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.

Input patient's signs and symptoms

Narrow down the case reports

Total: 190 (papers)

(per page)

Matched Phenotype Gene Mutation MeSH

Rank (Similarity)	PMID (PMCID)
11 (45.3%)	19579705	Visceral leishmaniasis in a patient with common variable immunodeficiency and evans syndrome: clinical remarks. Maio P, Leone S, Volpe S, dell'Aquila G, Giglio S, Magliocca M, Nigro FS, Pacifico P, Acone N. New Microbiol. 2009;32(2):223-7. [ Show abstract ] [ Hide abstract ]
		Splenomegaly Pancytopenia Fever


		Antiprotozoal Agents Common Variable Immunodeficiency Fever Glucocorticoids Homo sapiens Immunocompromised Host Leishmaniasis, Visceral Male Middle Aged Splenectomy Thrombocytopenia
12 (42.9%)	30733882	A Case of Pregnancy Complicated with Evans Syndrome with Sequential Development of Autoimmune Warm Antibody Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura. Suzuki H, Yamanoi K, Ogura J, Hirayama T, Yasumoto K, Shitanaka S, Inayama Y, Sakai M, Ohara T, Suginami K. Case Rep Obstet Gynecol. 2019;2019:2093612. [ Show abstract ] [ Hide abstract ]
		Purpura Hemolytic anemia
		HP


12 (42.9%)	30620809	[A case of Evans syndrome in a long-term hemodialysis patient]. Ishikawa M, Hirose C, Zhang J, Hori K, Oyama S, Sugano K, Ohno A, Tomita K. Nihon Jinzo Gakkai Shi. 2017;59(4):578-583. [ Show abstract ] [ Hide abstract ]
		Purpura Hemolytic anemia


		Fatal Outcome Females Homo sapiens Renal Dialysis Thrombocytopenia
12 (42.9%)	30223886 (6142628)	Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature. Alias H, Yong WL, Muttlib FAA, Koo HW, Loh CK, Lau SCD, Alauddin H, Azma RZ. J Med Case Rep. 2018;12(1):276. [ Show abstract ] [ Hide abstract ]
		Purpura Microangiopathic hemolytic anemia
		ADAMTS13

		ADAMTS13 Protein Child, Preschool Homo sapiens Male Purpura, Thrombotic Thrombocytopenic
12 (42.9%)	23603626	A case report of Evans Syndrome. Dosi RV, Ambaliya AP, Patell RD, Patil RS, Shah PJ. Indian J Med Sci. 2012;66(3-4):82-5. [ Show abstract ] [ Hide abstract ]
		Purpura Anemia


		Adult Blood Cell Count Dyspnea Epistaxis Females Glucocorticoids Homo sapiens Thrombocytopenia Young Adult
12 (42.9%)	23273499	Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome). Korkmaz H, Bugdaci MS, Temel T, Dagli M, Karabagli P. Clin Res Hepatol Gastroenterol. 2013;37(2):e45-50. [ Show abstract ] [ Hide abstract ]
		Purpura Anemia


		Antibodies, Antinuclear Autoantibodies Autoimmune Chronic Hepatitis Cholagogues and Choleretics Females Glucocorticoids Homo sapiens Middle Aged Mitochondria, Liver Thrombocytopenia
12 (42.9%)	22446656	Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome. Carey EJ, Somaratne K, Rakela J. Rev Med Chil. 2011;139(11):1484-7. [ Show abstract ] [ Hide abstract ]
		Purpura Anemia


		Adult Antibodies, Monoclonal, Murine-Derived Aspartate Transaminase Autoimmune Chronic Hepatitis Females Homo sapiens Monoclonal Antibodies Thrombocytopenia
12 (42.9%)	16679706	Autoimmune thrombocytopenic purpura, autoimmune hemolytic anemia and gastric cancer appeared in a patient with myasthenia gravis. Wakata N, Kiyozuka T, Konno S, Nakazora H, Nomoto N, Sugimoto H, Nemoto H. Intern Med. 2006;45(7):479-81. [ Show abstract ] [ Hide abstract ]
		Purpura Anemia


		Carcinoma, Signet Ring Cell Homo sapiens Male Middle Aged Myasthenia Gravis Stomach Neoplasms Thymectomy
12 (42.9%)	16440774	[Basedow disease associated with Evans syndrome]. Kuroda H, Kida M, Watanabe H, Matsunaga T, Niitsu Y, Matsumoto M. Rinsho Ketsueki. 2005;46(10):1118-22. [ Show abstract ] [ Hide abstract ]
		Hyperthyroidism Purpura Anemia
		TSHR

		Combination Drug Therapy Females Graves Disease Homo sapiens Middle Aged Plasma Exchange Pulse Therapy, Drug Syndrome
12 (42.9%)	16061453	[What is your diagnosis? Hypochromic microcytic anemia with Evans syndrome or autoimmune thrombocytopenic purpura?]. Nacoulma EW, Niamba P, Ouedraogo D, Tieno H, Traore R, Ouedraogo C, Drabo J. Sante. 2005;15(2):133-6. [ Show abstract ] [ Hide abstract ]
		Purpura Hypochromic microcytic anemia


		Adrenal Cortex Hormones Differential Diagnosis Diffuse Scleroderma Females Hemorrhage Homo sapiens Middle Aged

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