The POEMS syndrome with coexisting endocrinopathy - case report

Abstract

We present a case of a woman with unique multisystem disorder - POEMS syndrome and endocrine abnormalities coexisting with it. The POEMS acronym comprises the dominant features: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein (M protein), skin changes. Association between plasma cell dyscrasia and polyneuropathy, was described in 1956 year by Crow. The main features were coined in the acronym POEMS by Bardwick in 1980 year. The polisymptomatic clinical picture, progressive course and no-concurrent manifestations of main features impede the diagnosis.
In this case, the first symptoms were the sensimotor polyneuropathy, peripheral oedema, osteosclerotic bone lesions, skin changes, organomegaly. They proceded diagnosis by 3 years. The first endocrinopathy was hypothyroidism. Definite diagnosis was delayed because we couldn’t detect the presence of M protein. Immunoelectophoresis didn’t detect it, butanalysis by immunofixation detected M protein in serum and urine. Within 3 years of the first symptoms, she developed hypogonadism hypergonadotropic.
At first, the monotherapy with corticosteroids was used, then - melfalane with prednisone. Due to the progression of the disease, a thalidomide was used in therapy (it is anti-VEGF agent). One of the side effects of the treatment of thalidomide is the progression of polyneuropathy, which was observed in this patient. After finishing this therapy she received chemotherapy.
This case report imposes the necessity of constants observation of patients with POEMS syndrome because there is a possibility of their developing other disorders. In the event of coexistence polyneuropathy and plasma cell dyscrasia, this disease should be taken into consideration. (Pol J Endocrinol 2007; 58 (3): 238-243)