Endocrinopathy in polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome

Abstract

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome due to underlying plasma cell disorder. It belongs to group of plasma cell dyscrasias such as multiple myeloma, waldenstrom’s macroglobulinemia, castleman disease and monoclonal gammopathy of undetermined significance. The term POEMS syndrome was first coined in 1980 by Bardwick. In the last 10 years clinical criteria to diagnose POEMS syndrome have been elaborated to avoid overlap with other haematological disorders. Due to disease rarity there is very few case series and case reports described in literature. This is a haematological disease which frequently presents with peripheral neuropathy but endocrine dysfunction can be a presenting or an associated feature. We report two cases of POEMS syndrome who presented with endocrinopathy as a predominant feature with literature review of this syndrome

Castleman Disease: A Rare Condition with Endocrine Manifestations

“…This is translated into an increased angiogenesis and proliferation of polyclonal IgM-lambda plasma cells in the follicles of affected nodes [ 2 , 4 , 14 ]. Autoantibodies produced by plasma cells have been suggested as the effectors of the endocrinopathy; nonetheless, no circulating forms against the hormones or its receptors have been found [ 14 , 16 ]. Apparently, VEGF affects the endocrine axes by inducing an imbalance in angiogenic factors that regulate hormonal secretion [ 15 ].…”

“…The roles of both cytokines (IL-6 and VEGF) in CD have explained interchangeably the clinic presentation of these patients. An elevated VEGF level has been considered one of the criteria for POEMS syndrome, and due to the higher prevalence of bone lesions in this group, its levels have been associated with the degree of bone compromise [ 14 , 16 ].…”

“…The minor criteria consist of organomegaly, signs of extravascular volume overload (pleural effusion, ascites, and edema), endocrinopathy, polycythemia, papilledema, and skin changes. Organomegaly stands for lymphadenopathy, hepatomegaly, or splenomegaly [ 16 , 21 ].…”

“…Secondary hypogonadism stands as the most common endocrine dysfunction in 70% of cases. Reports from the Mayo series, only available for males, features erectile dysfunction as the predominant symptom, followed by gynecomastia [ 16 , 21 ]. The second most common endocrine manifestation is hypothyroidism in 58% of the patients, although it has a mild course.…”

“…Parathyroids are rarely assessed, but some cases with secondary hyperparathyroidism have been described. Therefore, it is important to establish if calcium levels are altered due to these glands' dysfunction or secondary to bone turnover [ 16 , 21 , 23 ]. The pituitary compromise is infrequent, with few reports of adenomas resulting in panhypopituitarism and acromegaly [ 21 , 24 - 25 ].…”