Daratumumab, Lenalidomide, and Dexamethasone Combination with Subsequent High-Dose Chemotherapy and Autologous Hematopoietic Stem Cell Transplantation in the Treatment of Newly Diagnosed POEMS Syndrome: A Case Report and Literature Review

Sergei Vyacheslavovich Semochkin, V.V. Lunin, A.E. Grachev, S.A. Akulshina, M.I. Akhmedov, Yu.B. Venediktova, M.A. Vernyuk, E.R. Nemtsova, L.S. Khayrullina, A.M. Chervontseva, I.V. Cherkashina, A.A. Fedenko, A.D. Kaprin,

DOI:

https://doi.org/10.21320/2500-2139-2026-19-4-464-471

POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes) is an orphan multisystem plasma cell dyscrasia-associated disease. It is challenging to diagnose because not all of the five signs included in this acronym may be evident on initial presentation. The diagnosis of POEMS syndrome is justified if two compulsory major criteria (polyneuropathy and monoclonal plasma cell proliferation), one of the three additional major and at least one of the six minor criteria are met. The current drug therapy for POEMS syndrome is aimed at eradicating the clonal plasma cell pool and is similar to that for multiple myeloma. High-dose chemotherapy (HDCT) and autologous hematopoietic stem cell transplantation (auto-HSCT) should constitute a mandatory part of induction therapy for patients aged under 65–70 years without significant comorbidities. This paper reports a case of a 29-year-old woman with newly diagnosed POEMS syndrome treated at a neurological clinic for 12 months for an “acute inflammatory demyelinating polyneuropathy”. The POEMS syndrome was diagnosed on the basis of two compulsory major (sensorimotor neuropathy and plasma cell proliferation), one additional (increased VEGF level), and four minor (hepatosplenomegaly, skin rash, hyperprolactinemia, and polycythemia) criteria. The patient received 4 RCd (lenalidomide, cyclophosphamide, dexamethasone) cycles and 6 DRd (daratumumab, lenalidomide, dexamethasone) cycles of induction therapy with subsequent HDCT and auto-HSCT. The treatment resulted in complete hematologic response, the VEGF level returned to normal, and neurological symptoms were almost entirely resolved. Maintenance therapy was administered with DR (daratumumab, lenalidomide) combination and yielded MRD-negative response 12 months later. Subsequently, the maintenance therapy with lenalidomide alone was continued. Thus, the DRd regimen can be regarded as an effective and safe option in the first- and subsequent-line therapies for POEMS syndrome.

  1. Jaccard A, Pascal V, Magy L, Roussel M. POEMS Syndrome. Presse Med. 2025;54(1):104270. doi: 10.1016/j.lpm.2025.104270. DOI: https://doi.org/10.1016/j.lpm.2025.104270
  2. Scheinker I. Myelom und Nervensystem. Deutsche Zeitschrift f. Nervenheilkunde. 1938;147:47–273. doi: 10.1007/BF01762445. DOI: https://doi.org/10.1007/BF01762445
  3. Crow RS. Peripheral neuritis in myelomatosis. Br Med J. 1956;2(4996):802–4. doi: 10.1136/bmj.2.4996.802. DOI: https://doi.org/10.1136/bmj.2.4996.802
  4. Bardwick PA, Zvaifler NJ, Gill GN, et al. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Medicine (Baltimore). 1980;59(4):311–22. doi: 10.1097/00005792-198007000-00006. DOI: https://doi.org/10.1097/00005792-198007000-00006
  5. Koike H, Sobue G. Crow-Fukase syndrome. Neuropathology. 2000;20(Suppl):S69–72. doi: 10.1046/j.1440-1789.2000.00309.x. DOI: https://doi.org/10.1046/j.1440-1789.2000.00309.x
  6. Dispenzieri A. POEMS syndrome: diagnosis, treatments, and outcomes. Hematology Am Soc Hematol Educ Program. 2025;2025(1):393–400. doi: 10.1182/hematology.2025000729. DOI: https://doi.org/10.1182/hematology.2025000729
  7. Пирадов М.А., Супонева Н.А., Гинзберг М.А. и др. POEMS-syndrome: обзор литературы и описание клинических наблюдений. Журнал неврологии и психиатрии им. С.С. Корсакова. 2014;114(4):4–10. [Piradov M.A., Suponeva N.A., Ginzberg M.A., et al. POEMS-syndrome: a literature review and case reports. S.S. Korsakov Journal of Neurology and Psychiatry. 2014;114(4):4–10. (In Russ)]
  8. Ishii A, Tsukamoto S, Mimura N, et al. Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry. Sci Rep. 2024;14(1):10362. doi: 10.1038/s41598-024-61034-1. DOI: https://doi.org/10.1038/s41598-024-61034-1
  9. Jurczyszyn A, Olszewska-Szopa M, Vesole D. POEMS Syndrome-Clinical Picture and Management. Current Knowledge. Clin Lymphoma Myeloma Leuk. 2023;23(8):575–82. doi: 10.1016/j.clml.2023.04.008. DOI: https://doi.org/10.1016/j.clml.2023.04.008
  10. Fajgenbaum DC, Shilling D. Castleman Disease Pathogenesis. Hematol Oncol Clin North Am. 2018;32(1):11–21. doi: 10.1016/j.hoc.2017.09.002. DOI: https://doi.org/10.1016/j.hoc.2017.09.002
  11. Tomasso A, Innocenti I, Autore F, et al. VEGF and IL-6 Correlation in POEMS: a Potential Upcoming Marker of Active Disease and Early Autologous BMT Response. Mediterr J Hematol Infect Dis. 2022;14(1):e2022007. doi: 10.4084/MJHID.2022.007. DOI: https://doi.org/10.4084/mjhid.2022.007
  12. D’Souza A, Hayman SR, Buadi F, et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood. 2011;118(17):4663–5. doi: 10.1182/blood-2011-06-362392. DOI: https://doi.org/10.1182/blood-2011-06-362392
  13. Sekiguchi Y, Misawa S, Shibuya K, et al. Ambiguous effects of anti-VEGF monoclonal antibody (bevacizumab) for POEMS syndrome. J Neurol Neurosurg Psychiatry. 2013;84(12):1346–8. doi: 10.1136/jnnp-2012-304874. DOI: https://doi.org/10.1136/jnnp-2012-304874
  14. Soubrier M, Dubost JJ, Serre AF, et al. Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum. 1997;40(4):786–7. doi: 10.1002/art.1780400430. DOI: https://doi.org/10.1002/art.1780400430
  15. Bélec L, Mohamed AS, Authier FJ, et al. Human herpesvirus 8 infection in patients with POEMS syndrome-associated multicentric Castleman’s disease. Blood. 1999;93(11):3643–53. DOI: https://doi.org/10.1182/blood.V93.11.3643
  16. Cousins E, Gao Y, Sandford G, Nicholas J. Human herpesvirus 8 viral interleukin-6 signaling through gp130 promotes virus replication in primary effusion lymphoma and endothelial cells. J Virol. 2014;88(20):12167–72. doi: 10.1128/JVI.01751-14. DOI: https://doi.org/10.1128/JVI.01751-14
  17. Gentile F, Terenghi F, Doneddu PE, et al. Regular assessment of serum vascular endothelial growth factor levels to monitor POEMS syndrome. Neurol Sci. 2024;45(2):727–33. doi: 10.1007/s10072-023-07064-5. DOI: https://doi.org/10.1007/s10072-023-07064-5
  18. Wang C, Su W, Zhang W, et al. Serum immunoglobulin free light chain and heavy/light chain measurements in POEMS syndrome. Ann Hematol. 2014;93(7):1201–6. doi: 10.1007/s00277-014-2019-y. DOI: https://doi.org/10.1007/s00277-014-2019-y
  19. Зиновьева О.Е., Новиков Д.К., Очил-зода Н.З., Рамеев В.В. Парапротеинемические полиневропатии. Российский неврологический журнал. 2024;29(2):4–16. doi: 10.30629/2658-7947-2024-29-2-4-16. [Zinovyeva O.E., Novikov D.K., Ochil-zoda N.Z., Rameev V.V. Paraproteinemic neuropathies. Russian neurological journal. 2024;29(2):4–16. doi: 10.30629/2658-7947-2024-29-2-4-16. (In Russ)] DOI: https://doi.org/10.30629/2658-7947-2024-29-2-4-16
  20. Graham RC, Hughes RA. A modified peripheral neuropathy scale: the Overall Neuropathy Limitations Scale. J Neurol Neurosurg Psychiatry. 2006;77(8):973–6. doi: 10.1136/jnnp.2005.081547. DOI: https://doi.org/10.1136/jnnp.2005.081547
  21. Clark MS, Howe BM, Glazebrook KN, et al. Osteolytic-variant POEMS syndrome: an uncommon presentation of “osteosclerotic” myeloma. Skeletal Radiol. 2017;46(6):817–23. doi: 10.1007/s00256-017-2607-8. DOI: https://doi.org/10.1007/s00256-017-2607-8
  22. Keddie S, Foldes D, Caimari F, et al. Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study. Neurology. 2020;95(3):e268–e279. doi: 10.1212/WNL.0000000000009940. DOI: https://doi.org/10.1212/WNL.0000000000009940
  23. Nasu S, Misawa S, Sekiguchi Y, et al. Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 2012;83(5):476–9. doi: 10.1136/jnnp-2011-301706. DOI: https://doi.org/10.1136/jnnp-2011-301706
  24. Khwaja J, D’Sa S, Lunn MP, Sive J. Evidence-based medical treatment of POEMS syndrome. Br J Haematol. 2023;200(2):128–36. doi: 10.1111/bjh.18400. DOI: https://doi.org/10.1111/bjh.18400
  25. Faizan U, Sana MK, Farooqi MS, Hashmi H. Efficacy and Safety of Regimens Used for the Treatment of POEMS Syndrome – A Systematic Review. Clin Lymphoma Myeloma Leuk. 2022;22(1):e26–e33. doi: 10.1016/j.clml.2021.07.033. DOI: https://doi.org/10.1016/j.clml.2021.07.033
  26. Семочкин С.В. Механизмы действия противоопухолевых иммуномодуляторов — от тератогенности к терапии множественной миеломы. Гематология и трансфузиология. 2022;67(2):240–60. doi: 10.35754/0234-5730-2022-67-2-240-260. [Semochkin S.V. Mechanisms of action of immunomodulatory drugs — from teratogenicity to treatment of multiple myeloma. Russian journal of hematology and transfusiology. 2022;67(2):240–60. doi: 10.35754/0234-5730-2022-67-2-240-260. (In Russ)] DOI: https://doi.org/10.35754/0234-5730-2022-67-2-240-260
  27. Cibeira MT, Rozman M, Segarra M, et al. Bone marrow angiogenesis and angiogenic factors in multiple myeloma treated with novel agents. Cytokine. 2008;41(3):244–53. doi: 10.1016/j.cyto.2007.11.017. DOI: https://doi.org/10.1016/j.cyto.2007.11.017
  28. Gao XM, Yu YY, Zhao H, et al. Bortezomib plus dexamethasone as first-line therapy for patients with POEMS syndrome. Ann Hematol. 2021;100(11):2755–61. doi: 10.1007/s00277-021-04609-6. DOI: https://doi.org/10.1007/s00277-021-04609-6
  29. Jurczyszyn A, Castillo JJ, Olszewska-Szopa M, et al. POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy – 108 Patients Multicenter Analysis. Clin Lymphoma Myeloma Leuk. 2022;22(5):297–304. doi: 10.1016/j.clml.2021.10.007. DOI: https://doi.org/10.1016/j.clml.2021.10.007
  30. Rajkumar SV. Multiple myeloma: 2024 update on diagnosis, risk-stratification, and management. Am J Hematol. 2024;99(9):1802–24. doi: 10.1002/ajh.27422. DOI: https://doi.org/10.1002/ajh.27422
  31. Vaxman I, Kumar SK, Buadi F, et al. Daratumumab, carfilzomib, and pomalidomide for the treatment of POEMS syndrome: The Mayo Clinic Experience. Blood Cancer J. 2023;13(1):91. doi: 10.1038/s41408-023-00859-x. DOI: https://doi.org/10.1038/s41408-023-00859-x
  32. Gavriatopoulou M, Ntanasis-Stathopoulos I, Fotiou D, et al. Upfront Daratumumab With Lenalidomide and Dexamethasone for POEMS Syndrome. Hemasphere. 2020;4(3):e381. doi: 10.1097/HS9.0000000000000381. DOI: https://doi.org/10.1097/HS9.0000000000000381
  33. Amabile E, Fazio F, Martelli M, Petrucci MT. First-line therapy with daratumumab, lenalidomide and dexamethasone for patient with POEMS syndrome: A case report. Leuk Res Rep. 2024;22:100491. doi: 10.1016/j.lrr.2024.100491. DOI: https://doi.org/10.1016/j.lrr.2024.100491
  34. Taenaka R, Shimokawa S, Katayama A, et al. Successful treatment with daratumumab, lenalidomide, and dexamethasone therapy followed by autologous stem cell transplantation for newly diagnosed polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome: a case report. J Med Case Rep. 2022;16(1):311. doi: 10.1186/s13256-022-03552-y. DOI: https://doi.org/10.1186/s13256-022-03552-y
  35. Wang J, Liao W, Liu Z, Kong D. Case report: DKRd regimen in the treatment of newly diagnosed POEMS syndrome and literature review. Front Oncol. 2024;14:1417380. doi: 10.3389/fonc.2024.1417380. DOI: https://doi.org/10.3389/fonc.2024.1417380

Keywords:

POEMS syndrome, Crow-Fukase syndrome, lenalidomide, daratumumab, DRd

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  • Sergei Vyacheslavovich Semochkin, MD, PhD, PA Gertsen Moscow Oncology Research Institute, branch of the NMRC of Radiology, 3 2-i Botkinskii pr-d, Moscow, Russian Federation, 125284, IM Sechenov First Moscow State Medical University, 8 korp. 2 Trubetskaya ul., Moscow, Russian Federation, 119991, e-mail: semochkin_sv@rsmu.ru

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Published

01.10.2026

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Vol. 19 No. 4 (2026)
RARE HEMATOLOGICAL TUMORS AND SYNDROMES

How to Cite

Semochkin S.V., Lunin V.V., Grachev A.E., et al. Daratumumab, Lenalidomide, and Dexamethasone Combination with Subsequent High-Dose Chemotherapy and Autologous Hematopoietic Stem Cell Transplantation in the Treatment of Newly Diagnosed POEMS Syndrome: A Case Report and Literature Review. Clinical Oncohematology. Basic Research and Clinical Practice. 2026;19(4):464–471. doi:10.21320/2500-2139-2026-19-4-464-471.

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