The characteristics of ascites in patients with POEMS syndrome

Cui, Rong; Yu, Songyan; Huang, Xu; Zhang, Jiatang; Li, Fēi; Pu, Chuan Qiang

doi:10.1007/s00277-013-1829-7

Cited by 24 publications

(23 citation statements)

References 20 publications

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“…(3,5,19), but was 87.5% when assessed by CT in the current study. By ultrasonography ascites were reported in 39.6% patients from a Chinese report (5) and were detected in 44.4% patients in this study, but ascites were detected by CT in 54.2% patients in the current study. The differences between detection rates by ultrasonography and by CT are probably due to the small volume of effusion which can only be detected by more sensitive CT.…”

Section: Discussioncontrasting

confidence: 79%

CT characteristics in 24 patients with POEMS syndrome

Shi

Luo

et al. 2015

Acta Radiol

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Section: Discussioncontrasting

confidence: 79%

CT characteristics in 24 patients with POEMS syndrome

Shi

Luo

et al. 2015

Acta Radiol

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“…The composition of the ascites was studied in 42 patients with POEMS syndrome. The ascitic fluid had low serum ascites albumin gradients consistent with an exudative rather than a portal hypertension process in 74% of cases [65].…”

Section: Diagnosismentioning

confidence: 97%

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

2015

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Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome.Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy.Risk‐adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3–6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. The benefit of anti‐VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 90:951–962, 2015. © 2015 Wiley Periodicals, Inc.

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“…However according to a recent study of 42 patients with ascites, they all demonstrated a SAAG of <1.1, suggesting a nonportal hypertensive cause. 3 This was not the case for this particular patient. She had 2 paracenteses that showed a SAAG of >1.1 before diagnosis.…”

Section: Conflicts Of Interestmentioning

confidence: 72%