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Review
. 2022 Nov 27;11(23):7011.
doi: 10.3390/jcm11237011.

Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

Maroun Bou Zerdan  1   2 , Tracy I George  3 , Silvia Tse Bunting  4 , Chakra P Chaulagain  1
Affiliations
Review

Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

Maroun Bou Zerdan et al. J Clin Med. .

Abstract

POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. Even though the pathophysiology is poorly understood, recent studies suggest that both clonal and polyclonal plasmacytosis leading to the production of pro-inflammatory cytokines and angiogenic mediators play the central role. These mediators including vascular endothelial growth factor (VEGF) are the driving forces of the syndrome. The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. Due to the presence of neuropathy, it can be confused with chronic inflammatory demyelinating polyradiculopathy (CIDP), and if thrombocytosis and splenomegaly are present, it can be confused with myeloproliferative neoplasms. Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. Immediate treatment targeting the underlying plasma cell proliferation results in a dramatic response in most patients. The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. For patients with localized bony disease, radiotherapy has proven to be very effective. Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.

Keywords: POEMS; pathogenesis; supportive care; treatment.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Bone marrow morphologic features in POEMS syndrome of a 64-year-old-man presenting with peripheral polyneuropathy and IgG lambda monoclonal gammopathy. (A) Normocellular bone marrow core biopsy for age (Hematoxylin-eosin stain, original magnification ×20). No lymphoid aggregates were observed. (B) Bone marrow aspirate smear highlighting a slightly enlarged plasma cell (center) in a background of mixed hematopoiesis. (Wright Giemsa stain, original magnification ×100). (C) Plasma cells are slightly increased on the core biopsy estimated at 5% (CD138 immunohistochemical stain, original magnification ×20), which show a perivascular distribution with scattered small clusters. (D) Original magnification ×40.
Figure 2
Figure 2
Treatment strategies used in different stages of the disease.
See this image and copyright information in PMC

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