Dados do Trabalho

Título

POEMS Syndrome, although rare, presents a constellation of clinical findings that allows its early diagnosis - A Case Report

RESUMO

Clinical case: A 55-year-old male patient presented acrocyanosis with intense pain in the hands and paresthesia in boot-glove pattern associated with a significant weight loss, asthenia and hyporexia, in 3 years of evolution. On clinical examination, in addition to acrocyanosis, he had telangiectasias on the upper body and face, bilateral gynecomastia, palpable hepatomegaly, global hyporeflexia, 4+ muscle strength in the extremities, reduced pain sensitivity in the lower limbs, reduced vibration in the upper and lower limbs. Complementary exams showed a polycythemia and monoclonal peak between beta 2 and gamma globulin fractions, a chronic inflammatory demyelinating pattern polyneuropathy and osteosclerotic and lytic areas in the body of the sternum and osteosclerotic lesions in the skull. A bone marrow aspirate was performed, which revealed 9% of plasma cells and in the sternal biopsy bone marrow hypocellularity with discrete polyclonal reactive plasmacytosis. The set of such findings allowed the final diagnosis of POEMS Syndrome, an Osteosclerotic Multiple Myeloma. Treatment was based on cycles of dexamethasone, disodium pamidronate, amitriptyline and gabapentin for neuropathic pain management, followed by a VTD protocol (Bortezomib+Doxorubicin+Thalidomide). Discussion: This report aims to expose a rare case, with an estimated prevalence of 0.3 per 100,000 people, which had an evolution of more than 3 years, and with a constellation of clinical presentations that may have made early diagnosis difficulties. POEMS Syndrome should be analyzed as a differential diagnosis of several diseases, such as polyneuropathies, osteosclerotic lesions, plasma cell dyscrasias, neoplasms, infections and vasculitis. Final Comments: The case in question portrays that the identification of the disease can be a challenge, but a good anamnesis and physical examination followed by appropriate tests can distinguish this Syndrome from its differential diagnoses.

Palavras Chave

POEMS Syndrome, Paraneoplastic Polyneuropathy, Case Reports