Poems Syndrome: An Update

Abstract

POEMS syndrome is a rare, chronic and disabling condition. The causes of this condition remain unknown; however, chronic overproduction of proinflammatory cytokines appears to be a major contributor. Early diagnosis is essential to start treatment before the clinical state of the patient becomes compromised.A complete evaluation of the disease at its onset is critical to the treatment decision. In localized disease, curative doses of radiation (50 Gy) is the recommended therapy. On the other hand, patients with disseminated disease should be given systemic therapy. Treatment-related morbidity can be minimized by an efficient induction therapy that modifies the cytokine status, improving clinical condition and control disease severity before mobilization and transplantation. Patients not suitable for hematopoietic stem cell transplantation (HSCT) are usually treated with alkylator-based therapy. Novel agents may also offer benefits to patients with a poor performance status or renal dysfunction, and induce transplantation eligibility. Given the biological characteristics of POEMS, immunomodulatory effects and the absence of neurotoxicity, lenalidomide appears to be an effective therapy for the treatment of POEMS, both as short induction therapy before PBSCT and in non-transplant eligible patients, as it showed high response rate and durable responses.At present, however, guidelines for the diagnosis and treatment of POEMS are not available and appear advocated.

Neurofascin‐140 and ‐155 antibodies in an atypical case of poems syndrome

“…The patient's demyelinating polyradiculoneuropathy, plasma cell dyscrasia, sclerotic bone lesion, and papilledema, as well as the initial thrombocytosis and polycythemia, skin findings, and signs of mild volume overload (left‐sided pleural effusion) fulfilled the diagnostic criteria of POEMS syndrome, even though the absence of elevated VEGF levels and of monoclonal lambda light‐chain gammopathy and the transient nature of her thrombocytosis were atypical. This case links NF‐140 and ‐155 antibodies with such an atypical presentation of POEMS syndrome.…”

“…POEMS syndrome occurs in the setting of an underlying plasma cell disorder and is named after the associated findings of polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes . It is often mistaken for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) as both entities present as subacute polyradiculoneuropathies with motor predominance.…”

Ischemic Stroke in a Patient With Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Syndrome Treated With Lenalidomide

“…POEMS syndrome is a rare, life-threatening, and disabling plasma cell disorder where the acronym stands for polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes [1]. Though the pathophysiology underlying the disorder remains unknown, it is postulated to be due to various proinflammatory growth factors and cytokines that are thought to stimulate VEGF expression, which likely plays a vital role in the pathogenesis of the disease [2]. Its presentation varies from person to person and, due to its diverse clinical manifestations, especially when presented to a primary care provider, can have a longer lead time for diagnosis ranging from 13-18 months, and could be even misdiagnosed as other disorders such as chronic inflammatory demyelinating polyneuropathy, amyloidosis, and multiple myeloma [2].…”

POEMS syndrome complicated by ischaemic stroke and cerebral vasculitis

“…Furthermore, the association of cerebrovascular events by age 50 in patients with POEMS and high clonal plasma cells counts by bone marrow biopsy supported this diagnosis. The diagnostic criteria of POEMS syndrome are seen in table 2 10–12…”