The POEMS syndrome is a rare plasma cell disorder involving pathological changes of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. The diagnostic and therapeutic management of the disease is clinically challenging. Given its rarity, the clinical information on POEMS is sparse with most evidence being small case studies. Jurczyszyn et al. (2022) summarised and reviewed the clinical features and therapeutic modes of 108 real-world cases of the disorder. For instance, the analysis revealed that skin lesions, thrombocythemia and polycythemia were present less frequently than reported previously. Regarding clinical interventions, proteasome inhibitors were used as the front-line treatment in 37% of POEMS, whereas bortezomib monotherapy was the most effective therapy achieving complete remission/very good partial remissions (CR/VGPR) in 69% of patients. 30% of patients proceeded to planned autologous stem cell transplant (ASCT) as part of the front-line treatment resulting in statistically superior progression-free (PFS) and overall survival (OS) compared to non-ASCT treated patients (P= 0.003). Moreover, the clinical data revealed that anaemia, thrombocytopenia, and age >60 were associated with a negative impact on patient outcomes.

Reference:

Jurczyszyn A, et al. Clin Lymphoma Myeloma Leuk. 2022 May;22(5):297-304.