POEMS SYNDROMES

NAME-ASMITA BASAK
B.TECH BIOTECHNOLOGY
ENROLLMENT NO-AU/2018/08/0002463
ROLL NO-UG/08/BTBIOT/2018/045
PROJECT WORK I
ADAMAS UNIVERSITY
UNDER THE SUPERVISION OF
Dr. Joydeep Paul
 Introduction
 Poems syndrome is a rare paraneoplastic syndrome that is caused by an
underlying plasma cell disorder.

main features include polyradiculoneuropathy, organomegaly, endocrinopathy,

monoclonal plasma cell disorder, and skin changes.

 It happens more often in men than in women and usually affects people in their
40s and 50s.

POEMS syndrome is rare and can be life-threatening. It is also known as

osteosclerotic myeloma, Crow-Fukase syndrome and Takatsuki syndrome.

The exact cause of POEMS syndrome is unknown, but research suggests that a
chemical called VEGF (vascular endothelial growth factor) plays an important role
in this disease.
 Signs and symptoms of POEMS syndromes
• Major criteria

Polyneuropathy
Monoclonal plasma cell disorder
 Minor criteria

Elevated serum or plasma vascular endothelial growth factor (VEGF)

Organomegaly (spleen, liver, or lymph nodes)
Endocrinopathy (thyroid, parathyroid, pituitary, pancreatic)
Skin changes (hyper-pigmentation, white nails, acrocyanosis, plethora)
Papilledema

 Others signs and symptoms

Weight loss
diarrhea
low B12 value
vision problem
Loss of muscle
numbness, pain, feeling very tired Fig1: Skin changes in POEMS symptoms: acrocyanosis, white nails clubbin
fluid retention throughout the body
 Fig 2: Cutaneous pigmentation Fig 3: Symmetrical edema of the double limbs

ig4: A 28-year-old male patient of POEMS syndrome showed (a) hyperpigmented cutaneous patch over chest (in the midline and right pyramid line loc
b) edema over a lower limb and (c) clubbing.
Diagnosis
 Biopsy
Blood and/or urine test
 Electromyelogram (EMG)
 Imaging
 Physical exam
 Other test as necessary

Treatment
 Chemotherapy
 Radiation therapy
 Physical therapy
 Other medications Fig 4 : MRI and MRA for a patient with POEMS syndrome and is. (A)
MRI of the brain showed a cerebral infarction of the right temporal
occipital lobe
(white arrow). A second MRI showed a new focal lesion of cerebral
infarction near the left centrum semiovale 1 month later (white arrow).
(C) MRA showed severe stenosis of the left intracranial internal carotid
artery, left middle cerebral artery, and left anterior artery (white arrows).
 Reference

1. Bardwick PA, Zvaifler NJ, Gill GN, et al. Plasma cell dyscrasia with polyneuropathy, organomegaly,
endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review
of the literature. Medicine (Baltimore) 1980; 59: 311–322.
2. Brown R, Ginsberg L. POEMS syndrome: clinical update. J Neurol 2019; 266: 268–277. DOI:
10.1007/s00415-018-9110-6 [PMC free article]
3.Dispenzieri A. POEMS syndrome: update on diagnosis, risk-stratification, and management. Am J
Hematol. 2015;90:951–962. [PubMed] [Google Scholar]2. Bardwick PA, Zvaifler NJ, Gill GN, Newman
D, Greenway GD, Resnick DL. Plasma cell dyscrasia with polyneuropathy, organomegaly,
endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review
of the literature. Medicine (Baltimore) 1980;59:311–322. [PubMed] [Google Scholar]
4. Lewerenz J, Gocht A, Hoeger PH, von den Driesch P, Eckert B, Lamszus K, Stuerenburg HJ, Methner
A. Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and
thrombocytosis in a case with POEMS syndrome. J Neurol. 2003;250:1488–1491. [PubMed] [Google
Scholar]
THANK YOU