POEMS syndrome caused refractory ascites in a polycystic disease patient undergoing hemodialysis

Higuchi, Makoto; Kamijo, Hiroshi; Koyama, Takayuki; Kaneko, Yoko; Kamijo, Yuji; Kobayashi, Nobuhiko; Shirakawa, Haruaki; Hora, Kazuhiko; Kiyosawa, Kendo

doi:10.1007/s10157-003-0247-x

Cited by 4 publications

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References 12 publications

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“…Vascular injury change of the peritoneal surface and/or permeability of the capillaries in visceral peritoneum are considered a mechanism of extravascular volume overload. 36 Extravascular volume overload can cause important morbidity in POEMS, and it is associated with shorter survival. 11 …”

Section: Minor Criteriamentioning

confidence: 99%

Poems Syndrome: An Update

Nozza¹

2017

Mediterr J Hematol Infect Dis

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POEMS syndrome is a rare, chronic and disabling condition. The causes of this condition remain unknown; however, chronic overproduction of proinflammatory cytokines appears to be a major contributor. Early diagnosis is essential to start treatment before the clinical state of the patient becomes compromised.A complete evaluation of the disease at its onset is critical to the treatment decision. In localized disease, curative doses of radiation (50 Gy) is the recommended therapy. On the other hand, patients with disseminated disease should be given systemic therapy. Treatment-related morbidity can be minimized by an efficient induction therapy that modifies the cytokine status, improving clinical condition and control disease severity before mobilization and transplantation. Patients not suitable for hematopoietic stem cell transplantation (HSCT) are usually treated with alkylator-based therapy. Novel agents may also offer benefits to patients with a poor performance status or renal dysfunction, and induce transplantation eligibility. Given the biological characteristics of POEMS, immunomodulatory effects and the absence of neurotoxicity, lenalidomide appears to be an effective therapy for the treatment of POEMS, both as short induction therapy before PBSCT and in non-transplant eligible patients, as it showed high response rate and durable responses.At present, however, guidelines for the diagnosis and treatment of POEMS are not available and appear advocated.

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Section: Minor Criteriamentioning

confidence: 99%

Poems Syndrome: An Update

Nozza¹

2017

Mediterr J Hematol Infect Dis

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The characteristics of ascites in patients with POEMS syndrome

Cui

Huang

et al. 2013

Ann Hematol

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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.

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