Pulmonary Manifestations in Patients With POEMS Syndrome

Allam, J. Shirine; Kennedy, Cassie C.; Aksamit, Timothy R.; Dispenzieri, Angela

doi:10.1378/chest.07-1800

Cited by 92 publications

(134 citation statements)

References 15 publications

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“…In our experience, finger-nail clubbing is seen in about 4% of cases, but others have reported rates as high as 49% [3,58] Laboratory findings are notable for an absence of cytopenias. In fact, nearly half of patients will have thrombocytosis or erythrocytosis [48].…”

Section: Diagnosismentioning

confidence: 46%

“…Respiratory complaints are usually limited given patients' neurologic status impairing their ability to induce cardiovascular challenges. In a series of 137 POEMS syndrome patients seen at our institution between 1975 and 2003, at presentation the frequency with which patients reported dyspnea, chest pain, cough, and orthopnea, were 20%, 10%, 8%, and 7%, respectively [58]. Patients are at increased risk for arterial and/or venous thromboses during their course, with nearly 20% of patients experiencing one of these complications [10,59].…”

Section: Diagnosismentioning

confidence: 99%

“…The pulmonary manifestations are protean, including pulmonary hypertension, restrictive lung disease, impaired neuromuscular respiratory function, and impaired diffusion capacity of carbon monoxide, but improve with effective therapy [58,79]. In a series of 20 patients with POEMS, followed over a 10-year period, 25% manifested pulmonary hypertension [79].…”

Section: Diagnosismentioning

confidence: 99%

“…In a larger series of 137 patients who were not uniformly tested, nearly 10% of patients had restrictive lung disease, reduced diffusing capacity of the lung for carbon dioxide (DLCO), and/or pulmonary hypertension. Nearly 25% had significant chest roentgenogram abnormalities [58]. Pulmonary hypertension has been reported to occur in 27% of unselected patients with POEMS a Percentages are based on the total number of patients in the series.…”

Section: Diagnosismentioning

confidence: 99%

“…The course of POEMS syndrome is usually chronic with modern estimated median survivals of nearly 14 years [48,58] Only fingernail clubbing, extravascular volume overload-i.e., effusions, edema, and ascites [48], respiratory symptoms [58], pulmonary hypertension [80] impaired DLCO, and papilledema [52] have been associated with a significantly shorter overall survival. The number of POEMS features does not affect survival [5,10].…”

Section: Risk Stratificationmentioning

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: 46%

Section: Diagnosismentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

Section: Risk Stratificationmentioning

confidence: 99%

See 3 more Smart Citations

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

2015

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Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome

Kuwabara

Dispenzieri

Arimura

et al. 2012

Cochrane Database of Systematic Reviews

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POEMS syndrome: 2011 update on diagnosis, risk‐stratification, and management

Dispenzieri

2011

American J Hematol

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119

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Disease overviewPOEMS 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 to 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. The benefit of anti‐VEGF antibodies is conflicting. 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. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 86:592–601, 2011. © 2011 Wiley‐Liss, Inc.

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Pulmonary Manifestations in Patients With POEMS Syndrome

Cited by 92 publications

References 15 publications

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

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

Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome

POEMS syndrome: 2011 update on diagnosis, risk‐stratification, and management

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