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Case Report

POEMS syndrome associated with Castleman

disease: a case report and literature review

Juan Kang, Fang Yang, Hong‑Ya Zhang, Meng‑Meng Hu, Feng Xia, Jin‑Cun Wang, Yan‑Chun Deng, Gang Zhao
Department of Neurology, Xijing Hospital, Fourth Military Medical University, Xi’an 710032, Shaanxi, China.

ABSTRA CT
Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS) syndrome is a multisystemic disorder that
clinically manifests as paraneoplastic and monoclonal plasma cell dyscrasia. Its acronym is derived from its principal characteristics:
polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes. Here, the authors reported a case of POEMS syndrome
that was also associated with Castleman disease. A 53-year-old female patient was admitted to our hospital with limb weakness,
numbness, edema, abdominal distention, and fever. Physical examination revealed tetraplegia, paraesthesia, and hyporeflexia in all
four limbs, in addition to lymphadenectasis, splenomegaly, skin hyperpigmentation, hypertrichosis, and pitting edema. Laboratory tests
and imaging revealed thrombocytosis, hypothyroidism, diabetes, hydropericardium, hydrothorax, splenomegaly, and lymphadenectasis.
Electromyography showed the characteristic patterns of both demyelinating disease and axonal degeneration. Serum protein
electrophoresis revealed monoclonal immunoglobulin G-lambda paraproteins. Histological examination clearly diagnosed the disease
as the hyaline vascular subtype. The final diagnosis in this case was POEMS syndrome in association with Castleman disease.

Key words: Castleman disease, hyaline vascular variant, M protein, polyneuropathy, POEMS syndrome

INTRODUCTION thrombosis, etc. [2,3,6-13] Early diagnosis is a challenge
because of the diverse clinical manifestations that are
Polyneuropathy, organomegaly, endocrinopathy, often accompanied with multiple organ injury. Here, we
M proteins, and skin changes (POEMS) syndrome, also reported a patient with the Castleman disease variant
known as Crow–Fukase syndrome, osteosclerotic myeloma, of POEMS syndrome, which we hope will prompt the
and Takatsuki syndrome, [1-4] is the paraneoplastic clinical universal recognition of POEMS.
manifestation of monoclonal plasma cell dyscrasia. POEMS
syndrome is a multisystemic disorder, and its acronym is CASE REPORT
derived from its principal characteristics: polyneuropathy,
organomegaly, endocrinopathy, M proteins, and skin A 53-year-old Chinese woman was admitted to the
changes. [5,6] Other important clinical features include Neurology Department of our hospital because of
progressive limbs weakness, numbness, and edema.
fever, papilledema, extravascular volume overload,
sclerosis, bone lesions, thrombocytosis, erythrocytosis, Approximately 7 months before admission, she began
elevated vascular endothelial growth factor (VEGF) levels, to develop limb weakness and numbness. Twenty
abnormal pulmonary function, predisposition toward days before admission, the patient developed fever
and edema. She had been diagnosed with diabetes
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and treated with insulin i.h. for 3 months prior to
Quick Response Code: admission. Her past medical history was unremarkable,
Website:
www.nnjournal.net with no history of smoking, alcohol use, HIV infection,
tuberculosis, or tumor.
DOI:
10.4103/2347-8659.135577 On examination, the patient was suffering
from progressive tetraplegia, paraesthesia, and

Corresponding Author: Prof. Gang Zhao, Department of Neurology, Xijing Hospital, Fourth Military Medical University,
No. 169 Changle West Road, Xi’an 710032, Shaanxi, China. E‑mail: zhaogang@fmmu.edu.cn

40 Neuroimmunol Neuroinflammation |Volume 1 | Issue 1 | June 2014