ASCT transplantation for POEMS syndrome – updated
Several conditions, most commonly Castleman’s with about 30% of cases, can be associated with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome, or it can present by itself. High-dose chemotherapy with autologous peripheral blood stem cell transplantation (ASCT) has been reported in case reports and there are no prospective trials and it can cause considerable peritransplant morbidity. In 2005 Dr. Dizspenzieri reported 16 cases with Castleman’s who had transplantation and 11 others from the literature; several more cases were subsequently also published. Most recent review is from International Blood and Marrow Transplant Research (CIBMTR) database, 2022 (Kansagra et al) and it concluded that Patients with POEMS syndrome had superior PFS/OS compared with MM at 5 years, consistent with known data. Zhao in 2019 concluded patients with higher risk may benefit more from ASCT treatment than melphalan + dexamethasone (MDex, N = 79), or lenalidomide + dexamethasone (LDex, N = 103).
High-dose melphalan is an established conditioning regimen and is medically necessary, as is the use of GCSF to assist peripheral stem cell collections.
Kansagra A, Dispenzieri A, Fraser R, Estrada-Merly N, Sidana S, Nishihori T, Hansen DK, Anderson LD Jr, Banerjee R, Bumma N, Dhakal B, Khouri J, Landau H, Lee C, Mian H, Nathan S, Savani B, Kumar S, Qazilbash M, Shah N, D’Souza A. Outcomes after autologous hematopoietic cell transplantation in POEMS syndrome and comparison with multiple myeloma. Blood Adv. 2022 Jul 12; 6(13): 3991-3995
Zhao, H., Huang, Xf., Gao, Xm. et al. What is the best first-line treatment for POEMS syndrome: autologous transplantation, melphalan and dexamethasone, or lenalidomide and dexamethasone?. Leukemia 33, 1023–1029 (2019).
Gordon Cook etal, POEMS syndrome: a retrospective study of the Plasma Cell Disorder sub-committee of the Chronic Malignancy Working Party of the European Society for Blood & Marrow Transplantation. Haematologica Vol. 102 No. 1 (2017): January, 2017