Endocrine Abstracts

Introduction: Poems Syndrome is a rare multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder and skin changes, among other features. Literature describes endocrine abnormalities at least in 93% of patients with Poems Syndrome during the diagnosis as well as during the follow-up. The most common deficiencies ranked by their frequency are hypogonadism, hyperprolactinemia, hypothyroidism, abnormal glucose metabolism and in rare cases adrenal insufficiency and elevated IGF-1 levels.

Case Presentation: 67-year-old man with history of right occipital stroke and 1-2 pack a day smoker with pulmonary disease with severe obstruction in spirometry is referred to endocrinology consultations because of erectile dysfunction with decreased libido and asthenia. At physical examination he presented with a group of cervical adenopathies and paresthesia in palms and soles that prevent walking of about one year of evolution. No mucosal hyperpigmentation but presence of hyperpigmented lesions in thorax and abdomen. Normal testicular size and presence of secondary sexual characteristics. No other relevant associated symptoms. Laboratory parameters confirmed by two determinations revealed: Hb 18.1 g/dl (12,0-18,0), Hematocrit 59% (37,0-52,0), HbA1c 5,73% (4,0-6,5); TSH 0,459 mU/l (0,380-5,330); T4L 1,22 ng/dl (0,54-1,24); Prolactine 14,7 ng/ml (2,6-13,1); GH 0,386 ng/ml (1,0-3,0); IGF-1 94,9 ng/ml (67,0-141,0). FSH 2,31 UI/l (1,27-19,26); LH 2,48 UI/l (1,2-8,6); Testosterone 1,42 ng/ml (1,75-7,81); establishing the diagnosis of secondary hypogonadism. Corticoid axis was not valuable due to chronic corticoid treatment for respiratory disease. Pituitary MRI was performed with no findings. The electromyogram showed polyneuropathy in palms and soles with axonal and demyelinating involvement. Hematology completed examination with lymph node biopsy resulting in plasmocellular variant of Castleman disease with low HHV-8 expression and monoclonal spike IGG Lambda (6,8% 0,4 g/dl). Diagnosis of Poems Syndrome was done following the diagnostic criteria:

• Mandatory criteria: monoclonal spike and sensory-motor polyneuropathy.

• Mayor criteria: Castleman disease.

• Minor criteria: secondary hypogonadism, cutaneous hyperpigmented lesions and polyglobulia.

The patient was discharged after clinical stability and improvement of symptoms. He continues with chemotherapy treatment in Hematology outpatient clinics and follow-up by Neurology and Endocrinology. Testosterone treatment was not started due to severe polyglobulia secondary to hematological disease.

Conclusions: Poems Syndrome involves multiple organs, and a multidisciplinary approach should include an endocrinologist. Patients should be systematically assessed for endocrinopathy and treated as indicated. Long-term follow-up is recommended since the appearance of new endocrinopathies as well as the remission of others.