Poems syndrome (takatsuki syndrome)

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

POEMS syndrome [1], also known as osteosclerotic myeloma, Takatsuki syndrome [2], and Crow–Fukase syndrome [3, 4], is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The acronym POEMS refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Not all of the features within the acronym are required to make the diagnosis. There are other important features not included in the POEMS acronym, including papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis (P.E.S.T.), elevated vascular endothelial growth factor (VEGF) levels, abnormal pulmonary function tests, and a predisposition towards thrombosis. Lastly, there is a Castleman’s disease variant of POEMS syndrome that may not be associated with a clonal plasma cell disorder [5, 6]. Table 15.1 outlines the range of expected frequencies of each of the features based on the largest published series [2, 7–11]. VEGF is the cytokine that correlates best with disease activity [12–20], although it may not be the driving force of the disease based on the mixed results seen with anti-VEGF therapy [5, 21–29]. The pathogenesis of the syndrome is not well understood. VEGF, which is expressed by osteoblasts, macrophages, tumor cells [30] (including plasma cells) [31, 32], and megakaryocytes/platelets [33], is known to target endothelial cells, induce a rapid and reversible increase in vascular permeability, and be important in angiogenesis. Both IL-1β and IL-6 have been shown to stimulate VEGF production [30]. Interleukin 12 has also shown to be quite elevated in patients with POEMS syndrome [34]. 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) [5].

Original languageEnglish (US)
Title of host publicationMultiple Myeloma: Diagnosis and Treatment
PublisherSpringer New York
Pages179-194
Number of pages16
ISBN (Print)9781461485209, 9781461485193
DOIs
StatePublished - Jan 1 2014

Fingerprint

POEMS Syndrome
Plasma Cells
Vascular Endothelial Growth Factor A
Immunoglobulin Light Chain Genes
Polyradiculoneuropathy
Giant Lymph Node Hyperplasia
Paraneoplastic Syndromes
Thrombocytosis
Polycythemia
Papilledema
Megakaryocytes
Respiratory Function Tests
Capillary Permeability
Interleukin-12
Osteoblasts
Interleukin-1
Interleukin-6
Thrombosis
Blood Platelets
Endothelial Cells

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Dispenzieri, A. (2014). Poems syndrome (takatsuki syndrome). In Multiple Myeloma: Diagnosis and Treatment (pp. 179-194). Springer New York. https://doi.org/10.1007/978-1-4614-8520-9_15

Poems syndrome (takatsuki syndrome). / Dispenzieri, Angela.

Multiple Myeloma: Diagnosis and Treatment. Springer New York, 2014. p. 179-194.

Research output: Chapter in Book/Report/Conference proceedingChapter

Dispenzieri, A 2014, Poems syndrome (takatsuki syndrome). in Multiple Myeloma: Diagnosis and Treatment. Springer New York, pp. 179-194. https://doi.org/10.1007/978-1-4614-8520-9_15
Dispenzieri A. Poems syndrome (takatsuki syndrome). In Multiple Myeloma: Diagnosis and Treatment. Springer New York. 2014. p. 179-194 https://doi.org/10.1007/978-1-4614-8520-9_15
Dispenzieri, Angela. / Poems syndrome (takatsuki syndrome). Multiple Myeloma: Diagnosis and Treatment. Springer New York, 2014. pp. 179-194
@inbook{1808a27f316548c59ba76213b1448719,
title = "Poems syndrome (takatsuki syndrome)",
abstract = "POEMS syndrome [1], also known as osteosclerotic myeloma, Takatsuki syndrome [2], and Crow–Fukase syndrome [3, 4], is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The acronym POEMS refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Not all of the features within the acronym are required to make the diagnosis. There are other important features not included in the POEMS acronym, including papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis (P.E.S.T.), elevated vascular endothelial growth factor (VEGF) levels, abnormal pulmonary function tests, and a predisposition towards thrombosis. Lastly, there is a Castleman’s disease variant of POEMS syndrome that may not be associated with a clonal plasma cell disorder [5, 6]. Table 15.1 outlines the range of expected frequencies of each of the features based on the largest published series [2, 7–11]. VEGF is the cytokine that correlates best with disease activity [12–20], although it may not be the driving force of the disease based on the mixed results seen with anti-VEGF therapy [5, 21–29]. The pathogenesis of the syndrome is not well understood. VEGF, which is expressed by osteoblasts, macrophages, tumor cells [30] (including plasma cells) [31, 32], and megakaryocytes/platelets [33], is known to target endothelial cells, induce a rapid and reversible increase in vascular permeability, and be important in angiogenesis. Both IL-1β and IL-6 have been shown to stimulate VEGF production [30]. Interleukin 12 has also shown to be quite elevated in patients with POEMS syndrome [34]. 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) [5].",
author = "Angela Dispenzieri",
year = "2014",
month = "1",
day = "1",
doi = "10.1007/978-1-4614-8520-9_15",
language = "English (US)",
isbn = "9781461485209",
pages = "179--194",
booktitle = "Multiple Myeloma: Diagnosis and Treatment",
publisher = "Springer New York",

}

TY - CHAP

T1 - Poems syndrome (takatsuki syndrome)

AU - Dispenzieri, Angela

PY - 2014/1/1

Y1 - 2014/1/1

N2 - POEMS syndrome [1], also known as osteosclerotic myeloma, Takatsuki syndrome [2], and Crow–Fukase syndrome [3, 4], is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The acronym POEMS refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Not all of the features within the acronym are required to make the diagnosis. There are other important features not included in the POEMS acronym, including papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis (P.E.S.T.), elevated vascular endothelial growth factor (VEGF) levels, abnormal pulmonary function tests, and a predisposition towards thrombosis. Lastly, there is a Castleman’s disease variant of POEMS syndrome that may not be associated with a clonal plasma cell disorder [5, 6]. Table 15.1 outlines the range of expected frequencies of each of the features based on the largest published series [2, 7–11]. VEGF is the cytokine that correlates best with disease activity [12–20], although it may not be the driving force of the disease based on the mixed results seen with anti-VEGF therapy [5, 21–29]. The pathogenesis of the syndrome is not well understood. VEGF, which is expressed by osteoblasts, macrophages, tumor cells [30] (including plasma cells) [31, 32], and megakaryocytes/platelets [33], is known to target endothelial cells, induce a rapid and reversible increase in vascular permeability, and be important in angiogenesis. Both IL-1β and IL-6 have been shown to stimulate VEGF production [30]. Interleukin 12 has also shown to be quite elevated in patients with POEMS syndrome [34]. 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) [5].

AB - POEMS syndrome [1], also known as osteosclerotic myeloma, Takatsuki syndrome [2], and Crow–Fukase syndrome [3, 4], is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The acronym POEMS refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Not all of the features within the acronym are required to make the diagnosis. There are other important features not included in the POEMS acronym, including papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis (P.E.S.T.), elevated vascular endothelial growth factor (VEGF) levels, abnormal pulmonary function tests, and a predisposition towards thrombosis. Lastly, there is a Castleman’s disease variant of POEMS syndrome that may not be associated with a clonal plasma cell disorder [5, 6]. Table 15.1 outlines the range of expected frequencies of each of the features based on the largest published series [2, 7–11]. VEGF is the cytokine that correlates best with disease activity [12–20], although it may not be the driving force of the disease based on the mixed results seen with anti-VEGF therapy [5, 21–29]. The pathogenesis of the syndrome is not well understood. VEGF, which is expressed by osteoblasts, macrophages, tumor cells [30] (including plasma cells) [31, 32], and megakaryocytes/platelets [33], is known to target endothelial cells, induce a rapid and reversible increase in vascular permeability, and be important in angiogenesis. Both IL-1β and IL-6 have been shown to stimulate VEGF production [30]. Interleukin 12 has also shown to be quite elevated in patients with POEMS syndrome [34]. 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) [5].

UR - http://www.scopus.com/inward/record.url?scp=84956488808&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84956488808&partnerID=8YFLogxK

U2 - 10.1007/978-1-4614-8520-9_15

DO - 10.1007/978-1-4614-8520-9_15

M3 - Chapter

AN - SCOPUS:84956488808

SN - 9781461485209

SN - 9781461485193

SP - 179

EP - 194

BT - Multiple Myeloma: Diagnosis and Treatment

PB - Springer New York

ER -