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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.



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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