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.

Purpura

Purpura (from Latin: purpura, meaning \"purple\") is the appearance of red or purple discolorations on the skin that do not blanch on applying pressure. They are caused by bleeding underneath the skin. This term refers to an abnormally increased susceptibility to developing purpura. Purpura are larger than petechiae.


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(per page)
PMID (PMCID)
30733882
 OTHER
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.
A Case of Pregnancy Complicated with Evans Syndrome with Sequential Development of Autoimmune Warm Antibody Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura.
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.
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.
30620809
 FEMALE
[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.
Differential diagnosis for anemia revealed that she had newly developed Evans syndrome, which is the complication of autoimmune hemolytic ane- mia (AIHA) and idiopathic thrombocytopenic purpura(ITP).
26150183
 FEMALE Adult
A Case of a TSH-secreting Pituitary Adenoma Associated with Evans' Syndrome.
Yasuda A, Seki T, Oki M, Takagi A, Inomoto C, Nakamura N, Atsumi H, Baba T, Matsumae M, Sasaki N, Suzuki Y, Fukagawa M.
Tokai J Exp Clin Med. 2015;40(2):44-50.
Evans' syndrome was diagnosed based on idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia.
24881920
 FEMALE
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.
One month later, her PRCA went into remission following treatment but she developed idiopathic thrombocytopenic purpura and was diagnosed with Evans syndrome.
22796025
 MALE Middle Aged
A case of Evans' syndrome following influenza vaccine.
Shlamovitz GZ, Johar S.
J Emerg Med. 2013;44(2):e149-51.
Evans' syndrome is an uncommon condition defined by the combination (either simultaneously or sequentially) of immune thrombocytopenia purpura and autoimmune hemolytic anemia with a positive direct antiglobulin test in the absence of known underlying etiology.
23801132
 MALE
CD56+ angioimmunoblastic T-cell lymphoma with evans syndrome : a case report and review of the literature.
Sekiguchi Y, Shimada A, Imai H, Wakabayashi M, Sugimoto K, Nakamura N, Sawada T, Komatsu N, Noguchi M.
J Clin Exp Hematop. 2013;53(1):37-47.
A 67-year-old man was diagnosed with CD56(+) angioimmunoblastic T cell lymphoma (AITL), which was associated with autoimmune thrombocytopenic purpura (ATP) and autoimmune hemolytic anemia (AIHA) (Evans syndrome).
23804021
 FEMALE
Microsurgical reconstruction of the mandible in a patient with evans syndrome: a case report and review of the literature.
Bedogni A, Anesi A, Fior A, Bettini G, Nocini PF.
J Reconstr Microsurg. 2013;29(8):545-50.
In this report, we describe the first successful case of microvascular free tissue transfer in a patient with Evans Syndrome (ES), a rare form of idiopathic thrombocytopenic purpura (ITP) and associated autoimmune hemolytic anemia (AIHA).
23321502
 FEMALE Middle Aged
Simultaneous romiplostin, eltrombopag, and prednisone were successful in severe thrombocytopenia of Evans syndrome refractory to hydrocortisone, splenectomy, intravenous IgG, and rituximab.
Ruiz-Arguelles GJ, Ruiz-Delgado GJ, Velazquez-Sanchez-de-Cima S, Zamora-Ortiz G.
Hematology. 2013;18(3):175-7.
A 58-year-old woman presented with rheumatoid arthritis-associated Evans syndrome (simultaneous autoimmune hemolytic anemia and autoimmune thrombocytopenic purpura); she was treated unsuccessfully with steroids, romiplostin, rituximab, immunoglobulin G, and splenectomy.
23603626
 FEMALE Adult
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.
Evans Syndrome (ES) is the rare simultaneous or subsequent development of immune thrombocytopenia purpura (ITP) and autoimmune hemolytic anemia (AIHA).
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.
21397466
 MIXED_SAMPLE Child
[Hodgkin disease and autoimmunity in children: 11 case reports].
Jarrasse C, Pagnier A, Edan C, Landman-Parker J, Mazingue F, Mansuy L, Bertrand Y, Paillard C, Pellier I, Margueritte G, Plantaz D.
Arch Pediatr. 2011;18(4):376-82.
Four patients had thrombocytopenic purpura, the remaining 7 each had a different autoimmune pathology: lupus syndrome, antiphospholipid syndrome with transient ischemic attack, Evans syndrome, leukocytoclastic vasculitis, autoimmune hemolytic anemia, autoimmune thyroiditis, and juvenile idiopathic arthritis.
18799944
 MALE
Pseudohypoparathyroidism Ia with Evans syndrome.
Pavone P, Pratico AD, Castellano-Chiodo D, Ruggieri M.
J Pediatr Hematol Oncol. 2008;30(8):628-30.
We report on a child with a classic form of pseudohypoparathyroidism and associated Albright syndrome who developed Evans syndrome (ie, the cooccurrence of severe autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura).
16807996
(2688166)
 FEMALE Adult
A 20-year-old woman with Hashimoto's thyroiditis and Evans' syndrome.
Kang MY, Hahm JR, Jung TS, Lee GW, Kim DR, Park MH.
Yonsei Med J. 2006;47(3):432-6.
Two years and five months later, she developed idiopathic thrombocytopenic purpura (ITP) and was diagnosed with Evans' syndrome.
16440774
 FEMALE Middle Aged
[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.
Based on a positive direct Coombs test and an elevated level of platelet-associated IgG (PAIgG), the patient was diagnosed as having autoimmune hemolytic anemia (AIHA) associated with idiopathic thrombocytopenic purpura (ITP), i.e., Evans syndrome.
16061453
 FEMALE Middle Aged
[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.
Hypochromic microcytic anemia with Evans syndrome or autoimmune thrombocytopenic purpura?
11175594
 FEMALE Adult
[Brain abscess caused by Nocardia sp in immunosuppressed patient].
Barata CH, Oliveira DA, Colombo AL, Pereira CA.
Rev Soc Bras Med Trop. 2000;33(6):609-12.
Patient with autoimmune haemolytic anaemia and thrombocytopenic purpura (Evans Syndrome), treated with immunosuppressive therapy (prednisone and azathioprine) developed brain abscess unresponsive to antimicrobial therapy, in spite of its 23 days duration.
11957294
 MALE Child
[Immunologic thrombopenic purpura associated with an autoimmune hemolytic anemia or Fisher-Evans syndrome: apropos of a case].
Ndiaye O, Sylla A, Ndiaye FS, Diagne I, Diallo-Chauvin M, Diouf S, Sallm G, Kuakuvi K.
Dakar Med. 1999;44(2):240-2.
We report one case of thrombocytopaenia purpura associated with an auto-immune haemolytic anaemia (positive erythrocyte Coombs test) or Fisher-Evans syndrome, found in a child aged 6 and a half years.
11957294
 MALE Child
[Immunologic thrombopenic purpura associated with an autoimmune hemolytic anemia or Fisher-Evans syndrome: apropos of a case].
Ndiaye O, Sylla A, Ndiaye FS, Diagne I, Diallo-Chauvin M, Diouf S, Sallm G, Kuakuvi K.
Dakar Med. 1999;44(2):240-2.
[Immunologic thrombopenic purpura associated with an autoimmune hemolytic anemia or Fisher-Evans syndrome: apropos of a case].