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Evans syndrome

Evans syndrome
Classification & external resources
ICD-10 D69.3
ICD-9 287.32
DiseasesDB 29724
eMedicine ped/721 

Evans' Syndrome is an autoimmune disease in which an individual's antibodies attack their own RBCs as well as their platelets,[1] both of these events may occur simultaneously or one follow on from the other.[2] Its overall pathology approximates to a combination of the two autoimmune induced conditions: 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 the platelets in the blood 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.

Additional recommended knowledge



Signs and symptoms

It has been variously reported that between 10%[3] and 23%[4] of patients who have autoimmune haemolytic anaemia, will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.[5]


The precise cause is not yet known, but the immunology does differ from patients with just ITP with decreased T4 (T-helper), increased T8 (T-suppressor) and a decreased T4:T8 ratio. Together with decreased serum levels of IgG, IgM, and IgA these findings suggest an unsuccessful autoimmune response to an unknown trigger.[6]


The diagnosis is made upon blood tests to confirm not only haemolytic anaemia and immune thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying aetiology.[2]

Other antibodies may occur directed against neutrophils and lymphocytes,[7] and "immunopancytopenia" has been suggested as a better term for this syndrome.[8]


Initial treatment is with glucocorticoid corticosteroids or intravenous immunoglobulin,[9][10] and particularly in children autoimmune hemolytic anemia is often an acute illness that responds in 80% to a short steroid course.[11]

Although the majority of cases initially respond well, the condition often relapses and immunosuppressive drugs (e.g. ciclosporin,[12][13] mycophenolate mofetil, vincristine[14] and danazol[15]) are then used,[2] or combinations of these.[16]

A drug, Rituxan, has given some good results in acute and refractory Evans syndrome cases,[11][17] although further relapse may occur within a year.[2]

Surgical splenectomy is also used in some cases,[18] but may give only transient benefit in some.[19]

The only prospect for a permanent cure is the high-risk option of an allogeneic hematopoietic stem cell transplantation (SCT).[20][21]


Evan's Syndrome is rare, serious, and has a reported mortality rate of 7%.[citation needed]

It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia,[22] 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.[23]


  1. ^ a b Evans RS, Takahashi K, Duane RT, Payne R, Liu C (1951). "Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology". A.M.A. archives of internal medicine 87 (1): 48-65. PMID 14782741.
  2. ^ 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.
  3. ^ Evan's syndrome at GPnotebook
  4. ^ Cai JR, Yu QZ, Zhang FQ (1989). "[Autoimmune hemolytic anemia: clinical analysis of 100 cases]" (in Chinese). Zhonghua Nei Ke Za Zhi 28 (11): 670–3, 701–2. PMID 2632179.
  5. ^ Ng SC (1992). "Evans syndrome: a report on 12 patients". Clinical and laboratory haematology 14 (3): 189–93. PMID 1451398.
  6. ^ Wang W, Herrod H, Pui CH, Presbury G, Wilimas J (1983). "Immunoregulatory abnormalities in Evans syndrome". Am. J. Hematol. 15 (4): 381–90. PMID 6606357.
  7. ^ 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. PMID 7104228.
  8. ^ Pui CH, Wilimas J, Wang W (1980). "Evans syndrome in childhood". J. Pediatr. 97 (5): 754–8. PMID 7191890.
  9. ^ 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. PMID 2438958.
  10. ^ Mehta JB, Singhal SB, Mehta BC (1992). "Intravenous immunoglobulin therapy of immune thrombocytopenia". The Journal of the Association of Physicians of India 40 (5): 340–2. PMID 1483999.
  11. ^ 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.
  12. ^ 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. PMID 8639426.
  13. ^ Liu H, Shao Z, Jing L (2001). "[The effectiveness of cyclosporin A in the treatment of autoimmune hemolytic anemia and Evans syndrome]" (in Chinese). Zhonghua Xue Ye Xue Za Zhi 22 (11): 581–3. PMID 11855146.
  14. ^ Yokoyama K, Kojima M, Komatsumoto S, et al (1992). "[Thrombotic thrombocytopenic purpura achieving complete remission by slow infusion of vincristine]" (in Japanese). Rinsho Ketsueki 33 (8): 1084–9. PMID 1404865.
  15. ^ Koike M, Ishiyama T, Saito K, et al (1993). "[Effective danazol therapy for a patient with Evans syndrome]" (in Japanese). Rinsho Ketsueki 34 (2): 143–6. PMID 8492411.
  16. ^ 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. PMID 7583383.
  17. ^ Shanafelt TD, Madueme HL, Wolf RC, Tefferi A (Nov 2003). "Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome" (PDF). Mayo Clin. Proc. 78 (11): 1340-6. PMID 14601692.
  18. ^ 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.
  19. ^ Mathew P, Chen G, Wang W (1997). "Evans syndrome: results of a national survey". J. Pediatr. Hematol. Oncol. 19 (5): 433–7. PMID 9329465.
  20. ^ Martino R, Sureda A, Brunet S (1997). "Peripheral blood stem cell mobilization in refractory autoimmune Evans syndrome: a cautionary case report" (PDF). Bone Marrow Transplant. 20 (6): 521. doi:10.1038/sj.bmt.1700924. PMID 9313889.
  21. ^ 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.
  22. ^ Wang WC (1988). "Evans syndrome in childhood: pathophysiology, clinical course, and treatment". The American journal of pediatric hematology/oncology 10 (4): 330–8. PMID 3071168.
  23. ^ 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.


  • Evans syndrome. Madisons Foundation (28 October 2003). Retrieved on 2007-10-29.

acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH

aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia • Hemochromatosis
Coagulation/plateletscoagulopathy: DIC • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease

Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP

primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency - Antiphospholipid syndrome - Factor V Leiden

other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome
HistiocytosisWHO-I Langerhans cell histiocytosis - non-Langerhans-cell histiocytosis/WHO-II (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) - malignant histiocytic disorders/WHO-III (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease)
OtherAsplenia/hyposplenism - Methemoglobinemia
  This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Evans_syndrome". A list of authors is available in Wikipedia.
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