Comprehensive Diagnosis and Management of POEMS Syndrome
Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delaye...
| Main Authors: | , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2022-11-01
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| Series: | HemaSphere |
| Online Access: | http://journals.lww.com/10.1097/HS9.0000000000000796 |
| _version_ | 1797227119713976320 |
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| author | Shirley D’Sa Jahanzaib Khwaja Stephen Keddie Ryan YS Keh Duncan Smyth Ruth Ronneberger Suraiya Dubash Suganya Sivabalasingham Simon Wan Chandrashekar Hoskote Stephanie Baldeweg Jonathan Sive Michael P. Lunn |
| author_facet | Shirley D’Sa Jahanzaib Khwaja Stephen Keddie Ryan YS Keh Duncan Smyth Ruth Ronneberger Suraiya Dubash Suganya Sivabalasingham Simon Wan Chandrashekar Hoskote Stephanie Baldeweg Jonathan Sive Michael P. Lunn |
| author_sort | Shirley D’Sa |
| collection | DOAJ |
| description | Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delayed by 12–16 months, by which time patients can be severely disabled. There are no established consensus guidelines. We provide clinicians a comprehensive blueprint for managing POEMS from diagnostic suspicion through the work-up, selection of therapy, follow-up, and treatment of relapse based on published evidence and our large single-center experience. A multidisciplinary approach is essential including expert hematologists, neurologists, histopathologists, radiologists, and neurophysiologists. The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. Supportive care considerations include management of endocrinopathy, neuropathy, thrombosis, and infection. Response assessment is centered on clinical, neuropathy, hematological, vascular endothelial growth factor, and radiological criteria. Future clinical trials are welcomed in this setting where evidence is limited. |
| first_indexed | 2024-04-24T14:35:44Z |
| format | Article |
| id | doaj.art-c63d39b172f24d7ca4ffc63a0b42d3cc |
| institution | Directory Open Access Journal |
| issn | 2572-9241 |
| language | English |
| last_indexed | 2024-04-24T14:35:44Z |
| publishDate | 2022-11-01 |
| publisher | Wiley |
| record_format | Article |
| series | HemaSphere |
| spelling | doaj.art-c63d39b172f24d7ca4ffc63a0b42d3cc2024-04-03T01:04:43ZengWileyHemaSphere2572-92412022-11-01611e79610.1097/HS9.0000000000000796202211000-00012Comprehensive Diagnosis and Management of POEMS SyndromeShirley D’Sa0Jahanzaib Khwaja1Stephen Keddie2Ryan YS Keh3Duncan Smyth4Ruth Ronneberger5Suraiya Dubash6Suganya Sivabalasingham7Simon Wan8Chandrashekar Hoskote9Stephanie Baldeweg10Jonathan Sive11Michael P. Lunn121 Department of Haematology, University College London Hospital, London, United Kingdom1 Department of Haematology, University College London Hospital, London, United Kingdom2 Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom2 Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom2 Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom3 Department of Endocrinology, University College London Hospital, London, United Kingdom4 Department of Oncology, University College London Hospital, London, United Kingdom4 Department of Oncology, University College London Hospital, London, United Kingdom5 Institute of Nuclear Medicine, University College London, United Kingdom6 Neuroradiology, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, United Kingdom3 Department of Endocrinology, University College London Hospital, London, United Kingdom1 Department of Haematology, University College London Hospital, London, United Kingdom2 Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, Queen Square, London, United KingdomPolyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delayed by 12–16 months, by which time patients can be severely disabled. There are no established consensus guidelines. We provide clinicians a comprehensive blueprint for managing POEMS from diagnostic suspicion through the work-up, selection of therapy, follow-up, and treatment of relapse based on published evidence and our large single-center experience. A multidisciplinary approach is essential including expert hematologists, neurologists, histopathologists, radiologists, and neurophysiologists. The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. Supportive care considerations include management of endocrinopathy, neuropathy, thrombosis, and infection. Response assessment is centered on clinical, neuropathy, hematological, vascular endothelial growth factor, and radiological criteria. Future clinical trials are welcomed in this setting where evidence is limited.http://journals.lww.com/10.1097/HS9.0000000000000796 |
| spellingShingle | Shirley D’Sa Jahanzaib Khwaja Stephen Keddie Ryan YS Keh Duncan Smyth Ruth Ronneberger Suraiya Dubash Suganya Sivabalasingham Simon Wan Chandrashekar Hoskote Stephanie Baldeweg Jonathan Sive Michael P. Lunn Comprehensive Diagnosis and Management of POEMS Syndrome HemaSphere |
| title | Comprehensive Diagnosis and Management of POEMS Syndrome |
| title_full | Comprehensive Diagnosis and Management of POEMS Syndrome |
| title_fullStr | Comprehensive Diagnosis and Management of POEMS Syndrome |
| title_full_unstemmed | Comprehensive Diagnosis and Management of POEMS Syndrome |
| title_short | Comprehensive Diagnosis and Management of POEMS Syndrome |
| title_sort | comprehensive diagnosis and management of poems syndrome |
| url | http://journals.lww.com/10.1097/HS9.0000000000000796 |
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