POEMS Syndrome—Clinical Picture and Management. Current Knowledge

Jurczyszyn, Artur; Olszewska-Szopa, Magdalena

doi:10.1016/j.clml.2023.04.008

Cited by 8 publications

(11 citation statements)

References 79 publications

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“…However, the optimal duration of LDex remains controversial. Although prolonged treatment may have a positive effect on achieving more progressionfree survival (PFS), it is necessary to consider the side effects of treatment, such as cytopenia, and the risk of other malignancies (34).…”

Section: Discussionmentioning

confidence: 99%

Case report: POEMS syndrome masquerades as diabetic foot

Zhou,

Xiao,

et al. 2024

Front. Neurol.

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We present the case of a 54-year-old woman with reasonable blood sugar control who presented with a diabetic foot combined with severe peripheral neuropathy and vascular disease. Lower limb muscle weakness, muscle atrophy, skin pigmentation, and emaciation were also observed. Although her muscle strength improved after glucocorticoid treatment, it remained challenging to account for the other symptoms in this particular patient with chronic inflammatory demyelinating polyneuropathy. Plump liver and spleen, hidden bone lesions combined with seemingly unexplained cerebral infarction, and serous effusion led us to suspect polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. The abnormal proliferation of monoclonal plasma cells and a significant increase in vascular endothelial growth factor (VEGF) levels confirmed the diagnosis of POEMS syndrome. After 1 month of treatment with lenalidomide and dexamethasone, the diabetic foot ulcers healed, and the symptoms of myasthenia and fatigue improved. Diabetic feet may represent only the tip of the iceberg of an underlying POEMS syndrome. Our report aimed to increase awareness of this rare yet significant situation, advocating for the prompt identification and treatment of POEMS syndrome.

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Section: Discussionmentioning

confidence: 99%

Case report: POEMS syndrome masquerades as diabetic foot

Zhou,

Xiao,

et al. 2024

Front. Neurol.

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“…Before initiating therapy, it is of utmost importance to confirm whether the patient meets the criteria for plasma cell myeloma. Although the therapeutic options may be similar for both POEMS syndrome and myeloma, treatment decisions should be influenced by the consideration that myeloma therapy may carry a higher potential for toxicity in patients with POEMS syndrome [2,18,19].…”

Section: Discussionmentioning

confidence: 99%

“…Therapeutic approaches differ between limited-and advanced-stage diseases [2,18,19]. In POEMS syndrome, the limited-stage disease describes cases where bone marrow biopsies reveal the absence of clonal plasma cells and only 1-2 lesions are present.…”

Section: Discussionmentioning

confidence: 99%

“…For patients in this stage, treatment typically includes involved-field radiation therapy at specific doses. Notably, a study conducted at the Mayo Clinic demonstrated that patients with limitedstage disease had a remarkable 4-year overall survival rate of 97% and an event-free survival rate of 52% [2,18,19].…”

Section: Discussionmentioning

confidence: 99%

“…Although the clinical response is associated with an enhanced quality of life, the attainment of hematological remission plays a pivotal role in shaping patient outcomes. Notably, those who achieve hematological complete response (CR) experience the most significant progression-free survival (PFS), with an impressive 88% 6-year PFS rate [2,18,19]. Another intriguing area of investigation revolves around CAR T-cell-based therapy for POEMS syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Unraveling the Neurological Complexity of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Syndrome: A Report of a Challenging Case of a Young Woman and Cutting-Edge Advancements in the Field

Furciniti,

Casalino,

Lo Russo

et al. 2023

Diseases

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POEMS syndrome—characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes—is an uncommon and complex paraneoplastic disorder encompassing a diverse array of symptoms. Here we report the challenging case of a 34-year-old female who sought medical attention at the emergency department due to distal lower limb weakness. She was breastfeeding her first child at that time. Her condition rapidly deteriorated, making it difficult for her to perform simple tasks independently. Initially, she struggled with activities like jumping or climbing stairs. Eventually, her ability to walk was also compromised. These symptoms underscored the swift evolution of her polyneuropathy. Nerve conduction studies and electromyography confirmed a diagnosis of mixed demyelinating and axonal polyneuropathy. Subsequent investigations, including bone marrow biopsy and immunochemistry testing, revealed a plasma cell disorder characterized by lambda monoclonal gammopathy, along with elevated levels of vascular endothelial growth factor (VEGF > 8000 pg/mL). This pivotal finding led to the diagnosis of POEMS syndrome, prompting the initiation of antineoplastic therapy (daratumumab-lenalidomide-dexamethasone) to manage this condition. An autologous cell transplantation was planned. The rarity of POEMS syndrome and its diverse clinical manifestations often lead to an incorrect or delayed diagnosis. Our case underscores the importance of considering this syndrome in patients presenting with acute or subacute polyneuropathy, even if the patients are young. In conclusion, this case elucidates the diagnostic complexities of POEMS syndrome, emphasizing the integral role of comprehensive multidisciplinary evaluations and the potential influence of increased VEGF as a diagnostic key element and possible therapeutic target.

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