POEMS syndrome medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]
Overview
Medical Therapy
- Patients with isolated bone lesion without bone marrow clonal plasma cells involvement can be treated with radiotherapy.[1]
- Patients with a disseminated disease (more bone lesions and/or bone marrow plasmacytosis) are the candidates for systemic therapy.[1]
Radiotherapy
- More than 50% of patients treated with radiotherapy show a significant improvement of neuropathy.[2]
- Maximal response to radiotherapy, i.e., improvement of neuropathy, can not be attained until 2–3 years since the first evidence of the effect of therapy.[3][4]
- Improvement of anasarca, papilledema, pulmonary hypertension and skin changes may occur within months after the end of radiotherapy.[3][4]
- Radiotherapy can be considered as primary therapy if the bone lesion is large despite an infiltrated bone marrow.
Systemic Therapy
Corticosteroids
- Corticosteroids can attenuate the symptoms for a short time without affecting the progression of the syndrome.[5]
Autologous Stem Cell Transplantation (ASCT)
- It is considered the preferred initial therapy in young patients.[6]
- It is associated with a durable response even if patients experience a relapse of the disease.[6]
- A retrospective study of the Plasma Cell Disorder sub-committee of the Chronic Malignancy Working Party of the European Society for Blood & Marrow Transplantation showed the 1-year non-relapse mortality to be 3.3%.[7]
References
- ↑ 1.0 1.1 Dispenzieri A (June 2012). "How I treat POEMS syndrome". Blood. 119 (24): 5650–8. doi:10.1182/blood-2012-03-378992. PMC 3425020. PMID 22547581.
- ↑ Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, Greipp PR, Witzig TE, Basu R, Suarez GA, Fonseca R, Lust JA, Gertz MA (April 2003). "POEMS syndrome: definitions and long-term outcome". Blood. 101 (7): 2496–506. doi:10.1182/blood-2002-07-2299. PMID 12456500.
- ↑ 3.0 3.1 Brewis MJ, Church AC, Peacock AJ, Thomson S, Tighe J, Johnson MK (December 2014). "Pulmonary hypertension in POEMS syndrome: resolution following radiotherapy". Pulm Circ. 4 (4): 732–5. doi:10.1086/678553. PMC 4278633. PMID 25610609.
- ↑ 4.0 4.1 Dispenzieri A (February 2014). "POEMS syndrome: 2014 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 89 (2): 214–23. doi:10.1002/ajh.23644. PMID 24532337.
- ↑ Dispenzieri A (October 2015). "POEMS syndrome: update on diagnosis, risk-stratification, and management". Am. J. Hematol. 90 (10): 951–62. doi:10.1002/ajh.24171. PMID 26331353.
- ↑ 6.0 6.1 D'Souza A, Lacy M, Gertz M, Kumar S, Buadi F, Hayman S, Dingli D, Zeldenrust S, Kyle R, Ansell S, Inwards D, Johnston P, Micallef I, Porrata L, Litzow M, Gastineau D, Hogan W, Dispenzieri A (July 2012). "Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience". Blood. 120 (1): 56–62. doi:10.1182/blood-2012-04-423178. PMID 22611150.
- ↑ Cook G, Iacobelli S, van Biezen A, Ziagkos D, LeBlond V, Abraham J, McQuaker G, Schoenland S, Rambaldi A, Halaburda K, Rovira M, Sica S, Byrne J, Sanz RG, Nagler A, van de Donk NW, Sinisalo M, Cook M, Kröger N, De Witte T, Morris C, Garderet L (January 2017). "High-dose therapy and autologous stem cell transplantation in patients with 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. 102 (1): 160–167. doi:10.3324/haematol.2016.148460. PMC 5210246. PMID 27634201.