Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated w...

Full description

Saved in:
Bibliographic Details
Main Authors: Shuji Ueda, Sayoko Yonemoto, Kazumasa Oka, Naohiko Fujii, Keiichi Nakata, Hitomi Matsunaga, Seiko Kataoka, Yuki Iwama, Hiroyuki Narahara, Yuichi Yasunaga, Yoshiaki Inui, Sumio Kawata
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2014/818946
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832550287653470208
author Shuji Ueda
Sayoko Yonemoto
Kazumasa Oka
Naohiko Fujii
Keiichi Nakata
Hitomi Matsunaga
Seiko Kataoka
Yuki Iwama
Hiroyuki Narahara
Yuichi Yasunaga
Yoshiaki Inui
Sumio Kawata
author_facet Shuji Ueda
Sayoko Yonemoto
Kazumasa Oka
Naohiko Fujii
Keiichi Nakata
Hitomi Matsunaga
Seiko Kataoka
Yuki Iwama
Hiroyuki Narahara
Yuichi Yasunaga
Yoshiaki Inui
Sumio Kawata
author_sort Shuji Ueda
collection DOAJ
description POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated with very-low-dose lenalidomide and dexamethasone. A 57-year-old Japanese man was admitted to our hospital with pleural effusion, massive ascites, and leg edema. The diagnosis of POEMS syndrome was made based on the combination of the following findings: peripheral neuropathy, organomegaly, endocrinopathy, serum monoclonal protein elevation, skin changes, plasma VEGF elevation, and evidence of extravascular volume overload. Renal dysfunction induced by biopsy-proven renal involvement of POEMS syndrome was observed. Massive ascites of the patient dramatically diminished with long-time treatment of very-low-dose lenalidomide and dexamethasone. Lenalidomide seems to be a very promising therapy for POEMS syndrome presenting with extravascular volume overload such as edema, pleural effusion, and ascites. Very-low-dose lenalidomide might be effective especially for the patients with POEMS-related nephropathy.
format Article
id doaj-art-85ddf6ffc4b24572a46a738e3458a3a2
institution Kabale University
issn 2090-6560
2090-6579
language English
publishDate 2014-01-01
publisher Wiley
record_format Article
series Case Reports in Hematology
spelling doaj-art-85ddf6ffc4b24572a46a738e3458a3a22025-02-03T06:07:09ZengWileyCase Reports in Hematology2090-65602090-65792014-01-01201410.1155/2014/818946818946Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive AscitesShuji Ueda0Sayoko Yonemoto1Kazumasa Oka2Naohiko Fujii3Keiichi Nakata4Hitomi Matsunaga5Seiko Kataoka6Yuki Iwama7Hiroyuki Narahara8Yuichi Yasunaga9Yoshiaki Inui10Sumio Kawata11Department of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Pathology, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Radiology, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanDepartment of Internal Medicine, Hyogo Prefectural Nishinomiya Hospital, 13-9 Rokutanji-cho, Nishinomiya, Hyogo 662-0918, JapanPOEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated with very-low-dose lenalidomide and dexamethasone. A 57-year-old Japanese man was admitted to our hospital with pleural effusion, massive ascites, and leg edema. The diagnosis of POEMS syndrome was made based on the combination of the following findings: peripheral neuropathy, organomegaly, endocrinopathy, serum monoclonal protein elevation, skin changes, plasma VEGF elevation, and evidence of extravascular volume overload. Renal dysfunction induced by biopsy-proven renal involvement of POEMS syndrome was observed. Massive ascites of the patient dramatically diminished with long-time treatment of very-low-dose lenalidomide and dexamethasone. Lenalidomide seems to be a very promising therapy for POEMS syndrome presenting with extravascular volume overload such as edema, pleural effusion, and ascites. Very-low-dose lenalidomide might be effective especially for the patients with POEMS-related nephropathy.http://dx.doi.org/10.1155/2014/818946
spellingShingle Shuji Ueda
Sayoko Yonemoto
Kazumasa Oka
Naohiko Fujii
Keiichi Nakata
Hitomi Matsunaga
Seiko Kataoka
Yuki Iwama
Hiroyuki Narahara
Yuichi Yasunaga
Yoshiaki Inui
Sumio Kawata
Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites
Case Reports in Hematology
title Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites
title_full Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites
title_fullStr Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites
title_full_unstemmed Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites
title_short Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites
title_sort lenalidomide and dexamethasone for a patient of poems syndrome presenting with massive ascites
url http://dx.doi.org/10.1155/2014/818946
work_keys_str_mv AT shujiueda lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT sayokoyonemoto lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT kazumasaoka lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT naohikofujii lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT keiichinakata lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT hitomimatsunaga lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT seikokataoka lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT yukiiwama lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT hiroyukinarahara lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT yuichiyasunaga lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT yoshiakiinui lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites
AT sumiokawata lenalidomideanddexamethasoneforapatientofpoemssyndromepresentingwithmassiveascites