Angiofollikuläre lymphatische Hyperplasie mit Plasmozytom und Polyneuropathie: Ein Fallbericht mit immunhistochemischer Untersuchung

Dworák, O.; Tschubel, K; Zhou, Huiqing; Meybehm, M.

doi:10.1007/bf01720834

Cited by 9 publications

(4 citation statements)

References 11 publications

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“…Castleman disease (or angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder which has many presentations, ranging from an asymptomatic unifocal mass to multifocal masses with a multitude of symptoms. The symptoms can range from simple B‐symptoms to various autoimmune phenomenon to a frank POEMS syndrome . Several published cases of “interesting features” associated with Castleman disease are likely cases of POEMS syndrome .…”

Section: Relationship To Castleman Disease and Castleman Disease Varimentioning

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: Relationship To Castleman Disease and Castleman Disease Varimentioning

confidence: 99%

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

2019

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“…Castleman Disease (or angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder which has many presentations, ranging from an asymptomatic unifocal mass to multifocal masses with a multitude of symptoms. The symptoms can range from simple B‐symptoms to various autoimmune phenomenon to a frank POEMS syndrome 3,5,53,105–139 . Several published cases of “interesting features” associated with Castleman disease are likely cases of POEMS syndrome 140–143 .…”

Section: Relationship To Castleman Disease and Castleman Disease Variant Of Poemsmentioning

confidence: 99%

POEMS syndrome: 2021 Update on diagnosis, risk‐stratification, and management

2021

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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. 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. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. Risk‐adapted therapy For those patients with a dominant 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. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.

show abstract

“…Castleman disease (or angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder which has many presentations, ranging from an asymptomatic unifocal mass to multifocal masses with a multitude of symptoms. The symptoms can range from simple B‐symptoms to various autoimmune phenomenon to a frank POEMS syndrome (Figure ) . Several published cases of “interesting features” associated with Castleman disease are likely cases of POEMS syndrome .…”

Section: Relationship To Castleman Disease and Castleman Disease Varimentioning

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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Angiofollikuläre lymphatische Hyperplasie mit Plasmozytom und Polyneuropathie: Ein Fallbericht mit immunhistochemischer Untersuchung

Cited by 9 publications

References 11 publications

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

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

POEMS syndrome: 2021 Update on diagnosis, risk‐stratification, and management

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

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