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Weekly Chest CasesArchive of Old Cases

Case No : 1025 Date 2017-06-19

  • Courtesy of Go Eun Yang, Dong Jin Im, Hye-Jeong Lee, Young Jin Kim / Severance Hospital, Chuncheon SungShim Hospital
  • Age/Sex 47 / F
  • Chief Complaintgeneralized edema and dyspnea for 20 days, sensorymotor polyneuropathy on electromyograph for 1 year and a half
  • Figure 1
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Diagnosis With Brief Discussion

Diagnosis
Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes (POEMS) syndrome
Radiologic Findings
Chest radiograph shows bilateral small pleural effusions.

Chest CT scan shows diffuse interlobular septal thickening and bilateral GGO suggesting pulmonary edema with bilateral pleural effusions. Small pericardial effusion and generalized subcutaneous edema were also noted.

Furthermore, several mild enlarged left supraclavicular lymph nodes and osteosclerotic lesion in T7 vertebral body are probably associated with autoimmune disorder including polyneuropathy. On PET/CT scan, there was diffuse mild FDG uptake on axial skeleton, suggesting hypermetabolic status. The plasma cells were 11.0% of absolute neutrophil counts on bone marrow aspiration.
Brief Review
Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and 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. Diagnosis is 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.
Significant advances have been made in the diagnosis and treatment of POEMS syndrome over the last decade. Autologous peripheral blood stem cell transplantation has become the first-line treatment for younger patients with normal organ function. Autologous transplantation has resulted in a high response rate and durable remission.
References
1. Jian Li, Dao-bin Zhou. New advances in the diagnosis and treatment of POEMS syndrome, British Journal of Haematology, May 2013.
2. Dispenzieri, A et al (2003) POEMS syndrome: definitions and long-term outcome. Blood, 101, 2496–2506.
3. Angela Dispenzieri, POEMS syndrome: Update on diagnosis, risk-stratification, and management, American Journal of Hematology, Vol 90, October 2015.
Keywords
POEMS syndrome, paraneoplastic syndrome, monoclonal gammopathy,

No. of Applicants : 101

▶ Correct Answer : 64/101,  63.4%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Hyogo Prefectural Amagasaki General Medical Center , Japan TOMOAKI OTANI
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Niigata University , Japan ATSUSHI UEHARA
  • - Korea University Guro Hospital , Korea (South) SEIYOUNG LEE
  • - Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - McGill University Health Center , Canada Alexandre Semionov
  • - NIMS, HYDERABAD , India BHASKAR K
  • - IRSA LA ROCHELLE , France, Metropolitan BIGOT
  • - Korea University Guro Hospital , Korea (South) Bit Na Park
  • - Ajou university hospital , Korea (South) Pae Sun Suh
  • - The University of Tokyo Hospital , Japan Yusuke Watanabe
  • - Chonbuk National University Hospital , Korea (South) EUN HA JUNG
  • - Chonnam national university hospital , Korea (South) GYEONG IL MOON
  • - Chungbuk National University Hospital , Korea (South) SOOJUNG LEE
  • - Seoul National University Hospital , Korea (South) SU YEON AHN
  • - consultant radiologist , India DEVINDERPAL SINGH DHANOTA
  • - Myongji Hospital , Korea (South) KYUNGHO KIM
  • - Other , Korea (South) SEONG SU KANG
  • - Asan Medical Center , Korea (South) HYUN JUNG KOO
  • - Busan National University Hospital , Korea (South) LEE YOU JIN
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - The Jikei university , Japan TAKU GOMI
  • - Korea University Guro Hospital , Korea (South) HYUNJI LEE
  • - Kyungpook national university hospital , Korea (South) Nari Jung
  • - Pusan national university hospital , Korea (South) SeungHyun Lee
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Ajou University Hospital , Korea (South) Taeyang Ha
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Yonsei University, Wonju Severance Christian Hospital , Korea (South) JUYEON LEE
  • - Other , Korea (South) SUNG HYUN YOON
  • - Seoul National University Hospital , Korea (South) JI EUN KIM
  • - Asan Medical Center , Korea (South) JIHYE KWON
  • - Chung buk national university hospital , Korea (South) hong gwon Byun
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Samsung Medical Center , Korea (South) MIN YEONG KIM
  • - Graduate school of Medical Sciences, International University of Health and Welfare , Japan SHIGERU KIRYU
  • - Kishiwada City Hospital , Japan HIROSHI TAGAWA
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Chonbuk National University Hospital , Korea (South) WON HYEONG IM
  • - Chungbuk National University Hospital , Korea (South) LEE JUNG HWAN
  • - Mallinckrodt Institute of Radiology , United States Naganathan BS Mani
  • - Other , Korea (South) HAYEON LEE
  • - Kyeongpook National University Hospital , Korea (South) SUNGJUN MOON
  • - Chungbuk national university hospital , Korea (South) Miran Yeon Yeon
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Chonbuk National University Hospital , Korea (South) WEON JANG
  • - The University of Tokyo Hospital , Japan TAKU TAJIMA
  • - Yujin Yamazaki Hospital , Japan MASASHI TAKAHASHI
  • - Max Hospital New Delhi , India RAJESH GOTHI
  • - Korea University Guro Hospital , Korea (South) JINWOO HAN
  • - Korea University Guro Hospital , Korea (South) SUN HONG
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Seoul National University Hospital , Korea (South) YONGSUB SONG
  • - Private sector , Greece VASILIOS TZILAS
  • - University of Utah , United States AKIHIKO SAKATA
  • - Pusan national university hospital , Korea (South) So min Park
▶ Correct Answer as Differential Diagnosis : 5/101,  5.0%
  • - , India RAMESH DHARAVATH
  • - Scans world , India PHILSON JOSEPH MUKKADA
  • - Kashan University of Medical Sciences, kashan , Iran EBRAHIM RAZI
  • - Wuhan Union Hospital , China QIGUANG CHENG
  • - Fortis hospital , Mohali , India SHALEEN RANA
▶ Semi-Correct Answer : 1/101,  1.0%
  • - Chonnam National University Hospital, Radiology department , Korea (South) Park byung jin
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