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POEMS syndrome is a rare multisystem disease associated with an underlying plasma cell disorder. Its name is an acronym for peripheral neuropathy (P), endocrinopathy (E), organomegaly (O), monoclonal plasma cell proliferative disorder (M), and skin changes (S). This case report describes a patient with POEMS syndrome who presented with progressive...
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POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome is rare, with polyneuropathy and monoclonal plasma cell disorder generally considered as essential diagnostic symptoms. We report two cases of POEMS syndrome without monoclonal protein expression. The first case was a 72-year-old man who had experienced...
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... POEMS syndrome is a rare paraneoplastic syndrome [3,5,6] that is described by the acronym in its name: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes [2][3][4][5][6][7][8]. It was first described in 1938 when Scheinker reported a case of a 39-year-old man with sensorimotor peripheral neuropathy, solitary plasmacytoma and cutaneous hyperpigmentation [2,4,9]. ...
... It was first described in 1938 when Scheinker reported a case of a 39-year-old man with sensorimotor peripheral neuropathy, solitary plasmacytoma and cutaneous hyperpigmentation [2,4,9]. The acronym was coined only in 1980 by Bardwick et al, and refers several but not all features of the POEMS syndrome [2,3,6,7,9]. Due to its rarity and wide clinical features, misdiagnosis as another disease is common [7,10], and with significant morbidity and mortality. ...
... The acronym was coined only in 1980 by Bardwick et al, and refers several but not all features of the POEMS syndrome [2,3,6,7,9]. Due to its rarity and wide clinical features, misdiagnosis as another disease is common [7,10], and with significant morbidity and mortality. ...
Castleman disease is a rare lymphoproliferative disorder. Co-presentation with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS syndrome) has been documented in 11-30% of Castleman disease cases. POEMS syndrome is a rare paraneoplastic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. Not all features are required to make the diagnosis. We report a case of a woman who presented with a 1-year history of a left-side supraclavicular swelling associated with constitutional symptoms and symmetrical paresthesia of the lower limbs. In addition, she had skin hyperpigmentation, multiple supra and infra-diaphragmatic lymphadenopathies, hepatosplenomegaly and osteosclerotic lesions. Serum immunofixation was positive for immunoglobulin G-kappa gammopathy. A lymph node excisional biopsy was compatible with Castleman disease. The diagnosis of POEMS syndrome associated with Castleman disease was made. Our patient started treatment with a combination of bortezomib, cyclophosphamide, and dexamethasone with clinical and analytical improvement. Current treatment of POEMS syndrome associated with Castleman disease is focused on the management of POEMS syndrome. Early diagnosis requires a high index of suspicion and is crucial to reduce morbidity and mortality. This case report aims to raise awareness about this rare entity.
Rationale:
This article presents the case of a patient with recurrent chronic diarrhea and cachexia who was misdiagnosed, followed by a literature review to summarize the reasons for misdiagnosis of POEMS syndrome and the treatment strategies.
Patient concerns:
The diagnosis and treatment of this patient suggest that with the improvement of M-protein detection levels, the diagnosis of patients with low M-protein levels, such as those with POEMS syndrome, has been greatly aided.
Diagnoses:
POEMS syndrome requires polyneuropathy and monoclonal plasma cell proliferation as mandatory diagnostic criteria. Therefore, patients presenting with polyneuropathy should routinely undergo M-protein testing and consider the possibility of POEMS syndrome.
Interventions:
The patient, in this case, was treated primarily with relatively conservative immunomodulatory agents.
Outcomes:
During follow-up after treatment, the patient's diarrhea and malnutrition showed significant improvement.
Lessons subsections:
POEMS syndrome has low clinical specificity and a high rate of misdiagnosis. However, once a definitive diagnosis is made, the treatment outcome is favorable.
The clinical case of a 30-year-old female patient with supraclavicular and ipsilateral axillary lymphadenopathy who underwent excisional biopsy with subsequent study is presented. Histopathological analysis of the resected tissue sample revealed a series of distinctive features associated with hyaline vascular variant Castleman disease. The presentation of this case not only provides detailed information about the clinical evolution of the patient, but also serves as a basis to illustrate Key aspects of histopathological is and immunohistochemical implications in Castleman disease. In addition to making a review of the topic regarding this rare pathology in which case reports are essential to increase the understanding of its clinical variability and its diagnostic approach, illustrating the challenges in differential diagnosis and how they should be addressed. Key words: CASTLEMAN, LYMPHOMA, LYMPHADENOPATHY, HYALINE VASCULAR, PLASMA CELLS
El síndrome de POEMS es una variante poco frecuente de discrasia de células plasmáticas; la forma de presentación de esta enfermedad es variable, sin embargo, la polineuropatía es uno de los síntomas clave perteneciente a este síndrome. La neuropatía periférica se caracteriza por ser de inicio distal, simétrico y progresivo, durante un período de semanas a meses, que se deriva en debilidad muscular severa hasta limitación de la marcha. Presentamos a un paciente con polineuropatía sensitivo-motora de larga evolución, agregándose hipotiroidismo, lesiones en piel, banda monoclonal, organomegalia y lesiones osteoescleróticas en cuerpo vertebral, donde fue confirmado la infiltración de células plasmáticas otorgando el diagnóstico de Síndrome de POEMS.
Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes (POEMS) syndrome is a rare disorder with multiple presentations and a constellation of symptoms. We present a 62 year-old female who presented to the Emergency Department for acute dyspnea. Chest Xray showed sclerotic lesions in the ribs and thoracic spine. Further imaging studies with computed tomography (CT) and positron emission tomography (PET) scans were suggestive of a benign process. Improvement was seen with supportive management. A few months later, patient developed neurological symptoms with reduced exercise tolerance. Mixed demyelinating and axonal polyneuropathy was diagnosed by electromyography. Further work up with bone marrow biopsy and immunochemistry testing revealed lambda and kappa plasma cell disorder, with elevated vascular endothelial growth factor (VEGF). Patient was diagnosed with POEMS and initiated on chemotherapy. POEMS syndrome is commonly missed due to its rarity and varied clinical presentations. VEGF plays a crucial role in the diagnosis. Management requires a multidisciplinary approach.
