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)
1
(56.9%)
11037862
Fatal lymphoproliferative disease as a complication of Evans syndrome.
Jasty R, Strouse PJ, Castle VP.
J Pediatr Hematol Oncol. 2000;22(5):460-3.
Petechiae Hepatosplenomegaly Recurrent fever Coombs-positive hemolytic anemia
Epstein-Barr Virus Infections Homo sapiens Infant Lymphoproliferative Disorders Male Syndrome
2
(56.4%)
19740448
(2753340)
Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report.
Yoshida H, Ishida H, Yoshihara T, Oyamada T, Kuwana M, Imamura T, Morimoto A.
J Hematol Oncol. 2009;2:40.
Jaundice Splenomegaly Anemia
Homo sapiens Infant Male Syndrome
3
(52.7%)
24881920
Diffuse large B-cell lymphoma with hemolytic crisis developed twenty years after the onset of Evans syndrome.
Yoshimura T, Nakane T, Kamesaki T, Inaba A, Nishimoto M, Mukai S, Sakabe M, Ohsawa M, Fujino K, Koh H, Nakao Y, Nakamae H, Hino M.
Rinsho Ketsueki. 2014;55(5):546-51.
Purpura Splenomegaly Hemolytic anemia
DDIT3
Age of Onset Anemia, Hemolytic Antineoplastic Combined Chemotherapy Protocols Diffuse Large B-Cell Lymphoma Females Homo sapiens Thrombocytopenia
4
(50.5%)
30631001
Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus.
Darmawan G, Hamijoyo L, Oehadian A, Bandiara R, Amalia L.
Acta Med Indones. 2018;50(4):343-345.
Proteinuria Hemolytic anemia Erythema Hepatitis
BID KCNMB2
Adult Anticoagulants Females Homo sapiens Immunosuppressive Agents Lupus Erythematosus, Systemic Magnetic Resonance Imaging Sinus Thrombosis, Intracranial Thrombocytopenia beta 2-Glycoprotein I
5
(50.0%)
21961487
Glucocorticoid-resistant Evans' syndrome successfully controlled with low-dose cyclosporine.
Janic D, Krivokapic-Dokmanovic L, Jovanovic N, Lazic J, Rodic P, Jankovic S.
Int J Clin Pharmacol Ther. 2011;49(10):622-5.
Splenomegaly Coombs-positive hemolytic anemia
CD4 CD8A
Child Glucocorticoids Homo sapiens Immunosuppressive Agents Male Thrombocytopenia
6
(49.8%)
17805039
A rare condition associated with celiac disease: Evans syndrome.
Yarali N, Demirceken F, Kondolat M, Ozkasap S, Kara A, Tunc B.
J Pediatr Hematol Oncol. 2007;29(9):633-5.
Diabetes mellitus Anemia Vitamin B12 deficiency Thyroiditis
Celiac Disease Duodenum Females Hemoglobin Homo sapiens Syndrome
7
(49.1%)
27384637
[Dermatomiositis and evans syndrome associated with HTLV-1 infection].
Loja-Oropeza D, Zavala-Flores E, Vilca-Vasquez M.
Rev Peru Med Exp Salud Publica. 2016;33(1):162-7.
Purpura Hemolytic anemia Dehydration
Females HTLV-I Infections Homo sapiens Middle Aged Thrombocytopenia
8
(48.0%)
30787781
Early-onset Evans Syndrome in a 4-Month-Old Infant: A Case Report and Review of Literature.
Mohamed KK, Al-Qurashi FO, Al-Qahtani MH, Osman YS.
Saudi J Med Med Sci. 2017;5(2):177-180.
Jaundice Anemia
8
(48.0%)
18618381
[Immune-mediated hemolytic anemia under oxaliplatin].
Gundling F, Tiller M, Fuchs M, Schepp W.
Z Gastroenterol. 2008;46(7):695-9.
Jaundice Hemolytic anemia
Antineoplastic Agents Differential Diagnosis Females Homo sapiens Organoplatinum Compounds
8
(48.0%)
17179698
(2721940)
A patient with mixed type Evans syndrome: efficacy of rituximab treatment.
Park CY, Chung CH.
J Korean Med Sci. 2006;21(6):1115-6.
Jaundice Hemolytic anemia
Antibodies, Monoclonal, Murine-Derived Females Homo sapiens Immunologic Factors Middle Aged Monoclonal Antibodies Syndrome
        

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