Serum Vegf Levels in Poems Syndrome and in Immune-Mediated Neuropathies

Nobile‐Orazio, Eduardo; Terenghi, F.; Giannotta, Claudia; Gallia, Francesca; Nozza, Andrea

doi:10.1212/01.wnl.0000344569.13496.ff

Cited by 82 publications

(59 citation statements)

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“…As in the case of the utility of serum VEGF, 2,6,7,20 plasma VEGF is neither 100% sensitive nor specific for the syndrome but can play an important role in making the diagnosis. Moreover, we have shown that plasma VEGF levels correlate better with clinical response than with CR.…”

Section: Resultsmentioning

confidence: 99%

“…[2][3][4][5] Although the true mediator of POEMS is yet unknown, VEGF has been used as a surrogate biomarker. To date, most published literature has evaluated serum VEGF levels in POEMS, [5][6][7] and levels are routinely used to aid in the diagnosis and monitoring of patients after treatment. Tokashiki et al suggested that serum VEGF may be the better test as it includes the VEGF level from the platelet compartment as well as the serum.…”

Section: Introductionmentioning

confidence: 99%

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The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome

D’Souza¹,

Hayman²,

Buadi³

et al. 2011

Blood

188

185

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The POEMS syndrome is associated with elevated vascular endothelial growth factor (VEGF) levels. Several studies have compared serum VEGF levels between POEMS patients and other disease entities showing higher serum VEGF in POEMS syndrome; however, it is unknown whether serum levels are reliable and reproducible given variable platelet release of VEGF. We therefore compared plasma levels of VEGF in 29 patients with POEMS syndrome with those of other disorders (n = 76). We demonstrated that plasma VEGF levels are useful in differentiating POEMS from other plasma cell dyscrasias, neuropathic processes, and multisystem illnesses. Plasma VEGF is also useful in monitoring disease activity after treatment and correlates with clinical improvements better than hematologic response.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome

D’Souza¹,

Hayman²,

Buadi³

et al. 2011

Blood

188

185

View full text Add to dashboard Cite

show abstract

“…The requirements set forth in Table I are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. Making the diagnosis can be a challenge, but a good history and physical examination followed by appropriate testing-most notably radiographic assessment of bones [40][41][42] measurement of VEGF [13,17,20,43,44], and careful analysis of a bone marrow biopsy [39]-can differentiate this syndrome from other conditions like CIDP, monoclonal gammopathy of undetermined significance (MGUS) neuropathy, and immunoglobulin light chain amyloid neuropathy. A high platelet count is seen in 54% of POEMS patients as compared to 1.5% of patients with CIDP [45].…”

Section: Diagnosismentioning

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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“…The requirements set forth in Table 1 are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. Making the diagnosis can be a challenge, but a good history and physical examination followed by appropriate testing (most notably radiographic assessment of bones, 34 measurement of VEGF, 14,18,35,36 and careful analysis of a bone marrow biopsy 37 ) can differentiate this syndrome from other conditions, such as chronic inflammatory polyradiculoneuropathy (CIDP), monoclonal gammopathy of undetermined significance, neuropathy, and immunoglobulin light chain amyloid neuropathy. Other important baseline tests include: complete blood count, creatinine, creatinine clearance, serum and urine protein electrophoresis with immunofixation, serum immunoglobulin free light chains, thyroid-stimulating hormone, prolactin, parathyroid hormone, testosterone (or estradiol), luteinizing hormone, follicle-stimulating hormone, plasma VEGF, bone marrow aspirate, and biopsy with immunohistochemical stains to document -restricted plasma cells, pulmonary function tests, electromyelogram with nerve conduction studies, and PET/CT, with special attention to the bone windows of the CT. A biopsy of a sclerotic lesion is not imperative in the proper clinical context.…”

Section: Diagnosismentioning

confidence: 99%

How I treat POEMS syndrome

Dispenzieri

2012

Blood

101

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POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. The other important features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, elevated VEGF, and abnormal pulmonary function. The diagnosis is based on having both the polyradiculoneuropathy and the monoclonal plasma cell disorder, and at least 1 of the other 3 major criteria (Castleman disease, sclerotic bone lesions, or elevated VEGF) and at least one minor criterion. The diagnosis is often delayed with intervening incorrect diagnoses of chronic inflammatory demyelinating polyradiculoneuropathy, myeloproliferative disorder, and monoclonal gammopathy of undetermined significance. Prompt treatment directed at the underlying plasma cell clone produces dramatic responses in the majority of patients. Although there are no randomized clinical trial data to direct best therapy, for patients with disseminated disease, highdose chemotherapy with peripheral blood transplantation has yielded durable benefit, whereas radiation therapy is typically effective for patients with a more localized presentation. More universal recognition of and more scientific inquiry into the underpinnings of the disease will provide direction toward the best treatment strategies in the future. (Blood. 2012;119(24):5650-5658) Introduction POEMS syndrome, 1 also known as osteosclerotic myeloma, Takatsuki syndrome, 2 and Crow-Fukase syndrome, 3,4 is a rare paraneoplastic syndrome resulting from an underlying plasma cell disorder. There acronym POEMS refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. There are 3 important points that relate to this memorable acronym. First, not all of the features within the acronym are required to make the diagnosis (Table 1). Second, there are other important features not included in the POEMS acronym, including papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, elevated VEGF levels, abnormal pulmonary function tests, and a predisposition toward thrombosis. Lastly, there is a Castleman disease variant of POEMS syndrome that may not be associated with a clonal plasma cell disorder. 5,6 Table 1 outlines the range of expected frequencies of each of the features based on the largest published series. 2,[7][8][9][10][11] The pathogenesis of the syndrome is not well understood. To date, VEGF is the cytokine that correlates best with disease activity, [12][13][14][15][16][17][18][19][20] although it may not be the driving force of the disease based on the mixed results seen with anti-VEGF therapy. 5,21-29 VEGF, which is expressed by osteoblasts, macrophages, tumor cells 30 (including plasma cells), 31,32 and megakaryocytes/pla...

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Serum Vegf Levels in Poems Syndrome and in Immune-Mediated Neuropathies

Cited by 82 publications

References 0 publications

The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome

The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome

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

How I treat POEMS syndrome

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