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.

Thrombocytopenia

A reduction in the number of circulating thrombocytes.

Total: 34

(per page)

PMID (PMCID)
30656041	OTHER
	Eltrombopag in long-term management of pediatric thrombocytopenia.
	Shah SJ, Etzl MM Jr. Clin Case Rep. 2019;7(1):196-201.
	We have observed rapid, robust, and sustained responses to eltrombopag in two young patients with refractory thrombocytopenia, one with primary immune thrombocytopenia and the other with Evans syndrome.
30223886 (6142628)	MALE
	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.
	At presentation, acquired thrombotic thrombocytopenia purpura in a very young child is commonly misdiagnosed as other conditions like idiopathic thrombocytopenic purpura, Evans syndrome, atypical hemolytic-uremic syndrome, or malignancy.
30631001	FEMALE	Adult
	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.
	One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy.
29531655 (5841940)	OTHER
	Parvovirus B19-triggered Acute Hemolytic Anemia and Thrombocytopenia in a Child with Evans Syndrome.
	Zikidou P, Grapsa A, Bezirgiannidou Z, Chatzimichael A, Mantadakis E. Mediterr J Hematol Infect Dis. 2018;10(1):e2018018.
	We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thrombocytopenia.
29531655 (5841940)	OTHER
	Parvovirus B19-triggered Acute Hemolytic Anemia and Thrombocytopenia in a Child with Evans Syndrome.
	Zikidou P, Grapsa A, Bezirgiannidou Z, Chatzimichael A, Mantadakis E. Mediterr J Hematol Infect Dis. 2018;10(1):e2018018.
	Parvovirus B19-triggered Acute Hemolytic Anemia and Thrombocytopenia in a Child with Evans Syndrome.
27195703	MALE
	Coombs Positive Thrombotic Thrombocytopenic Purpura in a Male Pediatric Patient: An Urgent Diagnostic Challenge.
	Zenno A, Richardson M. Pediatr Blood Cancer. 2016;63(10):1860-2.
	We report a case of an adolescent with thrombocytopenia and Coombs positive anemia, diagnosed with Evans syndrome, but ultimately found to have TTP.
26950085	MALE
	Evans Syndrome Secondary to Common Variable Immune Deficiency.
	Antoon JW, Metropulos D, Joyner BL Jr. J Pediatr Hematol Oncol. 2016;38(3):243-5.
	Evans syndrome is an underdiagnosed condition consisting of simultaneous or sequential combination of autoimmune hemolytic anemia and immune-mediated thrombocytopenia.
28292089 (5325491)	MALE	Middle Aged
	Multiple myeloma associated with an Evan's syndrome.
	Bechir A, Haifa R, Nesrine BS, Emna B, Senda M, Asma A, Amina BB, Mrabet S, Yosra BY, Mondher K, Abderrahim K. Pan Afr Med J. 2016;25:127.
	Here, we report one MM case complicated by Evans syndrome (Autoimmun hemolytic anemia (AIHA) associated with thrombocytopenia).
26309630	OTHER
	The positive therapeutic effect in a patient of Evans syndrome combined with acute myocardial infarction.
	Zhang Y, Zheng K, Zheng M, Liu Q, Meng L. Int J Clin Exp Med. 2015;8(6):9590-4.
	Evans syndrome (ES) is a rare combination of autoimmune hemolytic anemia and thrombocytopenia.
26125124	FEMALE	Adult
	Therapeutic management of evans syndrome in a pregnancy with maternal systemic lupus erythematosus.
	Nause SL, Spiegler J, Weichert J, Hartge DR. Z Geburtshilfe Neonatol. 2015;219(4):190-2.
	Maternal hematological evaluation revealed an Evans-syndrome-related thrombocytopenia based on a lupus erythematosus.
25876786	FEMALE	Middle Aged
	[Successful treatment with rituximab and romiplostim for thrombocytopenia associated with Waldenstrom's macroglobulinemia initially presenting as Evans syndrome].
	Nozaki Y, Koshiishi M, Sueki Y, Kawashima I, Yamamoto T, Nakajima K, Mitsumori T, Kirito K. Rinsho Ketsueki. 2015;56(3):317-22.
	[Successful treatment with rituximab and romiplostim for thrombocytopenia associated with Waldenstrom's macroglobulinemia initially presenting as Evans syndrome].
22472657	FEMALE	Child
	Acute onset altered mental status in a previously healthy teenager.
	Akbary S, Kannikeswaran N. Pediatr Emerg Care. 2012;28(4):376-9.
	Evans syndrome is a rare disease characterized by autoimmune hemolytic anemia and thrombocytopenia.
22446656	FEMALE	Adult
	Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome.
	Carey EJ, Somaratne K, Rakela J. Rev Med Chil. 2011;139(11):1484-7.
	One month later the patient was hospitalized for sudden onset of anemia and thrombocytopenia, suggestive of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura consistent with Evans syndrome.
21719526	MALE	Adult
	The efficacy of romiplostim in the treatment of severe thrombocytopenia associated to Evans syndrome refractory to rituximab.
	Gonzalez-Nieto JA, Martin-Suarez I, Quattrino S, Ortiz-Lopez E, Munoz-Beamud FR, Colchero-Fernandez J, Alcoucer-Diaz MR. Lupus. 2011;20(12):1321-3.
	We present a case of a man diagnosed with systemic lupus erythematosus, associated antiphospholipid syndrome and Evans syndrome, who developed a severe thrombocytopenia refractory to treatment with first-line drugs, cyclophosphamide and rituximab, and who responded to romiplostim with a normalization of the platelet recount, which later enabled a therapeutic splenectomy to be performed.
19398596	MALE	Middle Aged
	Autoimmune pancytopenia following combination chemotherapy for chronic lymphocytic leukaemia.
	MacCallum S, Groves M, Brass D, Cunningham J, Sales M, Gelly K, Tauro S. J Clin Pathol. 2009;62(5):468-70.
	Autoimmune haemolysis or thrombocytopenia can complicate purine nucleoside monotherapy for chronic lymphocytic leukaemia (CLL), but Evans syndrome is rare.
18709985	MALE	Middle Aged
	[Evans syndrome associated with idiopathic mixed-type autoimmune hemolytic anemia].
	Kakinoki Y, Nagase M, Okada K, Chiba K, Fukuhara T, Miyake T. Rinsho Ketsueki. 2008;49(7):516-20.
	Although thrombocytopenia (Evans syndrome) subsequently appeared, corticosteroid was extremely effective for both anemia and thrombocytopenia.
18709985	MALE	Middle Aged
	[Evans syndrome associated with idiopathic mixed-type autoimmune hemolytic anemia].
	Kakinoki Y, Nagase M, Okada K, Chiba K, Fukuhara T, Miyake T. Rinsho Ketsueki. 2008;49(7):516-20.
	Although thrombocytopenia (Evans syndrome) subsequently appeared, corticosteroid was extremely effective for both anemia and thrombocytopenia.
17179698 (2721940)	FEMALE	Middle Aged
	A patient with mixed type Evans syndrome: efficacy of rituximab treatment.
	Park CY, Chung CH. J Korean Med Sci. 2006;21(6):1115-6.
	She was diagnosed as having the mixed type Evans syndrome with the clinical features of symptomatic anemia, jaundice and thrombocytopenia.
16679706	MALE	Middle Aged
	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.
	He was diagnosed as Evans' syndrome (AIHA and AITP) with MG. High-doses of immunoglobulin administration improved the anemia, but thrombocytopenia continued.
17050195	FEMALE	Middle Aged
	Development of mixed-type autoimmune hemolytic anemia and Evans' syndrome following chicken pox infection in a case of low-titer cold agglutinin disease.
	Tanaka Y, Masuya M, Katayama N, Miyata E, Sugimoto Y, Shibasaki T, Yamamura K, Ohishi K, Minami N, Shiku H, Nobori T. Int J Hematol. 2006;84(3):220-3.
	A severe hemolytic crisis and thrombocytopenia (Evans' syndrome) occurred several days after infection with chicken pox, and the patient was referred to our hospital.