Effective Induction Therapy with Subcutaneous Administration of Bortezomib for Newly Diagnosed POEMS Syndrome: A Case Report and a Review of the Literature

Zeng, Ke; Yang, Jin-rong; Li, Jun; Wei, Qing; Yang, Yue; Liu, Ting; Niu, Ting

doi:10.1159/000343681

Cited by 24 publications

(14 citation statements)

References 42 publications

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“…Like lenalidomide and thalidomide, bortezomib also has anti‐VEGF and anti‐TNF effects. Bortezomib use has been reported in approximately 50 patients . The first report is difficult to interpret as the patient had a number of chemotherapies prior to receiving a bortezomib, doxorubicin, and dexamethasone combination.…”

Section: Management Of Poems Syndrome With Disseminated Bone Marrow Imentioning

confidence: 99%

POEMS Syndrome: 2019 Update on diagnosis, risk‐stratification, and management

2019

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Disease Overview: Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (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 PCD, 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. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. 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. 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.

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Section: Management Of Poems Syndrome With Disseminated Bone Marrow Imentioning

confidence: 99%

POEMS Syndrome: 2019 Update on diagnosis, risk‐stratification, and management

2019

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“…Bortezomib use has been reported in five patients [171][172][173][174][175]. The first report is difficult to interpret since the patient had a number of chemotherapies prior to receiving a bortezomib, doxorubicin, and dexamethasone combination.…”

Section: Management Of Poems Syndrome With Disseminated Bone Marrow Imentioning

confidence: 99%

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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“…During a median follow‐up period of 22 months, 4 of the 19 responders relapsed . Bortezomib has been noted to be effective as a single agent, or in combination with other agents (steroids or steroids plus cyclophosphamide) . Thalidomide has been reported to be effective, but is limited by the risk of peripheral neuropathy .…”

Section: Poems Syndromementioning

confidence: 99%

“…108 Bortezomib has been noted to be effective as a single agent, or in combination with other agents (steroids or steroids plus cyclophosphamide). [109][110][111][112] Thalidomide has been reported to be effective, but is limited by the risk of peripheral neuropathy. [113][114][115] Results with anti-VEGF therapy (bevacizumab) have varied widely, from death to improvement.…”

Section: Poems Syndromementioning

confidence: 99%

Paraproteinemic neuropathies

2014

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The paraproteinemias are a heterogeneous group of disorders in which monoclonal plasma cells cause the proliferation of monoclonal proteins. They are of importance to clinicians because they often occur in association with neuropathies. Neurologists play a particularly important role when the neuropathy is the presenting feature, in which case they may uncover clinical, laboratory, radiologic, electrodiagnostic, or biopsy findings that lead to identification of the underlying paraproteinemia. The frequency of neuropathies in these patients, and the extent to which such neuropathies dominate the clinical picture, varies significantly between the different paraproteinemias. Treatments may be aimed specifically at the neuropathy, or against the underlying hematologic disorder. In all patients with paraproteinemias, the neurologist can work collaboratively with the hematologist to formulate therapeutic plans and goals and can provide follow-up and monitoring to determine the response of the neuropathy to treatment.

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Effective Induction Therapy with Subcutaneous Administration of Bortezomib for Newly Diagnosed POEMS Syndrome: A Case Report and a Review of the Literature

Cited by 24 publications

References 42 publications

POEMS Syndrome: 2019 Update on diagnosis, risk‐stratification, and management

POEMS Syndrome: 2019 Update on diagnosis, risk‐stratification, and management

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

Paraproteinemic neuropathies

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