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Castleman disease variant of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes syndrome: A case report
Lih Jen Paw, Juen Kiem Tan, Chia Xuan Teoh, Puteri Nur Athirah Megat Mohamad Aminuddin, Ahmad Farhan Agusalim, Xiong Khee Cheong, Alvin Oliver Payus, Wan Alina Khadijah Wan Nik Ahmad Mustafa, Ummul Afila Omar, Norlaila Mustafa, Guang Yong Chong, Nor Rafeah Tumian, S Fadilah Abdul Wahid, Chia Yin Chong, Hui Jan Tan, Rabani Remli
Lih Jen Paw, Juen Kiem Tan, Chia Xuan Teoh, Puteri Nur Athirah Megat Mohamad Aminuddin, Ahmad Farhan Agusalim, Xiong Khee Cheong, Norlaila Mustafa, Guang Yong Chong, Nor Rafeah Tumian, S Fadilah Abdul Wahid, Hui Jan Tan, Rabani Remli, Department of Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur 56000, Malaysia
Juen Kiem Tan, Xiong Khee Cheong, Norlaila Mustafa, Guang Yong Chong, Nor Rafeah Tumian, S Fadilah Abdul Wahid, Hui Jan Tan, Rabani Remli, Department of Medicine, Hospital Canselor Tuanku Muhriz, Kuala Lumpur 56000, Malaysia
Alvin Oliver Payus, Department of Medicine, Faculty of Medicine and Health Sciences, Universiti Malaysia Sabah, Kota Kinabalu 88400, Sabah, Malaysia
Wan Alina Khadijah Wan Nik Ahmad Mustafa, Ummul Afila Omar, Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur 56000, Malaysia
Wan Alina Khadijah Wan Nik Ahmad Mustafa, Ummul Afila Omar, Department of Pathology, Hospital Canselor Tuanku Muhriz, Kuala Lumpur 56000, Malaysia
Chia Yin Chong, Department of Radiology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur 56000, Malaysia
Chia Yin Chong, Department of Radiology, Hospital Canselor Tuanku Muhriz, Kuala Lumpur 56000, Malaysia
Author contributions: Paw LJ, Tan JK, Teoh CX, Megat Mohamad Aminuddin PNA, Agusalim AF, Chong GY, and Chong CY contributed to manuscript writing and data collection; Cheong XK, Payus AO, Wan Nik Ahmad Mustafa WAK, Omar UA, Mustafa N, Tumian NR, Abdul Wahid SF, Tan HJ, and Remli R contributed to the data collection, analysis, and manuscript editing; Paw LJ, Tan JK, Chong GY, and Remli R contributed to the conceptualization and supervision; all authors have read and approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the patient and primary caregiver for publication of this report.
Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Rabani Remli, Associate Professor, Department of Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, Kuala Lumpur 56000, Malaysia.
rabaniremli@hctm.ukm.edu.my
Received: January 12, 2026
Revised: January 30, 2026
Accepted: February 24, 2026
Published online: March 16, 2026
Processing time: 64 Days and 19.8 Hours
BACKGROUND
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes (POEMS) syndrome is a rare plasma cell neoplasm with associated paraneoplastic syndrome. Castleman disease variant of POEMS syndrome, lacking detectable M-protein is uncommon and presents substantial diagnostic and therapeutic challenges. We illustrate a case of Castleman disease variant of POEMS syndrome and the complexity of its clinical management.
CASE SUMMARY
A 56-year-old man presented with limb weakness, sensory neuropathy, weight loss, and hyperpigmentation. He was diagnosed with Castleman disease variant of POEMS syndrome, having fulfilled 3 major criteria (polyneuropathy, Castleman disease, and vascular endothelial growth factor elevation) and 6 minor criteria (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, and thrombocytosis). He received melphalan plus dexamethasone, followed by three cycles of cyclophosphamide while planning for an autologous stem cell transplant, but suffered from seizures and thrombotic episodes throughout the course of his treatment. He gradually improved over 5 months, with improvement in limb strength, reduced pigmentation, and reduced fluid retention, but eventually succumbed to his illness due to sepsis.
CONCLUSION
Although a monoclonal plasma cell disorder is a mandatory diagnostic criterion for POEMS syndrome, it may be minimal or undetectable in some patients with the Castleman disease variant who otherwise fulfil diagnostic criteria.
Core Tip: Castleman disease variant of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes syndrome is a rare and challenging multisystem disorder to manage, with atypical variants without detectable M-protein exceptionally rare. This case highlights the importance of recognizing key clinical features and utilizing supportive markers, such as vascular endothelial growth factor and histopathology. To improve functional outcomes, quality of life, and minimize long-term morbidity, early diagnosis enables timely systemic chemotherapy treatment and multidisciplinary supportive care.
