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)

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Matched Phenotype Gene Mutation MeSH

Rank (Similarity)	PMID (PMCID)
12 (42.9%)	9081810	[Immune thrombocytopenia, anemia and leukopenia during pregnancy. Successful therapy with extracorporeal immunoadsorption]. Julius U, Patzak A, Schaich M, Ehninger G, Kamin G. Dtsch Med Wochenschr. 1997;122(8):220-4. [ Show abstract ] [ Hide abstract ]
		Petechiae Anemia


		Adult Autoantibodies Autoimmune Diseases Complement 3 Females Homo sapiens Immunosorbent Techniques Leukopenia Pregnancy Pregnancy Complications, Hematologic Renal Dialysis Thrombocytopenia
12 (42.9%)	3815308	Evans' syndrome associated with microcystic adenoma of the pancreas. Doll DC, List AF, Yarbro JW. Cancer. 1987;59(7):1366-8. [ Show abstract ] [ Hide abstract ]
		Purpura Hemolytic anemia


		Adenoma Anemia, Hemolytic Homo sapiens Male Pancreatic Neoplasm Syndrome
12 (42.9%)	2089175	Evans' syndrome associated with gastric plasmacytoma: case report and a review of the literature. Yonekura S, Nagao T, Arimori S, Miyaji M, Ogoshi K, Tsutsumi Y. Jpn J Med. 1990;29(5):512-5. [ Show abstract ] [ Hide abstract ]
		Purpura Hemolytic anemia


		Adult Antineoplastic Combined Chemotherapy Protocols Combined Modality Therapy Females Gastrectomy Homo sapiens Plasmacytoma Remission Induction Splenectomy Stomach Neoplasms Syndrome
12 (42.9%)	1925397	[Evans' syndrome caused by D-penicillamine in rheumatoid arthritis. Value of the corticoids-danazol combination]. Masson C, Bregeon C, Ifrah N, Berton V, Housseau F, Renier JC. Rev Rhum Mal Osteoartic. 1991;58(7):519-22. [ Show abstract ] [ Hide abstract ]
		Purpura Hemolytic anemia


		Combination Drug Therapy Homo sapiens Male Middle Aged Rheumatoid Arthritis Syndrome Time Factors
25 (42.5%)	18501091	Hodgkin lymphoma presenting with various immunologic abnormalities, including autoimmune hepatitis, Hashimoto's thyroiditis, autoimmune hemolytic anemia, and immune thrombocytopenia. Saitoh T, Matsushima T, Saito Y, Yamane A, Yokohoma A, Irisawa H, Handa H, Tsukamoto N, Karasawa M, Kojima M, Nojima Y, Murakami H. Clin Lymphoma Myeloma. 2008;8(1):62-4. [ Show abstract ] [ Hide abstract ]
		Thrombocytopenia Coombs-positive hemolytic anemia Hepatitis


		Autoimmune Chronic Hepatitis Females Hashimoto Disease Hodgkin Disease Homo sapiens Middle Aged
25 (42.5%)	12884819	[Myelodysplastic syndrome accompanied by Evans syndrome]. Takezako N, Shikai T, Tanimura A, Inokuchi C, Yamagata N, Masaki N, Miwa A. Rinsho Ketsueki. 2003;44(6):396-400. [ Show abstract ] [ Hide abstract ]
		Cirrhosis Anemia


		Autoimmune Diseases Females Hepatitis C Hepatitis C Antigens Homo sapiens Liver Cirrhosis Syndrome
25 (42.5%)	12540008	Intra-abdominal abscess caused by Listeria monocytogenes in a patient with acquired hemolytic anemia and thrombocytopenia. Sile H, Norwood J. South Med J. 2002;95(11):1350-2. [ Show abstract ] [ Hide abstract ]
		Thrombocytopenia Hemolytic anemia Liver abscess


		Abdominal Abscess Adult Anemia, Hemolytic Females Homo sapiens Listeriosis Postoperative Complications Splenectomy
25 (42.5%)	11910874	[Fatal bleeding complications caused by Evans syndrome (autoimmune thrombocytopenia and hemolytic anemia) and type II autoimmune hepatitis in a 56-year-old patient]. Zugmaier G, Jager R, Neubauer A, Mennel HD, Knabbe C, Dienes HP. Med Klin (Munich). 2002;97(2):88-90. [ Show abstract ] [ Hide abstract ]
		Portal hypertension Hemolytic anemia


		Autoimmune Chronic Hepatitis Brain Cerebral Hemorrhage Disease Progression Fatal Outcome Females Homo sapiens Middle Aged
29 (42.3%)	11254245	Induction of remission with intravenous immunoglobulin and cyclophosphamide in steroid-resistant Evans' syndrome associated with dermatomyositis. Chang DK, Yoo DH, Kim TH, Jun JB, Lee IH, Bae SC, Kim IS, Kim SY. Clin Rheumatol. 2001;20(1):63-6. [ Show abstract ] [ Hide abstract ]
		Purpura Reticulocytosis


		Dermatomyositis Females Homo sapiens Immunosuppressive Agents Middle Aged Remission Induction
30 (41.8%)	1433926	[Chronic lymphocytic leukemia diagnosed after corticosteroid therapy for Evans syndrome]. Tomioka M, Shimizu H, Matsuzaki T, Yuasa K, Iriuchijima T, Mori M, Murakami H, Naruse T. Rinsho Ketsueki. 1992;33(6):834-7. [ Show abstract ] [ Hide abstract ]
		Purpura Splenomegaly


		Anemia, Hemolytic Chronic Lymphocytic Leukemia Females Homo sapiens Lymphocyte Middle Aged Syndrome Thrombocytopenia

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