Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Correspondence
  • Published:

Poems syndrome: consolidation with autologous stem cell transplantation

Bone Marrow Transplantation volume 56, pages 2597–2599 (2021)Cite this article

Subjects

Access through your institution
Buy or subscribe

POEMS syndrome is a rare multisystem paraneoplastic disorder due to plasma cell dyscrasia. The acronym of POEMS refers to polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Other important manifestations not included in acronym are—sclerotic bone lesions and increased VEGF (vascular endothelial growth factor) levels, extravascular fluid accumulation, papilledema, thrombocytosis, erythrocytosis, plasmacytomas, clubbing, abnormal pulmonary functions, multicentric Castleman’s disease [1, 2].

The optimal treatment includes eliminating the plasma cell clone. Radiation therapy helps in localised disease while for those with disseminated state induction therapy on the lines of multiple myeloma is used [2]. Hogan et al. [3] first reported use of autologous stem cell transplantation (ASCT) for this disease. Subsequently, 223 cases have been reported in three large series from Europe (multicentric, n = 127) [4], Mayo Clinic (n = 60) [5] and Japan (n = 36) [6]. In addition, small series [7, 8] and case reports have also confirmed feasibility of ASCT. We report here our experience of 19 patients with sustained response at a long term follow up. Patients base line characteristics are shown in Table 1. Eligibility criteria included: diagnosis of POEMS syndrome as per IMWG (International Myeloma Working Group) criteria [9]. All patients had received at least four cycles of induction therapy using novel agents (n = 18) or cytotoxic chemotherapy (n = 1). Hematologic response was assessed on day +100. The modified Rankin score [10] (mRS: supplementary table 1) was used for neurological evaluation (at base line, post induction, and after transplant). Stata 13.0 software was used for data analysis. Overall survival (OS) was measured from date of diagnosis until death or last follow-up (30th September, 2020). Progression free survival (PFS) was calculated from date of transplant to the time of progression or death. The median follow up is 54 months, ranging from 14 to 109 months.

Table 1 Patients characteristics and transplant outcome.
Full size table

This is a preview of subscription content, access via your institution

Relevant articles

Open Access articles citing this article.

Access options

Access through your institution

Buy this article

  • Purchase on SpringerLink
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

References

  1. Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, et al. POEMS syndrome: definitions and long-term outcome. Blood. 2003;101:2496–506.

    Article  CAS  Google Scholar 

  2. Dispenzieri A. POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management. Am J Hematol. 2019;94:812–27. https://doi.org/10.1002/ajh.25495. Epub 2019 May 23.

    Article  PubMed  Google Scholar 

  3. Hogan WJ, Lacy MQ, Wiseman GA, Fealey RD, Dispenzieri A, Gertz MA. Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation. Bone Marrow Transpl. 2001;28:305–9.

    Article  CAS  Google Scholar 

  4. Cook G, Iacobelli S, van Biezen A, Ziagkos D, LeBlond V, Abraham J, et al. 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. 2017;102:160–7.

    Article  CAS  Google Scholar 

  5. D’Souza A, Lacy M, Gertz M, Kumar S, Buadi F, Hayman S, et al. Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center Experience. Blood. 2012;120:56–62.

    Article  Google Scholar 

  6. Ohwada C, Sakaida E, Kwajri-Manako C, Nagao Y, Oshima-Hasegawa N, Togasaki E, et al. Long-term evaluation of physical improvement and survival of autologous stem cell transplantation in POEMS syndrome. Blood. 2018;131:2173–6.

    Article  CAS  Google Scholar 

  7. Saini NY, Patel RD, Varma A, Bashir Q, Delgado R, Rondon G, et al. Long-term durable efficacy of autologous stem cell transplantation in POEMS syndrome. Am J Hematol. 2019;94:E72–E74.

    Article  Google Scholar 

  8. Pramanik R, Sharma A, Sharma A, Gogia A, Sahoo RK, Kumar L, et al. POEMS syndrome: Indian Experience from a tertiary care institute. Clin lymphoma, myeloma Leuk. 2019;19:e536–e544.

    Article  Google Scholar 

  9. Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014;15:e538–48. https://doi.org/10.1016/S1470-2045(14)70442-5.

    Article  PubMed  Google Scholar 

  10. Karam C, Klein CJ, Dispenzieri A, Dyck PJ, Mandrekar J, D’Souza A, et al. Polyneuropathy improvement following autologous stem cell transplantation for POEMS syndrome. Neurology. 2015;84:1981–7.

    Article  CAS  Google Scholar 

  11. DiPersio JF, Micallef IN, Stiff PJ, Bolwell BJ, Maziarz RT, Jacobsen E, et al. Phase III prospective randomized double-blind placebo-controlled trial of plerixafor plus granulocyte colony-stimulating factor compared with placebo plus granulocyte colony-stimulating factor for autologous stem-cell mobilization and transplantation for patients with non-Hodgkin’s lymphoma. J Clin Oncol. 2009;27:4767–73.

    Article  CAS  Google Scholar 

  12. Dao LindaN, Hanson CurtisA, Dispenzieri Angela, Morice WilliamG, Kurtin PaulJ, Hoyer JamesD. Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients. Blood. 2011;117:6438–44. https://doi.org/10.1182/blood-2010-11-316935

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Nakaseko C. Autologous stem cell transplantation for POEMS syndrome. Clin Lymphoma, Myeloma Leuk. 2014;14:21–3.

    Article  Google Scholar 

Download references

Acknowledgements

We gratefully acknowledge the help of Transplant team and laboratory staff for help in the investigations and management of these patients.

AIIMS Myeloma Group

Prabhat Singh Malik1, Ranjit Kumar Sahoo1, Atul Sharma1, Ahitagni Biswas2, Sanjay Thulkar3, Rakesh Kumar4, Saumyaranjan Malik5, Lalit Kumar1

Author information

Authors and Affiliations

  1. Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India

    Annie K. Baa, Santosh K. Chellapuram, Atul Sharma, Prabhat Singh Malik, Ranjit Kumar Sahoo & Lalit Kumar

  2. Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India

    Ahitagni Biswas

  3. Department of Radio-Diagnosis, All India Institute of Medical Sciences, New Delhi, India

    Sanjay Thulkar

  4. Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India

    S. T. Arun Raj & Rakesh Kumar

  5. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Saumyaranjan Malik

Authors
  1. Annie K. Baa
    View author publications

    Search author on:PubMed Google Scholar

  2. Santosh K. Chellapuram
    View author publications

    Search author on:PubMed Google Scholar

  3. Atul Sharma
    View author publications

    Search author on:PubMed Google Scholar

  4. Prabhat Singh Malik
    View author publications

    Search author on:PubMed Google Scholar

  5. Ranjit Kumar Sahoo
    View author publications

    Search author on:PubMed Google Scholar

  6. Ahitagni Biswas
    View author publications

    Search author on:PubMed Google Scholar

  7. Sanjay Thulkar
    View author publications

    Search author on:PubMed Google Scholar

  8. S. T. Arun Raj
    View author publications

    Search author on:PubMed Google Scholar

  9. Rakesh Kumar
    View author publications

    Search author on:PubMed Google Scholar

  10. Saumyaranjan Malik
    View author publications

    Search author on:PubMed Google Scholar

  11. Lalit Kumar
    View author publications

    Search author on:PubMed Google Scholar

Consortia

for AIIMS Myeloma Group

  • Prabhat Singh Malik
  • , Ranjit Kumar Sahoo
  • , Atul Sharma
  • , Ahitagni Biswas
  • , Sanjay Thulkar
  • , Rakesh Kumar
  • , Saumyaranjan Malik
  •  & Lalit Kumar

Corresponding author

Correspondence to Lalit Kumar.

Ethics declarations

Competing interests

The authors declare no competing interests.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Members of the AIIMS Myeloma Group are listed below Acknowledgements.

Supplementary information

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Baa, A.K., Chellapuram, S.K., Sharma, A. et al. Poems syndrome: consolidation with autologous stem cell transplantation. Bone Marrow Transplant 56, 2597–2599 (2021). https://doi.org/10.1038/s41409-021-01395-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue date:

  • DOI: https://doi.org/10.1038/s41409-021-01395-7

This article is cited by

Search

Advanced search

Quick links