Restricted Oligo-Clonal Usage of Monoclonal Immunoglobulin λ Light Chain Germline in POEMS Syndrome.

Nakaseko, Chiaki; Abe, Daijiro; Takeuchi, Masahiro; Takeda, Yusuke; Tanaka, Hiroaki; Oda, Kayo; Ohwada, Chikako; Ozawa, Shogo; Sakaida, Emiko; Shimizu, Naomi; Masuda, Saeko; Cho, Ryuko; Nishimura, Miki; Saitō, Yasushi; Misawa, Sonoko; Kuwabara, Satoshi

doi:10.1182/blood.v110.11.2483.2483

Cited by 9 publications

(9 citation statements)

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“… 18 The V-region of the Ig lambda gene interested was limited to the V lambda 1 subfamily (IGLV1). 8 , 19 Kang et al reported in 20 newly diagnosed POEMS cytogenetic aberrations similar to other plasma cell dyscrasias, but with a different incidence. 20 In particular, 14q32 (IGH) translocation was observed in 45% of the cases and included the t(4;14) and t(11;14) translocation (15% and 25% of the cases, respectively).…”

Section: Mandatory 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: Mandatory Criteriamentioning

confidence: 99%

Poems Syndrome: An Update

Nozza¹

2017

Mediterr J Hematol Infect Dis

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“…IL‐12 has also been shown to correlate with disease activity . Little is known about the plasma cells in POEMS syndrome except that more than 95% of the time they are lambda light chain restricted with restricted immunoglobulin light chain variable gene usage . Translocations and deletion of chromosome 13 have been described .…”

Section: Disease Overviewmentioning

confidence: 99%

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

Dispenzieri

2019

American J Hematol

225

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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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“…IL‐12 has also been shown to correlate with disease activity . Little is known about the plasma cells in POEMS syndrome except that more than 95% of the time they are lambda light chain restricted with restricted immunoglobulin light chain variable gene usage (IGLV1) . Translocations and deletion of chromosome 13 have been described, but hyperdiploidy is not seen …”

Section: Disease Overviewmentioning

confidence: 99%

POEMSsyndrome: 2017 Update on diagnosis, risk stratification, and management

2017

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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 3 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.

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Restricted Oligo-Clonal Usage of Monoclonal Immunoglobulin λ Light Chain Germline in POEMS Syndrome.

Cited by 9 publications

References 0 publications

Poems Syndrome: An Update

Poems Syndrome: An Update

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

POEMSsyndrome: 2017 Update on diagnosis, risk stratification, and management

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