Abstract
Introduction
POEMS syndrome is a paraneoplastic syndrome associated with a clonal plasma cell neoplasm, in general is a rare disease and its descriptions are based on series and case reports.
Objective
To describe the clinical features and outcomes in a case series of patients with POEMS syndrome.
Material and methods
Patients who met the diagnostic criteria for POEMS syndrome proposed by Dispenzieri were selected. These patients came from the medical consultation of different neurologists specialized in neuromuscular pathology in the period from 2005 to 2021; likewise, a review of the Colombian literature of all published cases of patients with diagnosis of POEMS syndrome was performed. As a result, 16 cases of patients with this diagnosis were collected and reviewed.
Discussion and conclusion
The symptoms, clinical course and treatment of our case series are similar to those published in the global literature. Early diagnosis and active treatment significantly modify the progression of the disease, as well as its overall prognosis. Patients with longer survival and lower disease burden were those who received adjuvant treatment with autologous stem cell transplantation.
Resumen
Introducción
El síndrome POEMS es un trastorno paraneoplásico relacionado con una neoplasia clonal de células plasmáticas. La evidencia disponible sobre esta enfermedad rara proviene de estudios de casos y series de casos.
Objetivo
Nuestro objetivo es describir las características clínicas y los resultados de una serie de casos de síndrome POEMS.
Materiales y método
Seleccionamos pacientes que cumplían con los criterios diagnósticos de síndrome POEMS propuestos por Dispenzieri. Dichos pacientes fueron atendidos en las consultas de neurólogos especializados en patología neuromuscular entre 2005 y 2021. También realizamos una revisión de la literatura que incluyó todos los casos de pacientes con síndrome POEMS diagnosticados en Colombia. La muestra final incluyó 16 pacientes con dicho diagnóstico, cuyos resultados revisamos.
Discusión y conclusión
Los síntomas, el curso clínico y el tratamiento en nuestra serie de casos fueron similares a los descritos en la literatura internacional. El diagnóstico precoz y un tratamiento activo logran modificar significativamente el avance de la enfermedad y el pronóstico de los pacientes. Aquellos pacientes sometidos a trasplante autólogo de células madre como tratamiento complementario mostraron una supervivencia mayor y una menor carga de la enfermedad.
Introduction
POEMS syndrome, identified by its acronyms P: polyneuropathy, O: organomegaly, E: endocrinopathy, M: M protein, S: skin changes (see Fig. 1 ); is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The diagnosis of this entity is often delayed because the syndrome is rare and can be confused with other neurological disorders, most commonly with a chronic inflammatory demyelinating polyradiculoneuropathy.
The first descriptions of this entity date back to 1938 by Scheinker. In 1980, the name POEMS was attached by Bardwick and later the understanding of this entity was expanded especially by the contributions of Dispenzieri. , It has also been called Crow-Fukase syndrome, osteosclerotic myeloma, or Takatsuki syndrome.
POEMS syndrome has a variable demographic distribution, the largest case series being from Japan, the United States, France, China, and India. In Japan, it has a prevalence of 0.3 cases per 100 000 population per year.
There are some important points that relate to the acronym of this syndrome and is that not all features within the acronym are required to make the diagnosis; likewise, there are other important features not included in the POEMS acronym, which include: papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, elevated levels of vascular endothelial growth factor (VEGF), a predisposition to thrombosis, and abnormal pulmonary function tests. ,
Its pathogenesis is not entirely clear, however, in recent years, VEGF has been proposed as central to the pathogenesis of this entity. Plasma cells exert inflammatory mechanisms explain the systemic involvement of this entity, especially mediated by cytokines (e.g., IL-6), procoagulant and inflammatory endothelial states, platelet aggregation, and tissue ischemia.
Regarding the diagnostic criteria proposed by Dispenzieri, these criteria are divided into mandatory, major, and minor criteria. To make the diagnosis of POEMS syndrome at least 1 major and 1 minor criterion is required, always in relation to the mandatory criteria, which are the presence of neuropathy and M protein. The minor criteria are: organomegaly, endocrinopathies, volume overload and extravasation, dermatologic changes, papilledema, thrombocytosis, and polycythemia.
Materials and methods
Patients who met the diagnostic criteria for POEMS syndrome proposed by Dispenzieri in 2003 during the period from 2005 to 2021 in the medical consultation of different neurologists specialized in neuromuscular pathology were selected. Also, a review of all published cases of POEMS syndrome in Colombian literature were included. For the analysis we included demographic features, signs, and symptoms, findings in diagnostic studies, general complications, treatment, and follow-up time to assess survival.
Results
As a result, 16 patients diagnosed with POEMS syndrome were obtained ( ). The criteria proposed by Dispenzieri were applied and their diagnosis was verified. Of the 16 patients selected from 2005 to 2021, the mean age was 49.5 years (range 37–67 years) and the sex ratio was 7 males and 9 females. We found that 100% of all patients had a polyneuropathy documented by electrodiagnosis, at least 75% of patients had a demyelinating pattern, and there were also predominantly axonal polyneuropathies in 3 cases; however, several of the cases reported in the literature do not detail the characteristics of the polyneuropathy ( see Table 1 ).
| Characteristics of patients with POEMS Syndrome |
Number of patients (n): 16 Demographic characteristics
|
Polyneuropathy (16/16) – 100%
|
| Plasmocitoma (4/16) – 25% |
| Castleman disease (5/16) – 31.2% |
Organomegaly (16/16) – 100%
|
Endocrinopathy (16/16) – 100%
|
M protein (16/16) – 100%
|
Skin changes (16/16) – 100%
|
Edema (13/16) – 81.2%
|
Other characteristics
|
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