Clinical Features of POEMS Syndrome In Southeast Asia: A Literature-Based Study

Purpose: To determine and analyze the clinical characteristics of POEMS Syndrome among Southeast Asian countries.Methods: We searched the literature using a pre-specified inclusion and exclusion criteria and using the search terms “[(POEMS) or (Takatsuki) or (PEP) or (Crow Fukase) and (syndrome)] AND [Countries/People of Southeast Asia]”.Results: Seven studies, including 5 case reports, 1 case series and 1 correspondence letter containing 8 patients were eligible for analysis. The median age of onset was 54 years, while the median duration to correct diagnosis was 5.5 months. The most common initial presentation was weakness (4/6) with 50% initially diagnosed as chronic inflammatory demyelinating polyneuropathy. On physical examination, 100% had evidence of length dependent polyneuropathy, 80% had papilledema, 75% had edema/effusion, 86% had skin changes and 67% had organomegaly. All had abnormal NCS and CT scan while 1 tested negative for monoclonal gammopathy restricted to lambda. Only 2 had VEGF results, one of which was normal. Melphalan and steroid combination was the most common treatment given with only 1 case dying of sepsis. Conclusion: Although the number of cases in Southeast Asia is lower, which can be attributed to difference in ethnicity and geographical location, the presenting signs and symptoms of this condition was similar to other countries. However, the new proposed criteria may not be applicable in the region as only few countries are capable of doing VEGF testing.

POEMS syndrome causing left ventricular hypertrophy, myocardial dysfunction and pericardial effusion: a case report

Background POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a paraneoplastic syndrome caused by a plasma cell proliferative disorder. Characteristics of POEMS syndrome include elevated pro-inflammatory and angiogenic cytokine levels that lead to multi-organ dysfunction. Patients have a variety of initial symptoms, but cardiac involvement is not common. Case summary We report a case of a 31-year-old Chinese woman with chief complaints of chest pain and dyspnoea who was diagnosed with POEMS syndrome. The cardiovascular system in the case study patient was characterized by pericardial effusion, enlarged left atria, abnormal myocardial segmental deformation, left ventricular hypertrophy, pulmonary hypertension, and increased glucose metabolism in the left and right ventricular myocardium. The pericardial effusion diminished, while cardiac function, left ventricular wall thickness, and pulmonary hypertension gradually returned to normal with dexamethasone and bortezomib treatment. Discussion This case suggests that cardiovascular system damage may be related to systemic diseases. Cardiovascular system damage caused by POEMS syndrome is recoverable after chemotherapy treatment. Echocardiography readily visualizes the changes in the heart of a patient with POEMS syndrome, clearly reflecting the changes in the structure and function of the heart before and after treatment.

POEMS Syndrome- An Overview

The purpose of this review is to provide the latest information on POEMS syndrome. The authors conducted a literature search of available sources describing the issue of POEMS syndrome with special focus on syndrome and made a comparison and evaluation of relevant findings. The results of this review indicate that POEMS syndrome is associated with a group of disorders known as monoclonal gammopathies or plasma cell dyscrasias. These disorders are characterized the uncontrolled growth of a single clone (monoclonal) of plasma cells, which results in the abnormal accumulation of M-proteins (also known as immunoglobulin) in the blood which has a significant impact on the quality of life of both the patients and his/her family. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. Therefore, early and proper diagnosis and treatment are necessary in order to reduce or even eliminate both symptoms and social burden of the patient.

Not the final diagnosis: from Addison’s disease to POEMS syndrome: a case report and literature review

We report the case of a 47-year-old male patient with pigmentation of the head, face and hands, who was initially diagnosed as having primary adrenal insufficiency (Addison’s disease). Laboratory testing, imaging and physical examination revealed subclinical hypothyroidism, high circulating prolactin and oestradiol concentrations, gynaecomastia, lymphadenopathy, splenomegaly and weakness of both lower limbs. These findings led us to consider whether a single or multiple diseases were present in this patient. Indeed, Addison’s disease can represent one aspect of a wider systemic disease. Therefore, we performed further examinations, and found high serum M protein (5.1%) and vascular endothelial growth factor [1005.30 pg/mL (normal range 0 to 142 pg/mL)] concentrations. As a consequence, we diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome. Consequently, when a single disease cannot fully explain the multiple symptoms and signs of one patient, clinicians should consider the possibility of the presence of a wider syndrome and undertake more detailed diagnostic testing.