Highly concentrated vascular endothelial growth factor in platelets in Crow-Fukase syndrome

Hashiguchi, Teruto; Arimura, Kimiyoshi; Matsumuro, Kenji; Otsuka, Reika; Watanabe, Osamu; Jonosono, Manabu; Maruyama, Yoshikazu; Maruyama, Ikuro; Osame, Mitsuhiro

doi:10.1002/1097-4598(200007)23:7<1051::aid-mus7>3.0.co;2-v

Cited by 126 publications

(95 citation statements)

References 23 publications

(30 reference statements)

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“…Plasma and serum levels of VEGF are markedly elevated in patients with POEMS [10,26,48] and correlate with the activity of the disease [12,16,26]. The principal isoform of VEGF expressed is VEGF165 [12].…”

Section: Diagnosismentioning

confidence: 95%

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

Dispenzieri

2011

American J Hematol

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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Section: Diagnosismentioning

confidence: 95%

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

Dispenzieri

2011

American J Hematol

119

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“…Serum VEGF levels (normal value was o500 pg/mL) were measured by ELISA assay as previously described. 11 Mobilization of PBSC The original mobilization regimen was designed as CY plus granulocyte CSF (G-CSF) (C þ G). Two patients developed neutropenia with fever and one patient had respiratory failure due to bacterial pneumonia among the first six patients who received C þ G. Then mobilization regimen was changed to G-CSF alone.…”

Section: Materials and Methods Patientsmentioning

confidence: 99%

PBSC mobilization in newly diagnosed patients with POEMS syndrome: outcomes and prognostic factors

Zhang

Duan

et al. 2012

Bone Marrow Transplant

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Autologous PBSC transplantation is a preferred treatment for patients with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS syndrome). However, data on stem cell mobilization in POEMS syndrome are limited. We retrospectively reviewed outcomes and factors prognostic of mobilization in newly diagnosed patients with POEMS syndrome. A total of 56 patients (41 men and 15 women) were included and the median age was 45 years (range, 24-62). All patients underwent mobilization with CY plus granulocyte CSF (G-CSF) (n ¼ 38) or G-CSF alone (n ¼ 18). The median total number of harvested CD34 þ cells was 2.01 Â 10 6 /kg (range, 0.18-9.0 Â 10 6 /kg). In all, 29 (51.8%) patients failed to yield more than 2 Â 10 6 /kg CD34 þ cells, and among them, 7 (12.5%) patients yielded o1 Â 10 6 /kg CD34 þ cells. The overall mobilization-related morbidity was 23.3% and acute renal failure was the most common complication during mobilization. Multivariate analysis showed that hepatomegaly (odds ratio 0.06, 95% confidence interval (CI) 0.01-0.43) and mobilization regimen of G-CSF alone (odds ratio 0.08, 95% CI 0.01-0.70) were independent prognostic factors for successful mobilization in POEMS syndrome. In conclusion, mobilization in newly diagnosed patients with POEMS syndrome is challenging with low yield of CD34 þ cells and high morbidity. Induction therapy before mobilization might improve the efficacy and safety of mobilization.

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“…Serum VEGF levels are 10-50 times higher plasma levels of VEGF [64] making it unclear which test is preferred. In patients with POEMS, VEGF is found in both plasma cells [30,31] and platelets [62]. The higher level observed in serum is attributable to the release of VEGF from platelets in vitro during serum processing.…”

Section: Diagnosismentioning

confidence: 98%

“…Plasma and serum levels of VEGF are markedly elevated in patients with POEMS [11,20,29,62] and correlate with the activity of the disease [13,17,20,29]. The principal isoform of VEGF expressed is VEGF165 [13].…”

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.

show abstract

Highly concentrated vascular endothelial growth factor in platelets in Crow-Fukase syndrome

Cited by 126 publications

References 23 publications

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

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

PBSC mobilization in newly diagnosed patients with POEMS syndrome: outcomes and prognostic factors

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

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