Addison disease was diagnosed in 18.8% of patients and none of them recovered their adrenal function. 15.6% presented with type 2DM. 78.6% had hypogonadism (19.1% secondary, 38.1% primary, 42.9% subclinical primary). Gonadal function recovered in 3 patients.
Conclusion: Endocrinopathy in POEMS was described in 90% of our cohort. The MDT team should include an endocrinologist. Patients should be systematically assessed for endocrinopathy. The most common deficiencies were hypogonadism, hyperprolactinemia and hypothyroidism. Normalisation of the endocrinopathy was common and should be considered when treating these patients.
20 May 2017 - 23 May 2017