Recommended articles on POEMS

Here's my personal list of the best articles on various topics in POEMS. Of course,looking at the reviews is always a good idea, since they synthesize data from many different sources. But in some cases a single article stands out, for me at least.

If you're interested in getting an overview of what we know about POEMS, the two review articles (by Dispenzieri and Li) are a good place to start.

Harry

last revised 2 December 2017

INDEX

reviews of POEMS
reports of (relatively!) large series of cases (all retrospective)
treatment options: general recommendations; melphalan-dexamethasone; radiation; autologous stem cell transplant; lenalidomide (Revlimid); thalidomide
prognosis
symptoms: endocrine symptoms; pulmonary effects; skin symptoms; eye symptoms
Castleman's disease and POEMS
diagnosis: questions about diagnostic criteria; bone marrow biopsies; VEGF; imaging skeletal lesions; CIDP
other: mutations; which lambda light chains are involved?

reviews of POEMS

Dispenzieri, Angela. 'POEMS syndrome: 2017 update on diagnosis, risk stratification, and management'. American journal of hematology 92 (2017) 814-829. doi: 10.1002/ajh.24802. (abstract)

Li J, Zhou DB. 'New advances in the diagnosis and treatment of POEMS syndrome'. British journal of hematology 161 #3 (May 2013) 303-315. doi: 10.1111/bjh.12236. (abstract)

reports of (relatively!) large series of cases (all retrospective)

Takatsuki K, Sanada I. 'Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases.' Japanese journal of clinical oncology 1983: 13: 543-555. (abstract)

Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y, Satoyoshi E, Tsubaki T, Igata A, Ozaki Y. 'The Crow-Fukase syndrome: a study of 102 cases in Japan.' Neurology 1984 Jun;34(6):712-20. (abstract)

Soubrier MJ, Dubost JJ, Sauvezie BJ. 'POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS Syndrome.' American journal of medicine 97 # 6 (Dec 1994) 543-53. (abstract)

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. 'POEMS syndrome: definitions and long-term outcome.' Blood 101 # 7 (1 April 2003) 2496-2506. (abstract)

Kourelis TV, Buadi FK, Kumar SK, Gertz MA, Lacy MQ, Dingli D, Go RS, Kapoor P, Lust JA, Hayman SR, Hwa Y, Rajkumar SV, Zeldenrust SR, Russell SJ, Lin Y, Leung N, Kyle RA, Gonsalves WI, Dispenzieri A. 'Long-term outcome of patients with POEMS syndrome: An update of the Mayo Clinic experience'. American journal of hematology 2016 Jun;91(6):585-9. doi: 10.1002/ajh.24356. (abstract)

[This supplements but does not completely replace the previous study]

Li J, Zhou DB, Huang Z, Jiao L, Duan MH, Zhang W, Zhao YQ, Shen T. 'Clinical characteristics and long-term outcome of patients with POEMS syndrome in China.' Annals of hematology 2011 Jul;90(7):819-26. doi: 10.1007/s00277-010-1149-0. (abstract)

Kulkarni GB, Mahadevan A, Taly AB, Yasha TC, Seshagiri KS, Nalini A, Satishchandra P, Veerendrakumar M, Shankar SK. 'Clinicopathological profile of polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome.' Journal of clinical neuroscience 2011 Mar;18(3):356-60. doi: 10.1016/j.jocn.2010.07.124. (abstract)

treatment options

general recommendations

Dispenzieri A. 'How I treat POEMS syndrome'. Blood 199 #24 (14 June 2012) 5650-5658. doi: 10.1182/blood-2012-03-378992. (abstract)

[add the proteasome inhibitor Kyprolis (carfilzomib) to the list; it received FDA approval in July 2012]
[also, Mayo is currently conducting a trial using ixazomib citrate (Ninlaro), lenalidomide (Revlimid), and dexamethasone; contact them for more information]
[Li's 2013 review argues for a more aggressive approach to treating solitary lesions; see Humeniuk 2013 (listed below) for evidence about the efficacy of irradiation]

Kuwabara S, Dispenzieri A, Arimura K, Misawa S, Nakaseko C. 'Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome.' Cochrane database of systematic reviews 2012 Jun 13;6:CD006828. doi: 10.1002/14651858.CD006828.pub3. (abstract)

melphalan-dexamethasone

Li J, Zhou DB. 'Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome'. Blood 117 #24 (16 June 2011) 6445-6449. doi: 10.1182/blood-2010-12-328112. (abstract)

radiation

Humeniuk MS, Gertz MA, Lacy MQ, Kyle RA, Witzig TE, Kumar SK, Kapoor P, Lust JA, Hayman SR, Buadi FK, Rajkumar SV, Zeldenrust SR, Russell SJ, Dingli D, Lin Y, Leung N, Dispenzieri A. 'Outcomes of patients with POEMS syndrome treated initially with radiation.' Blood 122 (4 July 2013) 68-73. doi: 10.1182/blood-2013-03-487025. (abstract)

autologous stem cell transplant

Dispenzieri A, Lacy MQ, Hayman SR, Kumar SK, Buadi F, Dingli D, Litzow MR, Gastineau DA, Inwards DJ, Elliott MA, Micallef IN, Ansell SM, Hogan WJ, Porrata LF, Johnston PA, Afessa B, Bryce A, Kyle RA, Gertz MA. 'Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome'. European journal of hematology 80 (2008) 397-406. doi: 10.1111/j.1600-0609.2008.01037.x. (abstract)

[I strongly recommend that you read this before going through an auto, and make sure that your physicians have read it too]

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. 'Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience.' Blood 2012 Jul 5;120(1):56-62. doi: 10.1182/blood-2012-04-423178. (abstract)

Karam C, Klein CJ, Dispenzieri A, Dyck PJ, Mandrekar J, D'Souza A, Mauermann ML. 'Polyneuropathy improvement following autologous stem cell transplantation for POEMS syndrome'. Neurology 84#19 (2015) 1981-1987. doi: 10.1212/WNL.0000000000001565. (abstract)

Chandrashekaran S, Dispenzieri A, Cha SS, Kennedy CC. 'Pulmonary morbidity improves after autologous stem cell transplantation in POEMS syndrome'. Respiratory Medicine 2015 Jan;109(1):122-30. doi: 10.1016/j.rmed.2014.11.005. (abstract)

[D'Souza 2012, Chandrashekaran 2014, and Karam 2015 discuss mostly the same patients; Karam focuses on neurological effects, and Chandreshekaran on pulmonary effects]

lenalidomide

Nozza A, Terenghi F, Gallia F, Adami F, Briani C, Merlini G, Giordano L, Santoro A, Nobile-Orazio E. 'Lenalidomide and dexamethasone in patients with POEMS syndrome: results of a prospective, open-label trial'. British journal of haematology 2017 Dec;179(5):748-755. doi: 10.1111/bjh.14966. (abstract)

thalidomide

Misawa S, Sato Y, Katayama K, Nagashima K, Aoyagi R, Sekiguchi Y, Sobue G, Koike H, Yabe I, Sasaki H, Watanabe O, Takashima H, Nishizawa M, Kawachi I, Kusunoki S, Mitsui Y, Kikuchi S, Nakashima I, Ikeda S, Kohara N, Kanda T, Kira J, Hanaoka H, Kuwabara S; Japanese POEMS Syndrome for Thalidomide (J-POST) Trial Study Group. 'Safety and efficacy of thalidomide in patients with POEMS syndrome: a multicentre, randomised, double-blind, placebo-controlled trial'. Lancet Neurology 2016 Oct;15(11):1129-37. doi: 10.1016/S1474-4422(16)30157-0. (abstract)

Katayama K, Misawa S, Sato Y, Sobue G, Yabe I, Watanabe O, Nishizawa M, Kusunoki S, Kikuchi S, Nakashima I, Ikeda S, Kohara N, Kanda T, Kira J, Hanaoka H, Kuwabara S. 'Japanese POEMS syndrome with Thalidomide (J-POST) Trial: study protocol for a phase II/III multicentre, randomised, double-blind, placebo-controlled trial'. BMJ Open 2015 Jan 8;5(1):e007330. doi: 10.1136/bmjopen-2014-007330. (abstract)

prognosis

Kourelis TV, Buadi FK, Gertz MA, Lacy MQ, Kumar SK, Kapoor P, Go RS, Lust JA, Hayman SR, Rajkumar V, Zeldenrust SR, Russell SJ, Dingli D, Lin Y, Leung N, Hwa YL, Gonsalves W, Kyle RA, Dispenzieri A. 'Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first line treatment'. Leukemia 2016 May;30(5):1079-85. doi: 10.1038/leu.2015.344. (abstract)

C Wang, X-F Huang, Q-Q Cai, X-X Cao, M-H Duan, H Cai, D-B Zhou and J Li. 'Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome'. Leukemia 31 (2017) 100-106; advance online publication, June 24, 2016. doi:10.1038/leu.2016.168 (abstract)

Symptoms

endocrine

Gandhi GY, Basu R, Dispenzieri A, Basu A, Montori VM, Brennan MD. 'Endocrinopathy in POEMS syndrome: the Mayo Clinic experience'. Mayo Clinic proceedings 82 #7 (July 2007) 836-842. (abstract)

Yang H, Huang X, Cai Q, Wang C, Cao X, Zhou D, Li J. 'Improvement of sexual function in POEMS syndrome after combination therapy of Lenalidomide and dexamethasone'. Orphanet J Rare Dis. 2016 Jun 18;11(1):80. doi: 10.1186/s13023-016-0461-8. (abstract)

pulmonary effects

Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A. 'Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients'. Chest 133 #4 (April 2008) 969-974. doi: 10.1378/chest.07-1800. (abstract)

Li J, Tian Z, Zheng HY, Zhang W, Duan MH, Liu YT, Cao XX, Zhou DB. 'Pulmonary hypertension in POEMS syndrome.' Haematologica 98 (Mar 2013) 393-398. doi: 10.3324/haematol.2012.073031. (abstract)

skin symptoms

Miest RY, Comfere NI, Dispenzieri A, Lohse CM, el-Azhary RA. 'Cutaneous manifestations in patients with POEMS syndrome.' International journal of dermatology 52 (Nov 2013) 1349-56. doi: 10.1111/j.1365-4632.2012.05648.x. (abstract)

eye symptoms

Kaushik M, Pulido JS, Abreu R, Amselem L, Dispenzieri A. 'Ocular findings in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome.' Ophthalmology 2011 Apr;118(4):778-82. doi: 10.1016/j.ophtha.2010.08.013. (abstract)

Castleman's disease and POEMS

Dispenzieri A, Armitage JO, Loe MJ, Geyer SM, Alfred H, Camoriano JK, Menke DM, Weisenburger DD, Ristow K, Dogan A, Habermann TN. 'The clinical spectrum of Castleman's disease'. American journal of hematology 87 (2012) 997-1002. doi: 10.1002/ajh.23291. (abstract)

Fajgenbaum DC, van Rhee F, Nabel CS. 'HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy'. Blood 123 (2014) 2924-2933. doi: 10.1182/blood-2013-12-545087. (abstract)

Diagnosis

questions about diagnostic criteria

Misawa S, Kuwabara S. 'Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (Crow-Fukase) syndrome: diagnostic criteria and treatment perspectives'. Clinical & experimental neuroimmunology 4 (2013) 318-325

bone marrow biopsies

Dao LN, Hanson CA, Dispenzieri A, Morice WG, Kurtin PJ, Hoyer JD. 'Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients'. Blood 117 #24 (16 June 2011) 6438-6444. doi: 10.1182/blood-2010-11-316935. (abstract)

VEGF

D'Souza A, Hayman SR, Buadi F, Maeurmann M, Lacy MQ, Gertz MA, Kyle RA, Kumar S, Greipp PR, Lust JA, Russell SJ, Zeldenrust S, Dingli D, Witzig TE, Rajkumar SV, Dispenzieri A. 'The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome'. Blood 118 #17 (27 Oct 2011) 4663-4665. doi: 10.1182/blood-2011-06-362392. (abstract)

Wang C, Zhou YL, Cai H, Cheng XQ, Zhang W, Kang WY, Qin XZ, Duan MH, Han HJ, Cao XX, Zhefou D, Li J. 'Markedly elevated serum total N-terminal propeptide of type I collagen is a novel marker for the diagnosis and follow-up of patients with POEMS syndrome'. Haematologica 2014 Jun;99(6):e78-80. doi: 10.3324/haematol.2013.102962. (abstract)

imaging skeletal lesions

Glazebrook K, Bonilla F L G, Johnson A, Leng S, Dispenzieri A. 'Computed tomography assessment of bone lesions in patients with POEMS syndrome'. European radiology 2015 Feb;25(2):497-504. doi: 10.1007/s00330-014-3428-y. (abstract)

POEMS and CIDP

Mauermann ML, Sorenson EJ, Dispenzieri A, Mandrekar J, Suarez GA, Dyck PJ, Dyck PJ. 'Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP'. Journal of neurology, neurosurgery & psychiatry 2012 May;83(5):480-6. doi: 10.1136/jnnp-2011-301472. (abstract)

Nasu S, Misawa S, Sekiguchi Y, Shibuya K, Kanai K, Fujimaki Y, Ohmori S, Mitsuma S, Koga S, Kuwabara S. 'Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy'. Journal of neurology, neurosurgery & psychiatry 2012 May;83(5):476-9. doi: 10.1136/jnnp-2011-301706. (abstract)

Naddaf E, Dispenzieri A, Mandrekar J, Mauermann ML. 'Thrombocytosis distinguishes POEMS syndrome from CIDP'. Muscle & Nerve. 52 #4 (October 2015) 658-659. doi: 10.1002/mus.24768. (abstract)

Other

mutations

Bryce AH, Ketterling RP, Gertz MA, Lacy M, Knudson RA, Kumar S, Kyle RA, Fonseca R, Greipp PR, Lust JA, Rajkumar VS, Hayman SR, Buadi F, Russell SJ, Zeldenrust SR, Dispenzieri A. 'A novel report of cig-FISH and cytogenetics in POEMS syndrome.' American journal of hematology 2008 Nov;83(11):840-1. doi: 10.1002/ajh.21285. (abstract)

Kang WY, Shen KN, Duan MH, Zhang W, Cao XX, Zhou DB, Li J. '14q32 translocations and 13q14 deletions are common cytogenetic abnormalities in POEMS syndrome.' European journal of haematology 2013 Dec;91(6):490-6. doi: 10.1111/ejh.12189. (abstract)

Rose C, Zandecki M, Copin MC, Gosset P, Labalette M, Hatron PY, Jauberteau MO, Devulder B, Bauters F, Facon T. 'POEMS syndrome: report on six patients with unusual clinical signs, elevated levels of cytokines, macrophage involvement and chromosomal aberrations of bone marrow plasma cells.' Leukemia 1997 Aug;11(8):1318-23. (abstract)

which lambda light chains?

Li J, Huang Z, Duan MH, Zhang W, Chen M, Cao XX, Guan HZ, Tian Z, Zhou DB. 'Characterization of immunoglobulin lambda light chain variable region (IGLV) gene and its relationship with clinical features in patients with POEMS syndrome.' Ann Hematol 2012 Aug;91(8):1251-5. doi: 10.1007/s00277-012-1455-9. (abstract)