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A Case Report on Poems Syndrome With High Parathyroid Hormone and Repeated Surgery, a Rare Presentation of a Rare Disease Publisher



Yaseri A1 ; Ghannadi S1, 2 ; Perseh L3 ; Tavassol ZH1 ; Hasaniranjbar S1
Authors
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Authors Affiliations
  1. 1. Obesity and Eating Habits Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Sports Medicine Research Center, Neuroscience Institute, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Valiasr Education and Treatment Center, Zanjan University of Medical Sciences [ZUMS], Zanjan, Iran

Source: International Journal of Surgery Case Reports Published:2023


Abstract

Introduction: Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare multisystemic disease that is named for its constellation which can easily be mistaken for other disorders. Case presentation: In present study we evaluated a 39-year-old man with hypercalcemia and parathyroid hormone. He underwent two failed surgeries for parathyroid adenoma but due to the presence of other features of the syndrome, a diagnosis of POEMS syndrome was made. His symptoms were multiple peripheral neuropathies, monoclonal plasma cell proliferative disorder, multiple bony lesions alongside endocrinopathy, lymphadenopathy, extravascular volume overload, mildly hypocellular marrow with polymorphic population, trilineage hematopoiesis, and progressive maturation less than 5 % plasma cell in bone marrow biopsy and aspiration. Patient was treated with the administration of Pomalidomide and autologous stem cell transplantation after which his symptoms improved and laboratory test results normalized. Discussion: POEMS syndrome is a rare disorder that is challenging to diagnose due to its variable clinical presentation and similarity to other disorders. However, specific criteria can help distinguish it from other syndromes. In this case, we witness the comorbidity of Ganglioneuroma. Also, hyperglycemia with hypercalcemia and high PTH in this patient are not common endocrine disorders in POEMS syndrome which there are some possible explanations for these symptoms' presentation. Conclusion: Although endocrine disorders are an important part of POEMs syndrome, some characteristic of this disease is unknown, and it becomes more difficult to diagnose in early stages, that in these cases, thorough physical examinations and laboratory results may help with the early diagnosis. © 2023