Skip to main content

Advertisement

Log in

The characteristics of ascites in patients with POEMS syndrome

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

The characteristics of ascites in patients with POEMS syndrome, which comprise polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes, are unknown. We described the frequency of ascites at presentation of POEMS syndrome and further evaluated for the pathogenesis and nature of the ascites. One hundred and six consecutive patients with POEMS syndrome in Chinese PLA General Hospital were evaluated for the presence of ascites, and the cellular and biochemical characteristics of the ascitic fluids were assessed. Serum levels of complement, cytokines, and clinical chemistry parameters were analyzed in peripheral blood samples of the patients with POEMS syndrome. Ascites was observed in 42 of 106 (39.6 %) patients with POEMS syndrome. Patients with ascites had significantly high serum levels of C3 and C4 complement components and TNF-α (all p < 0.01). In 31 (73.8 %) patients who underwent paracentesis, the ascitic fluids had low serum ascites albumin gradients (SAAG), indicating non-portal hypertension. Spontaneous bacterial peritonitis was not observed. Ascites is a common complication of POEMS syndrome and has characteristics of non-portal hypertension, based on low SAAG. Increased immune activation and inflammatory status could contribute to the pathogenesis of ascites in POEMS syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y, Satoyoshi E et al (1984) The Crow–Fukase syndrome: a study of 102 cases in Japan. Neurology 34:712–720

    Article  PubMed  CAS  Google Scholar 

  2. Cui RT, Huang XS, Shi Q, Tian CL, Liu JX, Pu CQ (2011) POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome in China. Intern Med J 41:481–485

    Article  PubMed  CAS  Google Scholar 

  3. Bardwick PA, Zveifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL (1980) Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes: the POEMS syndrome. Medicine 59:311–322

    Article  PubMed  CAS  Google Scholar 

  4. Dispenzieri A (2012) POEMS syndrome: update on diagnosis, risk-stratification, and management. Am J Hematol 87:804–814

    Article  PubMed  Google Scholar 

  5. Singh D, Wadhwa J, Kumar L, Raina V, Agarwal A, Kochupillai V (2003) POEMS syndrome: experience with fourteen cases. Leuk Lymphoma 44:1749–1752

    Article  PubMed  Google Scholar 

  6. Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR et al (2003) POEMS syndrome: definitions and long-term outcome. Blood 101:2496–2506

    Article  PubMed  CAS  Google Scholar 

  7. Higuchi M, Kamijo H, Koyama T, Kaneko Y, Kamijo Y, Kobayashi N et al (2003) POEMS syndrome caused refractory ascites in a polycystic disease patient undergoing hemodialysis. Clin Exp Nephrol 7:301–305

    Article  PubMed  Google Scholar 

  8. Watanabe O, Maruyama I, Arimura K, Kitajima I, Arimura H, Hanatani M et al (1998) Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow–Fukase (POEMS) syndrome. Muscle Nerve 21:1390–1397

    Article  PubMed  CAS  Google Scholar 

  9. Yamada Y, Sawai S, Misawa S, Kanai K, Shibuya K, Mori M et al (2013) Multiple angiogenetic factors are upregulated in POEMS syndrome. Ann Hematol 92:245–248

    Article  PubMed  CAS  Google Scholar 

  10. Light RW, Macgregor MI, Luchsinger PC, Ball WC Jr (1972) Pleural effusions: the diagnostic separation of transudates and exudates. Ann Intern Med 77:507–513

    Article  PubMed  CAS  Google Scholar 

  11. Nakazawa K, Itoh N, Shigematsu H, Koh CS (1992) An autopsy case of Crow–Fukase (POEMS) syndrome with a high level of IL-6 in the ascites. Special reference to glomerular lesions. Acta Pathol Jpn 42:651–656

    PubMed  CAS  Google Scholar 

  12. Bocsi J, Hambsch J, Osmancik P, Schneider P, Valet G, Tarnok A (2002) Preoperative prediction of pediatric patients with effusions and edema following cardiopulmonary bypass surgery by serological and routine laboratory data. Crit Care 6:226–233

    Article  PubMed  Google Scholar 

  13. Dyck PJ, Engelstad J, Dispenzieri A (2006) Vascular endothelial growth factor and POEMS. Neurology 66:10–12

    Article  PubMed  Google Scholar 

  14. Scarlato M, Previtali SC (2011) POEMS syndrome: the matter-of-fact approach. Curr Opin Neurol 24:491–496

    Article  PubMed  CAS  Google Scholar 

  15. Gherardi RK, Bélec L, Fromont G, Divine M, Malapert D, Ganlard P et al (1994) Elevated levels of interleukin-1β (IL-1β) and IL-6 in serum and increased production of IL-1β mRNA in lymph nodes of patients with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome. Blood 83:2587–2593

    PubMed  CAS  Google Scholar 

  16. D’Souza A, Hayman SR, Buadi F, Mauermann M, Lacy MQ, Gertz MA et al (2011) The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood 118:4663–4665

    Article  PubMed  Google Scholar 

  17. Mineta M, Hatori M, Sano H, Hosaka M, Kokubun S, Hiroki E et al (2006) Recurrent Crow–Fukase syndrome associated with increased serum levels of vascular endothelial growth factor: a case report and review of the literature. Tohoku J Exp Med 210:269–277

    Article  PubMed  Google Scholar 

  18. Scarlato M, Previtali SC, Carpo M, Pareyson D, Briani C, Del Bo R et al (2005) Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain 128(pt 8):1911–1920

    Article  PubMed  Google Scholar 

  19. Yamada Y, Sawai S, Misawa S, Kanai K, Shibuya K, Mori M et al (2013) Multiple angiogenetic factors are upregulated in POEMS syndrome. Ann Hematol 92:245–248

    Article  PubMed  CAS  Google Scholar 

  20. Chimenti MS, Ballanti E, Perricone C, Cipriani P, Giacomelli R, Perricone R (2013) Immunomodulation in psoriatic arthritis: focus on cellular and molecular pathways. Autoimmun Rev 12:599–606

    Article  PubMed  CAS  Google Scholar 

  21. Szczygiel AM, Brzezinka G, Targosz-Korecka M, Chlopicki S, Szymonski M (2012) Elasticity changes anti-correlate with NO production for human endothelial cells stimulated with TNF-α. Pflugers Arch 463:487–496

    Article  PubMed  CAS  Google Scholar 

  22. Nobile-Orazio E, Terenghi F, Giannotta C, Gallia F, Nozza A (2009) Serum VEGF levels in POEMS syndrome and immune-mediated neuropathies. Neurology 72:1024–1026

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Chuan Qiang Pu.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Cui, R.T., Yu, S.Y., Huang, X.S. et al. The characteristics of ascites in patients with POEMS syndrome. Ann Hematol 92, 1661–1664 (2013). https://doi.org/10.1007/s00277-013-1829-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-013-1829-7

Keywords

Navigation