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)
1
(55.1%)
22453045
[A case of POEMS syndrome associated with Waldenstrom's macroglobulinemia and treated with lenalidomide].
Furuta N, Tashiro Y, Ikeda M, Fujita Y, Okamoto K.
Rinsho Shinkeigaku. 2012;52(3):186-9.
Edema Hepatosplenomegaly Normochromic anemia
IL2RB IL6 VEGFA
Homo sapiens Male Middle Aged POEMS Syndrome Waldenstrom Macroglobulinemia
2
(48.9%)
20513935
POEMS syndrome: a rare case of monoclonal plasmaproliferative disorder.
Oehadian A, Prasetya D, Fadjari TH.
Acta Med Indones. 2010;42(2):100-3.
Hypertension Hepatosplenomegaly Anemia
Bone Marrow Combination Drug Therapy Differential Diagnosis Electrophoresis Females Glucocorticoids Homo sapiens Middle Aged Myeloablative Agonists POEMS Syndrome
2
(48.9%)
18376288
Case report: POEMS syndrome in childhood.
Sevketoglu E, Hatipoglu S, Ayan I, Dogan O, Salihoglu B.
J Pediatr Hematol Oncol. 2008;30(3):235-8.
Hypogonadism Hepatosplenomegaly Anemia
Abdomen Anemia Delayed Puberty Differential Diagnosis Females Growth Disorders Hepatomegaly Homo sapiens Hypogonadism POEMS Syndrome Paraproteinemias Pigmentation Disorders Predictive Value of Tests Steroids
4
(46.2%)
29113155
(5656028)
POEMS syndrome presentation with progressive weakness in upper and lower limbs: A case report.
Cao CJ, Dou CY, Zhou KH, Liu JB, Lai H.
Oncol Lett. 2017;14(5):5197-5202.
Edema Splenomegaly Thrombocytosis Hepatomegaly
5
(44.2%)
29739345
(5938810)
Atypical central retinal artery occlusion as the first presentation of POEMS syndrome: a case report.
Jindahra P, Dejthevaporn C, Niparuck P, Waisayarat J, Cheecharoen P, Threetong T, Petpiroon P, Sujirakul T, Poonyathalang A, Vanikieti K.
BMC Neurol. 2018;18(1):64.
Thrombocytosis Hepatomegaly Cystoid macular edema
VEGFA
Homo sapiens Male Middle Aged POEMS Syndrome Retinal Artery Occlusion
5
(44.2%)
28906499
[Incomplete POEMS syndrome with multicentric Castleman's disease].
Gonzalez de la Aleja P, Garcia-Navarro M, Sanchez-Martinez R, Ramos JM.
An Sist Sanit Navar. 2017;40(3):475-478.
Hepatosplenomegaly Pancytopenia Fever
Adult Homo sapiens Male POEMS Syndrome
5
(44.2%)
26634133
(4664845)
Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome.
Kumar S, Sharma S.
Oxf Med Case Reports. 2015;2015(3):237-40.
Edema Thrombocytosis Hepatomegaly
MYOM2 VEGFA
5
(44.2%)
23801136
Thrombocytopenia with reticulin fibrosis accompanied by fever, anasarca and hepatosplenomegaly : a clinical report of five cases.
Takai K, Nikkuni K, Momoi A, Nagai K, Igarashi N, Saeki T.
J Clin Exp Hematop. 2013;53(1):63-8.
Hepatosplenomegaly Thrombocytopenia Fever
Edema Females Fever Hepatomegaly Homo sapiens Male Middle Aged Thrombocytopenia
5
(44.2%)
21915731
[POEMS syndrome. An interdisciplinary clinical challenge].
Braun SA, Albrecht P, Methner A, Hanneken S.
Hautarzt. 2011;62(10):722-5.
Edema Thrombocytosis Hepatomegaly
VEGFA
Antineoplastic Agents Antineoplastic Agents, Hormonal Boronic Acids Combination Drug Therapy Cooperative Behavior Homo sapiens Immunoglobulin lambda-Chains Interdisciplinary Communication Male Middle Aged POEMS Syndrome Paraproteinemias Pyrazines Vascular Endothelial Growth Factor A
5
(44.2%)
20534952
[Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly].
Takai K, Nikkuni K, Shibuya H, Hashidate H.
Rinsho Ketsueki. 2010;51(5):320-5.
Hepatosplenomegaly Thrombocytopenia Fever
DDIT3
Ascites Differential Diagnosis Fatal Outcome Females Fever Hepatomegaly Homo sapiens Immunosuppressive Agents Male Middle Aged Primary Myelofibrosis Splenomegaly Syndrome Thrombocytopenia
        

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