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Published on: September 19, 2019
[POEMS syndrome. Report of three cases and literature review]
Crhistian Alejandro Aguilar-Vázquez1, Nallely Denisse Ruvalcaba-Sánchez2
1Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Especialidades "Dr. Bernardo Sepúlveda", Servicio de Neurofisiología. Ciudad de México, México.
Background:
POEMS syndrome is a debilitating paraneoplastic disorder associated with plasma cell neoplasia. Clinically, it is characterized by predominantly demyelinating peripheral neuropathy that often starts in the pelvic limbs, organomegaly, endocrinopathy, skin changes, and elevated serum M protein. Diagnosis requires meeting a set of clinical criteria in which polyradiculoneuropathy and plasma cell disorder are the two mandatory criteria. The aim of this article is to describe 3 clinical cases, their diagnostic approach, and therapeutic management.
Clinical Cases:
In this study, three clinical cases with features compatible with POEMS syndrome are presented. Nerve conduction studies revealed demyelinating involvement with sensory-motor impairment, and evidence of a monoclonal spike was found. Treatment was successful in two cases and partially effective in one, although the latter was associated with delayed diagnosis.
Conclusion:
Despite significant advancements in understanding the pathophysiology and treatment of this disease, diagnosis remains challenging, especially in locations lacking the necessary resources for accurate diagnosis. It is essential to emphasize that it requires a multidisciplinary approach. The prognosis of this disease is closely linked to early diagnosis. Here, we describe three cases from a reference center in Mexico to increase awareness of the disease's diagnosis.
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