Evans syndrome

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Evans syndrome
SpecialtyHematology Edit this on Wikidata

Evans syndrome is an autoimmune disease in which an individual's immune system attacks their own red blood cells, white blood cells, and/or platelets.[1][2] These events may occur simultaneously or one may follow on from the other.[3]

Its overall pathology resembles a combination of autoimmune hemolytic anemia and immune thrombocytopenic purpura.[1] Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry oxygen and carbon dioxide are destroyed by an autoimmune process. Immune thrombocytopenic purpura is a condition in which platelets are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of blood clots in the body to prevent bleeding.

The syndrome was first described in 1951 by R. S. Evans and colleagues.[1]

Signs and symptoms[edit]

It has been variously reported that between 10%[4] and 23%[5] of patients who have autoimmune hemolytic anemia, will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.[6]


Although Evans syndrome seems to be a disorder of immune regulation, the exact pathophysiology is unknown. Autoantibodies targeted at different antigenic determinants on red cells and platelets are assumed to cause isolated episodes of hemolytic anemia and thrombocytopenia, respectively.[7]


The diagnosis is made upon blood tests to confirm not only hemolytic anemia and immune thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying cause.[3]

Other antibodies may occur directed against neutrophils and lymphocytes,[8] and "immunopancytopenia" has been suggested as a better term for this syndrome.[9]


Initial treatment is with glucocorticoid corticosteroids or intravenous immunoglobulin, a procedure that is also used in ITP cases.[10][11]  In children, good response to a short steroid course is achieved in approximately 80 percent of cases.[12]  Although the majority of cases initially respond well to treatment, relapses are not uncommon and immunosuppressive drugs (e.g. ciclosporin,[13][14] mycophenolate mofetil, vincristine[15] and danazol[16]) are subsequently used,[3] or combinations of these.[17]

The off-label use of rituximab (trade name Rituxan) has produced some good results in acute and refractory cases,[12][18] although further relapse may occur within a year.[3]  Splenectomy is effective in some cases,[19] but relapses are not uncommon.[20]

The only prospect for a permanent cure is the high-risk option of an allogeneic hematopoietic stem cell transplantation (SCT).[21][22]


In a nationwide study of Evans syndrome the median survival was 7.2 years (primary Evans syndrome: 10.9 years; secondary Evans syndrome: 1.7 years). Secondary Evans syndrome was associated with higher mortality rate than primary Evans syndrome, with a 5-year survival of 38%. Among patients with Evans syndrome, the prevailing causes of death were bleeding, infections, and hematological cancer.[23]

It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia,[24] with recent research finding that 58% of children with Evans syndrome have CD4-/CD8- T cells which is a strong predictor for having autoimmune lymphoproliferative syndrome.[25]


Evans syndrome is considered a very rare autoimmune disease. Only one study has estimated incidence and prevalence. In Denmark in 2016 the annual incidence was 1.8 per 1,000,000 person years, and the prevalence was 21.3 per 1,000,000 living persons.[23]

See also[edit]


  1. ^ a b c Evans RS, Takahashi K, Duane RT, Payne R, Liu C (1951). "Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology". Archives of Internal Medicine. 87 (1): 48–65. doi:10.1001/archinte.1951.03810010058005. PMID 14782741.
  2. ^ "Evans syndrome". Genetic and Rare Diseases Information Center. Retrieved 2018-04-17.
  3. ^ a b c d Norton A, Roberts I (2006). "Management of Evans syndrome". Br. J. Haematol. 132 (2): 125–37. doi:10.1111/j.1365-2141.2005.05809.x. PMID 16398647.
  4. ^ "Evan's syndrome". GPnotebook.
  5. ^ Cai JR, Yu QZ, Zhang FQ (1989). "[Autoimmune hemolytic anemia: clinical analysis of 100 cases]". Zhonghua Nei Ke Za Zhi (in Chinese). 28 (11): 670–3, 701–2. PMID 2632179.
  6. ^ Ng SC (1992). "Evans syndrome: a report on 12 patients". Clinical and Laboratory Haematology. 14 (3): 189–93. doi:10.1111/j.1365-2257.1992.tb00364.x. PMID 1451398.
  8. ^ Pegels JG, Helmerhorst FM, van Leeuwen EF, van de Plas-van Dalen C, Engelfriet CP, von dem Borne AE (1982). "The Evans syndrome: characterization of the responsible autoantibodies". Br. J. Haematol. 51 (3): 445–50. doi:10.1111/j.1365-2141.1982.tb02801.x. PMID 7104228.
  9. ^ Pui CH, Wilimas J, Wang W (1980). "Evans syndrome in childhood". J. Pediatr. 97 (5): 754–8. doi:10.1016/S0022-3476(80)80258-7. PMID 7191890.
  10. ^ Nuss R, Wang W (1987). "Intravenous gamma globulin for thrombocytopenia in children with Evans syndrome". The American Journal of Pediatric Hematology/Oncology. 9 (2): 164–7. doi:10.1097/00043426-198722000-00012. PMID 2438958.
  11. ^ Mehta JB, Singhal SB, Mehta BC (1992). "Intravenous immunoglobulin therapy of idiopathic thrombocytopenia". The Journal of the Association of Physicians of India. 40 (5): 340–2. PMID 1483999.
  12. ^ a b Zecca M; Nobili B; Ramenghi U; et al. (15 May 2003). "Rituximab for the treatment of refractory autoimmune hemolytic anemia in children". Blood. 101 (10): 3857–61. doi:10.1182/blood-2002-11-3547. PMID 12531800.
  13. ^ Emilia G, Messora C, Longo G, Bertesi M (1996). "Long-term salvage treatment by cyclosporin in refractory autoimmune haematological disorders". Br. J. Haematol. 93 (2): 341–4. doi:10.1046/j.1365-2141.1996.4871026.x. PMID 8639426.
  14. ^ Liu H, Shao Z, Jing L (2001). "[The effectiveness of cyclosporin A in the treatment of autoimmune hemolytic anemia and Evans syndrome]". Zhonghua Xue Ye Xue Za Zhi (in Chinese). 22 (11): 581–3. PMID 11855146.
  15. ^ Yokoyama K; Kojima M; Komatsumoto S; et al. (1992). "[Thrombotic thrombocytopenic purpura achieving complete remission by slow infusion of vincristine]". Rinsho Ketsueki (in Japanese). 33 (8): 1084–9. PMID 1404865.
  16. ^ Koike M; Ishiyama T; Saito K; et al. (1993). "[Effective danazol therapy for a patient with Evans syndrome]". Rinsho Ketsueki (in Japanese). 34 (2): 143–6. PMID 8492411.
  17. ^ Scaradavou A, Bussel J (1995). "Evans syndrome. Results of a pilot study utilizing a multiagent treatment protocol". J. Pediatr. Hematol. Oncol. 17 (4): 290–5. doi:10.1097/00043426-199511000-00003. PMID 7583383.
  18. ^ Shanafelt TD, Madueme HL, Wolf RC, Tefferi A (November 2003). "Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome" (PDF). Mayo Clin. Proc. 78 (11): 1340–6. doi:10.4065/78.11.1340. PMID 14601692.
  19. ^ Hamidah A, Thambidorai CR, Jamal R (2005). "Prolonged remission after splenectomy for refractory Evans syndrome--a case report and literature review". Southeast Asian J. Trop. Med. Public Health. 36 (3): 762–4. PMID 16124452.
  20. ^ Mathew P, Chen G, Wang W (1997). "Evans syndrome: results of a national survey". J. Pediatr. Hematol. Oncol. 19 (5): 433–7. doi:10.1097/00043426-199709000-00005. PMID 9329465.
  21. ^ Martino R, Sureda A, Brunet S (1997). "Peripheral blood stem cell mobilization in refractory autoimmune Evans syndrome: a cautionary case report". Bone Marrow Transplant. 20 (6): 521. doi:10.1038/sj.bmt.1700924. PMID 9313889.
  22. ^ Oyama Y, Papadopoulos EB, Miranda M, Traynor AE, Burt RK (2001). "Allogeneic stem cell transplantation for Evans syndrome". Bone Marrow Transplant. 28 (9): 903–5. doi:10.1038/sj.bmt.1703237. PMID 11781654.
  23. ^ a b Hansen DL; Möller S; Andersen K; Gaist D; Frederiksen H (2019). "Evans syndrome in adults - incidence, prevalence, and survival in a nationwide". Am J Hematol. doi:10.1002/ajh.25574. PMID 31292991.
  24. ^ Wang WC (1988). "Evans syndrome in childhood: pathophysiology, clinical course, and treatment". The American Journal of Pediatric Hematology/Oncology. 10 (4): 330–8. doi:10.1097/00043426-198824000-00013. PMID 3071168.
  25. ^ Teachey DT; Manno CS; Axsom KM; et al. (2005). "Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS)". Blood. 105 (6): 2443–8. doi:10.1182/blood-2004-09-3542. PMID 15542578.

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