POEMS syndrome

POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). Other features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, and elevated VEGF levels.



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Narrow down the case reports



Total: 449 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
11
(43.6%)
18477219
Glomeruloid hemangioma.
Yuri T, Yamazaki F, Takasu K, Shikata N, Tsubura A.
Pathol Int. 2008;58(6):390-5.
Purpura Ascites Pleural effusion
CD34 CD68 PECAM1
Biomarkers, Tumor Females Hemangioma Homo sapiens Middle Aged POEMS Syndrome Parotid Gland Sjogren's Syndrome Skin Neoplasms
12
(40.6%)
14673584
Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and thrombocytosis in a case with POEMS syndrome.
Lewerenz J, Gocht A, Hoeger PH, von den Driesch P, Eckert B, Lamszus K, Stuerenburg HJ, Methner A.
J Neurol. 2003;250(12):1488-91.
Hypertension Thrombocytosis Arteriovenous fistula Vitamin B12 deficiency
MYOM2 VEGFA
Adult Females Homo sapiens Lung POEMS Syndrome Paraproteinemias Spinal Cord Spinal Cord Diseases Thrombocytosis Vascular Diseases Vascular Endothelial Growth Factor A Vitamin B 12 Deficiency
13
(39.7%)
1786657
[A case of Crow-Fukase syndrome with increased serum interleukin-6].
Hitoshi S, Okazawa H, Shimizu J, Suzuki K, Sakuta M.
Rinsho Shinkeigaku. 1991;31(7):730-3.
Cyanosis Edema Thrombocytosis Increased circulating interleukin 6
IL6
Females Follow-Up Studies Hemangioma Homo sapiens Middle Aged POEMS Syndrome Skin Neoplasms Thrombosis
14
(38.8%)
25984208
(4421663)
POEMS syndrome with renal plasmacytoma and classic polyarteritis nodosa: a case report.
Jha PK, Vankalakunti M, Bonu R, Babu K, Ballal HS.
NDT Plus. 2011;4(6):406-9.
Edema Ascites Thrombocytosis
15
(38.2%)
1888577
[A case of Crow-Fukase syndrome associated with idiopathic thrombocytopenic purpura].
Kawaguchi Y, Nagasato K, Yoshimura T, Motomura M, Tsujihata M, Nagataki S.
No To Shinkei. 1991;43(4):377-80.
Edema Purpura
Adult Autoantibodies Autoimmune Diseases Axon Blood Platelets Edema Homo sapiens Male Nerve Degeneration Pigmentation Disorders Polyneuropathy Syndrome
16
(38.0%)
29279594
POEMS syndrome.
Nyunt WWT, Remli R, Abdul Muttlib FA, Leong CF, Masir N, Tumian NR, Abdul Wahid SFS.
Malays J Pathol. 2017;39(3):297-303.
Polyneuropathy Hepatosplenomegaly Thrombocytosis
Anti-Inflammatory Agents Females Homo sapiens Myeloablative Agonists POEMS Syndrome
16
(38.0%)
25332557
Multicentric, Hyaline Vascular Variant of Castleman's Syndrome.
Mukta V, Susmitha C, Kar R, Basu D, Das AK.
Indian J Hematol Blood Transfus. 2014;30(Suppl 1):126-30.
Polyneuropathy Hepatosplenomegaly Plasmacytosis
16
(38.0%)
19943169
Intracranial hemangiomas in a patient with POEMS syndrome.
Maurer GD, Schittenhelm J, Ernemann U, Kempf VA, Ritz R, Weller M, Schmidt F.
J Neurol. 2010;257(3):484-7.
Hemangioma Thrombocytosis Hepatomegaly
Biological Markers Brain Brain Neoplasms Diplopia Females Hemangioma, Cavernous, Central Nervous System Homo sapiens Low Vision Magnetic Resonance Imaging Middle Aged Neurosurgical Procedures POEMS Syndrome Peripheral Nervous System Diseases Plasmacytoma Up-Regulation (Physiology) Vascular Endothelial Growth Factor A
19
(37.7%)
14503355
[A case of anaphylactoid shock occurring immediately after the initiation of second intravenous administration of high-dose immunoglobulin (IVIg) in a patient with Crow-Fukase syndrome].
Takahashi T, Ono S, Ogawa K, Tamura M, Mizutani T.
Rinsho Shinkeigaku. 2003;43(6):350-5.
Cyanosis Edema Hepatosplenomegaly
VEGFA
Biological Markers Capillary Permeability Endothelial Growth Factors Females Homo sapiens Intercellular Signaling Peptides and Proteins Lymphocyte Activation Lymphokines Middle Aged POEMS Syndrome Pathologic Neovascularization Vascular Endothelial Growth Factor A Vascular Endothelial Growth Factors
20
(37.6%)
20403034
POEMS syndrome with idiopathic portal hypertension: autopsy case and review of the literature.
Inoue R, Nakazawa A, Tsukada N, Katoh Y, Nagao T, Nakanuma Y, Mukai K.
Pathol Int. 2010;60(4):316-20.
Edema Splenomegaly Portal fibrosis
MYOM2 VEGFA
Adult Fatal Outcome Females Fibrosis Hepatomegaly Homo sapiens Liver Middle Aged POEMS Syndrome Portal Hypertension
        

Phenotype(s) retrieved from Orphanet

    Total: 46

HPO ID Term Frequency
HP:0001271 Polyneuropathy Obligate (100%)
HP:0000135 Hypogonadism Very frequent (99-80%)
HP:0000818 Abnormality of the endocrine system Very frequent (99-80%)
HP:0003271 Visceromegaly Very frequent (99-80%)
HP:0005523 Lymphoproliferative disorder Very frequent (99-80%)
HP:0010702 Increased antibody level in blood Very frequent (99-80%)
HP:0011122 Abnormality of skin physiology Very frequent (99-80%)
HP:0000771 Gynecomastia Frequent (79-30%)
HP:0000819 Diabetes mellitus Frequent (79-30%)
HP:0000821 Hypothyroidism Frequent (79-30%)
HP:0000953 Hyperpigmentation of the skin Frequent (79-30%)
HP:0000969 Edema Frequent (79-30%)
HP:0000998 Hypertrichosis Frequent (79-30%)
HP:0001028 Hemangioma Frequent (79-30%)
HP:0001072 Thickened skin Frequent (79-30%)
HP:0001085 Papilledema Frequent (79-30%)
HP:0001284 Areflexia Frequent (79-30%)
HP:0001324 Muscle weakness Frequent (79-30%)
HP:0001541 Ascites Frequent (79-30%)
HP:0001698 Pericardial effusion Frequent (79-30%)
HP:0001820 Leukonychia Frequent (79-30%)
HP:0001824 Weight loss Frequent (79-30%)
HP:0001894 Thrombocytosis Frequent (79-30%)
HP:0002092 Pulmonary arterial hypertension Frequent (79-30%)
HP:0002202 Pleural effusion Frequent (79-30%)
HP:0002694 Sclerosis of skull base Frequent (79-30%)
HP:0002716 Lymphadenopathy Frequent (79-30%)
HP:0003401 Paresthesia Frequent (79-30%)
HP:0004054 Sclerosis of hand bone Frequent (79-30%)
HP:0004576 Sclerotic vertebral endplates Frequent (79-30%)
HP:0004979 Metaphyseal sclerosis Frequent (79-30%)
HP:0008207 Primary adrenal insufficiency Frequent (79-30%)
HP:0012378 Fatigue Frequent (79-30%)
HP:0012531 Pain Frequent (79-30%)
HP:0100639 Erectile abnormalities Frequent (79-30%)
HP:0100759 Clubbing of fingers Frequent (79-30%)
HP:0100925 Sclerosis of foot bone Frequent (79-30%)
HP:0000870 Prolactin excess Occasional (29-5%)
HP:0001063 Acrocyanosis Occasional (29-5%)
HP:0001901 Polycythemia Occasional (29-5%)
HP:0002111 Restrictive deficit on pulmonary function testing Occasional (29-5%)
HP:0002747 Respiratory insufficiency due to muscle weakness Occasional (29-5%)
HP:0004420 Arterial thrombosis Occasional (29-5%)
HP:0004936 Venous thrombosis Occasional (29-5%)
HP:0009125 Lipodystrophy Occasional (29-5%)
HP:0100963 Hyperesthesia Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 129

HPO ID Term # of case reports
HP:0001271 Polyneuropathy 214
HP:0000969 Edema 30
HP:0009830 Peripheral neuropathy 26
HP:0000822 Hypertension 20
HP:0001541 Ascites 13
HP:0002240 Hepatomegaly 13
HP:0002716 Lymphadenopathy 13
HP:0001433 Hepatosplenomegaly 12
HP:0031357 Glomeruloid hemangioma 12
HP:0001085 Papilledema 11
HP:0000821 Hypothyroidism 10
HP:0001744 Splenomegaly 9
HP:0001894 Thrombocytosis 9
HP:0000998 Hypertrichosis 8
HP:0001297 Stroke 8
HP:0000135 Hypogonadism 7
HP:0012050 Anasarca 7
HP:0001028 Hemangioma 5
HP:0001409 Portal hypertension 5
HP:0000846 Adrenal insufficiency 4
HP:0001217 Clubbing 4
HP:0005523 Lymphoproliferative disorder 4
HP:0031047 Paraproteinemia 4
HP:0100726 Kaposi's sarcoma 4
HP:0000771 Gynecomastia 3
HP:0000819 Diabetes mellitus 3
HP:0001698 Pericardial effusion 3
HP:0002094 Dyspnea 3
HP:0002140 Ischemic stroke 3
HP:0002202 Pleural effusion 3
HP:0002878 Respiratory failure 3
HP:0004420 Arterial thrombosis 3
HP:0012398 Peripheral edema 3
HP:0000093 Proteinuria 2
HP:0000112 Nephropathy 2
HP:0000622 Blurred vision 2
HP:0000793 Membranoproliferative glomerulonephritis 2
HP:0001138 Optic neuropathy 2
HP:0001370 Rheumatoid arthritis 2
HP:0001638 Cardiomyopathy 2
HP:0001640 Cardiomegaly 2
HP:0001873 Thrombocytopenia 2
HP:0001952 Glucose intolerance 2
HP:0002196 Myelopathy 2
HP:0002721 Immunodeficiency 2
HP:0003470 Paralysis 2
HP:0004934 Vascular calcification 2
HP:0004936 Venous thrombosis 2
HP:0005306 Capillary hemangioma 2
HP:0011505 Cystoid macular edema 2
HP:0012089 Arteritis 2
HP:0100778 Cryoglobulinemia 2
HP:0100820 Glomerulopathy 2
HP:0000044 Hypogonadotrophic hypogonadism 1
HP:0000083 Renal insufficiency 1
HP:0000097 Focal segmental glomerulosclerosis 1
HP:0000100 Nephrotic syndrome 1
HP:0000238 Hydrocephalus 1
HP:0000572 Visual loss 1
HP:0000802 Impotence 1
HP:0000823 Delayed puberty 1
HP:0000832 Primary hypothyroidism 1
HP:0000843 Hyperparathyroidism 1
HP:0000952 Jaundice 1
HP:0000958 Dry skin 1
HP:0000979 Purpura 1
HP:0001289 Confusion 1
HP:0001369 Arthritis 1
HP:0001635 Congestive heart failure 1
HP:0001658 Myocardial infarction 1
HP:0001701 Pericarditis 1
HP:0001824 Weight loss 1
HP:0001876 Pancytopenia 1
HP:0001903 Anemia 1
HP:0001909 Leukemia 1
HP:0001919 Acute kidney injury 1
HP:0001953 Diabetic ketoacidosis 1
HP:0002028 Chronic diarrhea 1
HP:0002102 Pleuritis 1
HP:0002170 Intracranial hemorrhage 1
HP:0002273 Tetraparesis 1
HP:0002315 Headache 1
HP:0002326 Transient ischemic attack 1
HP:0002516 Increased intracranial pressure 1
HP:0002664 Neoplasm 1
HP:0002754 Osteomyelitis 1
HP:0002797 Osteolysis 1
HP:0002901 Hypocalcemia 1
HP:0003207 Arterial calcification 1
HP:0003270 Abdominal distention 1
HP:0003271 Visceromegaly 1
HP:0003447 Axonal loss 1
HP:0003450 Axonal regeneration 1
HP:0003552 Muscle stiffness 1
HP:0004947 Arteriovenous fistula 1
HP:0005162 Left ventricular dysfunction 1
HP:0005521 Disseminated intravascular coagulation 1
HP:0006597 Diaphragmatic paralysis 1
HP:0007131 Acute demyelinating polyneuropathy 1
HP:0007133 Progressive peripheral neuropathy 1
HP:0007141 Sensorimotor neuropathy 1
HP:0007305 CNS demyelination 1
HP:0007663 Reduced visual acuity 1
HP:0008462 Cervical instability 1
HP:0008720 Primary testicular failure 1
HP:0010310 Chylothorax 1
HP:0010741 Pedal edema 1
HP:0011506 Choroidal neovascularization 1
HP:0011510 Drusen 1
HP:0012115 Hepatitis 1
HP:0012474 Carotid artery occlusion 1
HP:0012595 Mild proteinuria 1
HP:0012722 Heart block 1
HP:0025168 Left ventricular diastolic dysfunction 1
HP:0025406 Asthenia 1
HP:0025615 Abscess 1
HP:0030150 Plasmacytosis 1
HP:0030169 Gastric varix 1
HP:0030783 Increased serum interleukin-6 1
HP:0030880 Raynaud phenomenon 1
HP:0031273 Shock 1
HP:0031284 Flushing 1
HP:0031287 Seborrheic keratosis 1
HP:0032252 Granuloma 1
HP:0040049 Macular edema 1
HP:0100502 Vitamin B12 deficiency 1
HP:0100774 Hyperostosis 1
HP:0200034 Papule 1
HP:0200042 Skin ulcer 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID