Poems syndrome

Wint Wint Thu Nyunt, Rabani Remli, Farah Azima Abdul Muttlib, Leong Chooi Fun, Noraidah Masir, Nor Rafeah Tumian, S Fadilah S. Abdul Wahid

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

POEMS syndrome is the syndrome of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and typical Skin changes. A 65-year-old lady presented with the 2-day-history of inability to walk, 4-month-history of progressive worsening of muscle weakness of both lower limbs and 1-year-history of progressive worsening of bilateral numbness of lower limbs. Nerve conduction study revealed generalized sensorimotor demyelinating polyneuropathy. She was initially treated as chronic inflammatory demyelinating polyradiculoneuropathy with intravenous immunoglobulin (IVIG) and high-dose prednisolone. However, she had no significant neurological improvement despite getting standard therapy. In addition to peripheral neuropathy, the presence of hepatosplenomegaly, skin changes, polycythaemia and thrombocytosis prompted for further investigations. She was diagnosed as POEMS syndrome based on the presence of two mandatory major criteria [polyneuropathy, monoclonal plasma cell proliferative disorder (lambda)], one major criterion (sclerotic bone lesions) and three minor criteria (organomegaly, skin changes and thrombocytosis/polycythaemia). She received treatment with melphalan and prednisolone. She achieved clinical improvement and partial response (haematologic and radiological) after six cycles of therapy. We highlight the awareness of this rare syndrome, for patients presenting with peripheral neuropathy and not responding to its standard therapy, by recognizing other associated clinical manifestations and proceeding further diagnostic work-up.

Original languageEnglish
Pages (from-to)297-303
Number of pages7
JournalMalaysian Journal of Pathology
Volume39
Issue number3
Publication statusPublished - 1 Dec 2017

Fingerprint

POEMS Syndrome
Thrombocytosis
Polycythemia
Polyneuropathies
Peripheral Nervous System Diseases
Prednisolone
Skin
Lower Extremity
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Melphalan
Hypesthesia
Intravenous Immunoglobulins
Neural Conduction
Muscle Weakness
Therapeutics
Plasma Cells
Bone and Bones
Proteins

Keywords

  • Monoclonal plasma cell proliferative disorder
  • Peripheral neuropathy
  • POEMS syndrome

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Cell Biology

Cite this

Poems syndrome. / Nyunt, Wint Wint Thu; Remli, Rabani; Abdul Muttlib, Farah Azima; Chooi Fun, Leong; Masir, Noraidah; Tumian, Nor Rafeah; S. Abdul Wahid, S Fadilah.

In: Malaysian Journal of Pathology, Vol. 39, No. 3, 01.12.2017, p. 297-303.

Research output: Contribution to journalArticle

Nyunt WWT, Remli R, Abdul Muttlib FA, Chooi Fun L, Masir N, Tumian NR et al. Poems syndrome. Malaysian Journal of Pathology. 2017 Dec 1;39(3):297-303.
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