Society for Endocrinology Annual Meeting 2005
London, UK
07 November 2005 - 09 November 2005
Society for Endocrinology
Poems from the endocrinologist
Russells Hall Hospital, West Midlands, United Kingdom.
A 42 year old lady was referred to the endocrine clinic with a five month history suggestive of hypothyroidism and a high TSH and low T4 levels. Clinical examination revealed increased skin pigmentation, low systemic blood pressure, bilateral axillary lymphadenopathy and hepatosplenomegaly. Short synacthen test confirmed cortisol insuffiency and she was started on thyroxine and hydrocortisone, and went on to have further investigations for the lymphadenopathy and hepatosplenomegaly. Liver biopsy showed non specific inflammatory changes, and trephine bone marrow biopsy showed findings suggestive of an underlying myeloproliferative process and no features of lymphoma or leukaemia and a normal bone marrow cytogenetics. Despite initial improvement in symptoms, the patient deteriorated and developed symptoms suggestive of mild peripheral motor and sensory neuropathy and sclerodermatous skin changes. Free IgA-lambda light chains were detected in the urine specimen and an osteosclerotic focus was noted in the left pelvis. These features were diagnostic of POEMS syndrome. She had an autologous haematopoietic cell transplantation and chemotherapy. She is currently fit and well and looking forward to going back to her job as a nurse.
Discussion: POEMS Syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal-gammopathy and Skin changes) is a rare condition with a prevalence of nearly 1in 2.7 million and considered to be an osteosclerotic variant of myeloma. It is interesting to note that nearly 80% of men and 70% of women with POEMS syndrome have endocrine problems. Also in our case, neuropathy, which is considered to be a dominant feature of the syndrome developed late after initial presentation. Further enquiry revealed that she was diagnosed with Benign intracranial hypertension. This association is previously undescribed although papilloedema has been described as part of POEMS syndrome. This case illustrates how a multisystem disorder may present in an endocrinology clinic.
Endocrine Abstracts (2005) 10 P22