A spectrum of clinicopathological features of nephropathy associated with POEMS syndrome

Nakamoto, Yasushi; Imai, Hirokazu; Yasuda, Tadashi; Wakui, Hideki; Miura, Akira B.

doi:10.1093/ndt/14.10.2370

Cited by 89 publications

(85 citation statements)

References 25 publications

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“…In POEMS syndrome, the renal histologic findings are diverse with membranoproliferative features and evidence of endothelial injury being most common [87]. On both light and electron microscopy, mesangial expansion, narrowing of capillary lumina, basement membrane thickening, subendothelial deposits, widening of the sub-endothelial space, swelling and vacuolization of endothelial cells, and mesangiolysis predominate [88][89][90][91][92][93][94]. Standard immunofluorescence is negative [89,95], which differentiates it from primary membranoproliferative glomerulitis [87].…”

Section: Diagnosismentioning

confidence: 99%

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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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Section: Diagnosismentioning

confidence: 99%

“…Standard immunofluorescence is negative [89,95], which differentiates it from primary membranoproliferative glomerulitis [87]. Rarely, infiltration by plasma cell nests or Castleman-like lymphoma can be seen [94].…”

Section: Diagnosismentioning

confidence: 99%

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

2015

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“…La mayoría de los pacientes al diagnóstico del sP tiene una creatininemia normal y sólo 6% tiene ≥ 1,5 mg/dl; sin embargo, si se utiliza la cistatina C como índice de filtración glomerular, esta frecuentemente está elevada (71%) 11 . otras series han comunicado una frecuencia de creatininemias > 1,5 mg/dl superior (50%) 12 . en una serie china, 37% de los pacientes tenían un clearance de creatinina < 60 ml/min/1,73 m 2 , 9% < 30 ml/min/1,73 m 2 13 .…”

Section: Discussionunclassified

“…La relación BUN/creatinina a menudo está elevada, sugiriendo contracción de volumen intravascular. esto es más común en pacientes con ascitis 6,12 .…”

Section: Discussionunclassified

Compromiso renal en síndrome de Poems: Caso clínico

Vega

2016

Rev. méd. Chile

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jvegastieb@gmail.com e l síndrome de Poems (sP) es una rara manifestación paraneoplásica de un desorden monoclonal de células plasmáticas. Poems es un acrónimo en inglés de poliradiculoneuropatía, organomegalia, endocrinopatía, desorden clonal de células plasmáticas y alteraciones cutá-neas. La mayoría de los casos se han descrito en población asiática, pero hay comunicaciones en occidente y también en Chile 1-4 . el sP puede comprometer los riñones. La frecuencia del compromiso renal no está precisada 1,6,7 . Puede manifestarse solamente por alteraciones del examen de orina (hematuria y/o proteinuria) o comprometer la función renal severamente, requiriendo diálisis 7 . Las biopsias renales muestran características morfológicas que parecen deberse a la acción de una alta concentración de citoquinas y factores de crecimiento originadas en las células plasmáticas monoclonales 1,7 .se comunica el caso de una paciente con sP y afectación renal que tuvo una larga evolución de esta patología. el propósito de la comunicación es el advertir a los lectores de la posibilidad de compromiso renal en esta rara enfermedad. Caso clínicomujer de 30 años, sin antecedentes mórbidos, a quien a raíz de una consulta por un trastorno en la marcha bilateral ("steppage"), se diagnosticó una neuropatía periférica motora, que se confirmó con medición de conducción nerviosa. Destacaba hiperpigmentación cutánea.Reapareció 6 años más tarde relatando que había dejado de trabajar por debilidad de extremidades inferiores. La hiperpigmentación había aumentado, su peso disminuido, estaba en amenorrea y sufría diarrea en forma crónica. Al examen físico destacó una piel dura, acartonada, oscura y descamativa sin hiperpigmentación de pliegues; uñas blancas en vidrio de reloj, bocio

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“…Finally, a membranproliferativelike lesion with mesangiolysis and microaneurysm suggestive of microangiopathy have been reported in patients with POEMS syndrome characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (33,34). Although these lesions are reminiscent of thrombotic microangiopathy, there is no evidence of ongoing microangiopathic hemolytic anemia (35). The glomerular changes are thought to be secondary to cytokines (IL-6 and vascular endothelial growth factor) activation by the l-restricted monoclonal proteins.…”

Section: The Role Of Monoclonal Proteins In Kidney Diseasesmentioning

confidence: 99%

A Patient with Abnormal Kidney Function and a Monoclonal Light Chain in the Urine

Leung

Nasr

2016

CJASN

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Monoclonal gammopathy is increasingly recognized as a cause of kidney injury. These renal conditions behave differently than ones without monoclonal gammopathy and require specific treatment. To avoid misdiagnosis, testing for paraprotein should be performed in addition to vasculitis and autoimmune diseases serologies in adults with unexplained AKI or proteinuria. Because the prevalence of monoclonal gammopathy is much more common than glomerular diseases, the nephrotoxicity of the monoclonal protein must be confirmed before cytotoxic therapy is initiated. This can only be done by a kidney biopsy. After a monoclonal gammopathy of renal significant is verified, the evaluation should then focus on the identification of the pathologic clone, because therapy is clone specific. We present this patient to illustrate the clinical presentation of a patient with renal dysfunction and a monoclonal gammopathy. This patient is also used to discuss the diagnostic process in detail when monoclonal gammopathy-associated renal disease is suspected.

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A spectrum of clinicopathological features of nephropathy associated with POEMS syndrome

Cited by 89 publications

References 25 publications

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

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

Compromiso renal en síndrome de Poems: Caso clínico

A Patient with Abnormal Kidney Function and a Monoclonal Light Chain in the Urine

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