POEMS syndrome: A multisystem clonal disorder

Ali, Tayyaba; Qazilbash, Muzaffar H.

doi:10.1111/ejh.13514

Cited by 14 publications

(18 citation statements)

References 41 publications

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“…Neuropathy -specifically a peripheral, ascending, symmetric neuropathy affecting sensory and motor function -is frequently the primary presenting complaint in POEMS syndrome [6].…”

Section: Discussionmentioning

confidence: 99%

Leave It to the Prose: A Case of POEMS Syndrome

Samant¹,

Reguerro²

2021

Cureus

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POEMS syndrome is a rare paraneoplastic syndrome named for its constellation of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. As a proliferative plasma cell disorder, it has a multisystem presentation and chronic cytokine overproduction. It often presents in the fifth or sixth decade of life, mostly in males. Presentations vary widely, making it extremely difficult to diagnose. A chronic progressive distal, sensorimotor polyneuropathy in conjunction with a monoclonal plasma cell dyscrasia suggests POEMS.A sixty-one-year-old man with a significant past medical history of bilateral lower extremity weakness and a cerebral meningioma presented with anorexia, intermittent dysphagia, and early satiety with intermittent vomiting and diarrhea over the past three months with a 20-pound weight loss. A CT of the abdomen and pelvis was obtained due to vomiting, showing expansile lesions of the left ischium, acetabulum, and ilium, with small-volume ascites and splenomegaly. Because of these findings and the noted bilateral lower extremity neuropathic weakness, we obtained further testing to corroborate suspicions of a plasma cell disorder. Labs were significant for IgG lambda with a monoclonal spike (M spike), low cortisol, low testosterone, and elevated thyroid-stimulating hormone (TSH). Protein electrophoresis was positive for biclonal lambda, and his vascular endothelial growth factor (VEGF) levels were elevated to 377. Bone marrow core biopsy indicated malignancy. Given his polyneuropathy, organomegaly, endocrinopathy, IgG lambda, skin changes, and extravascular fluid overload, POEMS syndrome was diagnosed. He was initiated on lenalidomide and dexamethasone and eventually received a stem cell autologous bone marrow transplant.Given his non-specific presentation, and the confounding factor of a known meningioma causing hypopituitarism, diagnosing POEMS required extensive workup. A contrast CT demonstrating bone lesions associated with myeloma-type disease was crucial, pairing his endocrinopathy and neuropathy with a plasma cell dyscrasia. Although POEMS is exceedingly rare, accurate diagnosis is vital, as treatment requires a multidisciplinary approach. While high-dose chemotherapy-conditioned autologous stem cell transplantation is the gold-standard treatment for POEMS syndrome, patients who are diagnosed in a late stage of the disease are not candidates, underlining the need for early identification of the disorder.

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“…Neuropathy -specifically a peripheral, ascending, symmetric neuropathy affecting sensory and motor function -is frequently the primary presenting complaint in POEMS syndrome [6].…”

Section: Discussionmentioning

confidence: 99%

Leave It to the Prose: A Case of POEMS Syndrome

Samant¹,

Reguerro²

2021

Cureus

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“…According to the International Myeloma Working Group criteria, the diagnosis of POEMS syndrome requires the presence of both mandatory criteria (polyneuropathy and monoclonal plasma cell proliferative disorder), plus at least one major criterion (sclerotic bone lesions, CD and elevated VEGF), and at least one minor criteria (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema and thrombocytosis or polycythemia) [2,3,6].…”

Section: Discussionmentioning

confidence: 99%

“…Castleman disease (CD) is a rare [1,2] and heterogeneous lym-phoproliferative disorder [1][2][3][4], whose pathophysiology is not fully understood [1]. CD is classified clinically into unicentric or multicentric form (MCD) [1].…”

Section: Introductionmentioning

confidence: 99%

“…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]. 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].…”

Section: Introductionmentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

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Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Associated With Castleman Disease: A Common Misdiagnosis

et al. 2021

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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.

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