Evans syndrome

A rare chronic hematologic disorder characterized by the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA; a disorder in which auto-antibodies are directed against red blood cells causing anemia of varying degrees of severity) with immune thrombocytopenic purpura (ITP; a coagulation disorder in which auto-antibodies are directed against platelets causing hemorrhagic episodes) and occasionally autoimmune neutropenia, in the absence of a known underlying etiology.



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Narrow down the case reports



Total: 190 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
177
(9.2%)
12634732
Refractory Evans' syndrome treated with allogeneic SCT followed by DLI. Demonstration of a graft-versus-autoimmunity effect.
Marmont AM, Gualandi F, Van Lint MT, Bacigalupo A.
Bone Marrow Transplant. 2003;31(5):399-402.
Autoimmunity
Adult Graft-vs-Host Disease Homo sapiens Lymphocyte Transfusion Male Tissue Donors Transplantation, Homologous
177
(9.2%)
11856438
Case report. Phaeohyphomycosis caused by Phialophora verrucosa developed in a patient with non-HIV acquired immunodeficiency syndrome.
Ohira S, Isoda K, Hamanaka H, Takahashi K, Nishimoto K, Mizutani H.
Mycoses. 2002;45(1-2):50-4.
Immunodeficiency
Acquired Immunodeficiency Syndrome Anti-Inflammatory Agents Dermatomycoses Fatal Outcome Females HIV Seronegativity Homo sapiens Japan Middle Aged Opportunistic Infections Phialophora Skin
177
(9.2%)
9273214
[Evan's syndrome with antiphospholipid-protein antibodies].
Frolow M, Jankowski M, Swadzba J, Musial J.
Pol Merkur Lekarski. 1996;1(5):344-5.
Lupus anticoagulant
F2
Adult Antiphospholipid Antibodies Antiphospholipid Syndrome Homo sapiens Male Syndrome Thrombocytopenia
184
(9.2%)
26681781
(5545791)
Fatal Myocarditis Associated With HHV-6 Following Immunosuppression in Two Children.
Stefanski HE, Thibert KA, Pritchett J, Prusty BK, Wagner JE, Lund TC.
Pediatrics. 2016;137(1):.
Myocarditis
Child, Preschool Fatal Outcome Females Herpesviridae Infections Homo sapiens Immunocompromised Host Male Myocarditis
185
(5.0%)
30394803
A rare case of Evans syndrome associated with sudden loss of vision: A case report.
Ababneh LT, Mahmoud IH, Al-Rimawi HS.
Eur J Ophthalmol. 2018;:1120672118809579.
Visual loss
Child Homo sapiens Male Retinal Artery Occlusion Retinitis Thrombocytopenia Vision Disorders
185
(5.0%)
29163997
(5661089)
Evans Syndrome Complicated by Intratubular Hemoglobin Cast Nephropathy.
Gonzalez I, Rais R, Gaut JP, Dehner LP.
Case Rep Pediatr. 2017;2017:5184587.
Nephropathy
185
(5.0%)
26938883
Endogenous mycotic endophthalmitis in a dog with candiduria and Evans syndrome.
Enders A, van der Woerdt A, Donovan T.
Vet Ophthalmol. 2017;20(1):84-88.
Glaucoma
Animals Candidiasis Canis familiaris Dog Diseases Endophthalmitis Females Thrombocytopenia
185
(5.0%)
11688823
Association of Graves' disease with Evans' syndrome in a patient with IgA nephropathy.
Ikeda K, Maruyama Y, Yokoyama M, Kato N, Yamanoto H, Kaguchi Y, Nakayama M, Shimada T, Tojo K, Kawamura T, Hosoya T.
Intern Med. 2001;40(10):1004-10.
Nephropathy
Adult Bone Marrow Females Graves Disease Homo sapiens IGA Glomerulonephritis Kidney Syndrome Thyroid Function Tests
185
(5.0%)
10715388
Vitrectomy for vitreous bleeding and tractional retinal detachment in a case of Evans syndrome.
Ohnishi A, Kaneko M, Hori S, Teramura M, Kato S.
Jpn J Ophthalmol. 2000;44(2):177-9.
Tractional retinal detachment
Adult Anemia, Hemolytic Autoimmune Diseases Eye Hemorrhage Fluorescein Angiography Homo sapiens Male Retinal Detachment Syndrome Vitrectomy
185
(5.0%)
4056835
Superior sagittal sinus thrombosis associated with Evans' syndrome of haemolytic anaemia.
Shiozawa Z, Ueda R, Mano T, Tsugane R, Kageyama N.
J Neurol. 1985;232(5):280-2.
Headache
Adult Cranial Sinuses Homo sapiens Intracranial Embolism and Thrombosis Male Syndrome Thrombophlebitis
        

Phenotype(s) retrieved from Orphanet

    Total: 13

HPO ID Term Frequency
HP:0001890 Autoimmune hemolytic anemia Obligate (100%)
HP:0001973 Autoimmune thrombocytopenia Obligate (100%)
HP:0000967 Petechiae Very frequent (99-80%)
HP:0001904 Neutropenia in presence of anti-neutropil antibodies Very frequent (99-80%)
HP:0000421 Epistaxis Frequent (79-30%)
HP:0000952 Jaundice Frequent (79-30%)
HP:0000978 Bruising susceptibility Frequent (79-30%)
HP:0000980 Pallor Frequent (79-30%)
HP:0001254 Lethargy Frequent (79-30%)
HP:0001324 Muscle weakness Frequent (79-30%)
HP:0002094 Dyspnea Frequent (79-30%)
HP:0012378 Fatigue Frequent (79-30%)
HP:0001279 Syncope Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 79

HPO ID Term # of case reports
HP:0001873 Thrombocytopenia 32
HP:0000979 Purpura 22
HP:0001878 Hemolytic anemia 17
HP:0001903 Anemia 9
HP:0001875 Neutropenia 5
HP:0100646 Thyroiditis 5
HP:0004844 Coombs-positive hemolytic anemia 4
HP:0030731 Carcinoma 4
HP:0000819 Diabetes mellitus 3
HP:0001909 Leukemia 3
HP:0000112 Nephropathy 2
HP:0000821 Hypothyroidism 2
HP:0001250 Seizures 2
HP:0001370 Rheumatoid arthritis 2
HP:0001876 Pancytopenia 2
HP:0001945 Fever 2
HP:0002721 Immunodeficiency 2
HP:0002960 Autoimmunity 2
HP:0011974 Myelofibrosis 2
HP:0032366 Positive direct antiglobulin test 2
HP:0200123 Chronic hepatitis 2
HP:0000093 Proteinuria 1
HP:0000099 Glomerulonephritis 1
HP:0000100 Nephrotic syndrome 1
HP:0000123 Nephritis 1
HP:0000541 Retinal detachment 1
HP:0000822 Hypertension 1
HP:0000836 Hyperthyroidism 1
HP:0000952 Jaundice 1
HP:0000962 Hyperkeratosis 1
HP:0000969 Edema 1
HP:0001045 Vitiligo 1
HP:0001297 Stroke 1
HP:0001342 Cerebral hemorrhage 1
HP:0001658 Myocardial infarction 1
HP:0001698 Pericardial effusion 1
HP:0001744 Splenomegaly 1
HP:0001824 Weight loss 1
HP:0001880 Eosinophilia 1
HP:0001882 Leukopenia 1
HP:0001888 Lymphopenia 1
HP:0001896 Reticulocytopenia 1
HP:0001919 Acute kidney injury 1
HP:0001952 Glucose intolerance 1
HP:0002013 Vomiting 1
HP:0002014 Diarrhea 1
HP:0002015 Dysphagia 1
HP:0002018 Nausea 1
HP:0002027 Abdominal pain 1
HP:0002090 Pneumonia 1
HP:0002098 Respiratory distress 1
HP:0002113 Pulmonary infiltrates 1
HP:0002138 Subarachnoid hemorrhage 1
HP:0002170 Intracranial hemorrhage 1
HP:0002315 Headache 1
HP:0002326 Transient ischemic attack 1
HP:0002514 Cerebral calcification 1
HP:0002716 Lymphadenopathy 1
HP:0002955 Granulomatosis 1
HP:0002958 Immune dysregulation 1
HP:0003003 Colon cancer 1
HP:0003701 Proximal muscle weakness 1
HP:0005305 Cerebral venous thrombosis 1
HP:0007430 Generalized edema 1
HP:0007917 Tractional retinal detachment 1
HP:0011854 Hemoperitoneum 1
HP:0012050 Anasarca 1
HP:0012251 ST segment elevation 1
HP:0012486 Myelitis 1
HP:0012531 Pain 1
HP:0012819 Myocarditis 1
HP:0025428 Bronchospasm 1
HP:0025615 Abscess 1
HP:0030049 Brain abscess 1
HP:0031246 Nonproductive cough 1
HP:0032061 Hypereosinophilia 1
HP:0100279 Ulcerative colitis 1
HP:0100778 Cryoglobulinemia 1
HP:0100827 Lymphocytosis 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID