As white as ivory: sclerotic vertebra in POEMS syndrome

Urea and electrolytes, liver function tests, C-reactive protein, ferritin, vitamin B12, and creatine phosphokinase were normal. 24 h urinary cortisol, serum cortisol, and corticotropin were normal and she had suppressed cortisol in response to an overnight 1 mg dexamethasone suppression test, which...

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Bibliographic Details
Published in:The Lancet (British edition) 2017-06, Vol.389 (10085), p.2261-2262
Main Authors: Liapis, Konstantinos, Dr, Vlachou, Sophia, MD, Markou, Athina, MD, Kakiopoulos, Georgios, MD, Kounadi, Theodora, MD, Anagnostopoulos, Nikolaos I, PhD, Piaditis, George P, PhD
Format: Article
Language:English
Subjects:
Adrenocorticotropic hormone
Androgens
Biocompatibility
Biomedical materials
Biopsy
Blood diseases
C-reactive protein
Case reports
Cortisol
Creatine
Creatine kinase
Cushing syndrome
Cyanocobalamin
Demyelination
Dexamethasone
Diabetes
Diabetes Mellitus
Diagnosis, Differential
Electrolytes
Electrolytic cells
Endocrine disorders
Endometrial cancer
Estradiol - blood
Female
Ferritin
Growth factors
Hepatitis
Hormones
Humans
Internal Medicine
Lesions
Liver
Lymphoma
Medical diagnosis
Middle Aged
Nervous system diseases
Pathology
Pituitary
Plasma cells
Plasmacytoma - pathology
POEMS Syndrome - complications
POEMS Syndrome - diagnosis
POEMS Syndrome - diagnostic imaging
Polyneuropathy
Proteins
Radiation therapy
Skin
Skin diseases
Skin lesions
Skin Pigmentation
Spinal Neoplasms - diagnostic imaging
Spine
Thoracic Vertebrae - diagnostic imaging
Tomography, X-Ray Computed
Tumors
Urea
Vascular diseases
Vascular endothelial growth factor
Vascular Endothelial Growth Factor A - analysis
Vascular Endothelial Growth Factor A - blood
Vertebrae
Vitamin B12
Weight Loss
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Description
Summary:Urea and electrolytes, liver function tests, C-reactive protein, ferritin, vitamin B12, and creatine phosphokinase were normal. 24 h urinary cortisol, serum cortisol, and corticotropin were normal and she had suppressed cortisol in response to an overnight 1 mg dexamethasone suppression test, which ruled out Cushing's syndrome. POEMS syndrome is a rare form of plasma cell dyscrasia, usually associated with osteosclerotic lesions.1 The designation POEMS was proposed by Bardwick and colleagues in 1980 as an acronym for this symptom complex, but not every patient has polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin lesions, and the acronym does not encompass every manifestation of this syndrome (appendix).1 The variability of clinical signs often makes the diagnosis of POEMS syndrome difficult. POEMS syndrome should always be considered in cases of demyelinating polyneuropathy or endocrinopathy of obscure cause.1,2 The mechanism of POEMS syndrome is not fully understood but might be related to very high concentrations of vascular endothelial growth factor (VEGF) produced by the plasma cells.3 VEGF-mediated vasculopathy and vasopermeability are thought to have a major role in its development. The so-called ivory vertebra is well described in association with metastatic cancer, lymphoma, Paget's disease, sarcoidosis, Pott's disease, and primary bony tumours.5
ISSN:0140-6736
1474-547X
DOI:10.1016/S0140-6736(17)31374-0