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Evans Syndrome Differential Diagnoses

  • Author: Prasad Mathew, MBBS, DCH, FAAP; Chief Editor: Hassan M Yaish, MD  more...
 
Updated: Apr 11, 2016
 
 

Diagnostic Considerations

Evans syndrome may not be apparent when a child presents with an isolated cytopenia. Therefore, diagnosis may be delayed until a second episode of cytopenia manifests.

Evans syndrome is a diagnosis of exclusion. Other causes of immune cytopenias must therefore be ruled out before the diagnosis can be made. In addition to the conditions listed in the differential diagnosis, other problems to be considered include the following:

  • Immunoglobulin A (IgA) deficiency
  • AIDS
  • Autoimmune lymphoproliferative syndrome (ALPS)
  • Paroxysmal nocturnal hemoglobinuria
  • Thrombotic thrombocytopenic purpura
  • Hypersplenism
  • Rosai-Dorfman disease (may be associated with ALPS)

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Prasad Mathew, MBBS, DCH, FAAP Professor of Pediatrics, Division of Hematology/Oncology, University of New Mexico School of Medicine

Prasad Mathew, MBBS, DCH, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society on Thrombosis and Haemostasis, American Society of Clinical Oncology, National Hemophilia Foundation, Hemophilia and Thrombosis Research Society, International Society of Paediatric Oncology, World Federation of Hemophilia

Disclosure: Received salary from Bayer HC for payment for services rendered.

Chief Editor

Hassan M Yaish, MD Medical Director, Intermountain Hemophilia and Thrombophilia Treatment Center; Professor of Pediatrics, University of Utah School of Medicine; Director of Hematology, Pediatric Hematologist/Oncologist, Department of Pediatrics, Primary Children's Medical Center

Hassan M Yaish, MD is a member of the following medical societies: American Academy of Pediatrics, New York Academy of Sciences, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Michigan State Medical Society

Disclosure: Nothing to disclose.

References
  1. Jaime-Perez JC, Guerra-Leal LN, Lopez-Razo ON, Mendez-Ramirez N, Gomez-Almaguer D. Experience with Evans syndrome in an academic referral center. Rev Bras Hematol Hemoter. 2015 Jul-Aug. 37 (4):230-5. [Medline].

  2. Evans RS, Takahashi K, Duane RT. Primary thrombocytopenic purpura and acquired hemolytic anemia. Arch Intern Med. 1951. 87:48-65.

  3. Wang W, Herrod H, Pui CH. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol. 1983 Dec. 15(4):381-90. [Medline].

  4. Wang WC. Evans syndrome in childhood: pathophysiology, clinical course, and treatment. Am J Pediatr Hematol Oncol. 1988 Winter. 10(4):330-8. [Medline].

  5. Savasan S, Warrier I, Ravindranath Y. The spectrum of Evans' syndrome. Arch Dis Child. 1997 Sep. 77(3):245-8. [Medline].

  6. Teachey DT, Manno CS, Axsom KM, et al. Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). Blood. 2005 Mar 15. 105(6):2443-8. [Medline].

  7. Costallat GL, Appenzeller S, Costallat LT. Evans syndrome and Systemic Lupus Erythematosus: Clinical presentation and outcome. Joint Bone Spine. 2011 Sep 22. [Medline].

  8. Franklin VL, Torrance T, Peebles M, Wilkie R, Greene S. Life-threatening autoimmunity with diabetes: management with an insulin pump. Pediatr Diabetes. 2003 Sep. 4(3):151-4. [Medline].

  9. Keung YK, Cobos E, Bolanos-Meade J, et al. Evans syndrome after autologous bone marrow transplant for recurrent Hodgkin's disease. Bone Marrow Transplant. 1997 Dec. 20(12):1099-101. [Medline].

  10. Yarali, Nese; Demirceken, Fulya; Kondolat, Meda; Ozkasap, Serdar; Kara, Abdurrahman; Tunc, et al. A Rare Condition Associated With Celiac Disease: Evans Syndrome. Journal of Pediatric Hematology/Oncology. September 2007. 29 (9):633-635.

  11. Pirofsky B. Immune haemolytic disease: the autoimmune haemolytic anaemias. Clin Haematol. Feb 1975. 4(1):167-89.

  12. Pui CH, Wilimas J, Wang W. Evans syndrome in childhood. J Pediatr. 1980 Nov. 97(5):754-8. [Medline].

  13. Habibi B, Homberg JC, Schaison G, Salmon C. Autoimmune hemolytic anemia in children. A review of 80 cases. Am J Med. 1974 Jan. 56(1):61-9. [Medline].

  14. Ng SC. Evans syndrome: a report on 12 patients. Clin Lab Haematol. 1992. 14(3):189-93. [Medline].

  15. Mathew P, Chen G, Wang W. Evans syndrome: results of a national survey. J Pediatr Hematol Oncol. 1997 Sep-Oct. 19(5):433-7. [Medline].

  16. Genty I, Michel M, Hermine O, Schaeffer A, Godeau B, Rochant H. [Characteristics of autoimmune hemolytic anemia in adults: retrospective analysis of 83 cases]. Rev Med Interne. 2002 Nov. 23(11):901-9. [Medline].

  17. Fagiolo E. Platelet and leukocyte antibodies in autoimmune hemolytic anemia. Acta Haematol. 1976. 56(2):97-106. [Medline].

  18. Kakaiya RM, Sherman LA, Miller WV, Katz AJ. Nature of platelet antibody in Evans syndrome: a case report. Ann Clin Lab Sci. 1981 Nov-Dec. 11(6):511-5. [Medline].

  19. Pegels JG, Helmerhorst FM, van Leeuwen EF. The Evans syndrome: characterization of the responsible autoantibodies. Br J Haematol. 1982 Jul. 51(3):445-50. [Medline].

  20. Fan J, He H, Zhao W, et al. Clinical Features and Treatment Outcomes of Childhood Autoimmune Hemolytic Anemia: A Retrospective Analysis of 68 Cases. J Pediatr Hematol Oncol. 2016 Mar. 38 (2):e50-5. [Medline].

  21. Raetz E, Beatty PG, Adams RH. Treatment of severe Evans syndrome with an allogeneic cord blood transplant. Bone Marrow Transplant. 1997 Sep. 20(5):427-9. [Medline].

  22. Blanchette VS, Kirby MA, Turner C. Role of intravenous immunoglobulin G in autoimmune hematologic disorders. Semin Hematol. 1992 Jul. immunology(3 Suppl 2):72-82. [Medline].

  23. Nuss R, Wang W. Intravenous gamma globulin for thrombocytopenia in children with Evans syndrome. Am J Pediatr Hematol Oncol. 1987 Summer. 9(2):164-7. [Medline].

  24. Janiä D, Krivokapiä-Dokmanoviä L, Jovanoviä N, Laziä J, Rodiä P, Jankoviä S. Glucocorticoid-resistant Evans' syndrome successfully controlled with low-dose cyclosporine. Int J Clin Pharmacol Ther. 2011 Oct. 49(10):622-5. [Medline].

  25. Galor A, O'Brien T. Rituximab treatment for relapsed autoimmune hemolytic anemia in Evans syndrome. Int J Hematol. 2003 Nov. 78(4):335-6. [Medline].

  26. Mantadakis E, Danilatou V, Stiakaki E, Kalmanti M. Rituximab for refractory Evans syndrome and other immune-mediated hematologic diseases. Am J Hematol. 2004 Nov. 77(3):303-10. [Medline].

  27. Zecca M, Nobili B, Ramenghi U, et al. Rituximab for the treatment of refractory autoimmune hemolytic anemia in children. Blood. 2003 May 15. 101(10):3857-61. [Medline].

  28. Shanafelt TD, Madueme HL, Wolf RC, Tefferi A. Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome. Mayo Clin Proc. 2003 Nov. 78(11):1340-6. [Medline].

  29. Maloney DG, Grillo-Lopez AJ, White CA, et al. IDEC-C2B8 (Rituximab) anti-CD20 monoclonal antibody therapy in patients with relapsed low-grade non-Hodgkin's lymphoma. Blood. 1997 Sep 15. 90(6):2188-95. [Medline].

  30. Rao A, Kelly M, Musselman M, et al. Safety, efficacy, and immune reconstitution after rituximab therapy in pediatric patients with chronic or refractory hematologic autoimmune cytopenias. Pediatr Blood Cancer. Jun 14, 2007. [Epub ahead of print]:

  31. Kashif M, Qureshi A, Adil SN, Khurshid M. Successful use of rituximab in Evans syndrome and refractory immune thrombocytopenic purpura. J Pak Med Assoc. 2010 Jan. 60(1):64-5. [Medline].

  32. Grossi A, Santini V, Longo G, et al. Treatment with anti CD20 antibodies of patients with autoimmune thrombocytopenia with or without hemolytic anemia: worsening in hemoglobin level [abstract]. Blood. 2000. 96:1089A.

  33. Norton A, Roberts I. Management of Evans syndrome. Br J Haematol. 2006. 132:125-137.

  34. Willis F, Marsh JCW, Bevan DH, et al. The effect of treatment with Campath-1H in patients with autoimmune cytopenias. Br J Haematol. 2001. 114:891-898.

  35. Scaradavou A, Bussel J. Evans syndrome. Results of a pilot study utilizing a multiagent treatment protocol. J Pediatr Hematol Oncol. 1995 Nov. 17(4):290-5. [Medline].

  36. Aladjidi N, Fernandes H, Leblanc T, et al. Evans Syndrome in Children: Long-Term Outcome in a Prospective French National Observational Cohort. Front Pediatr. 2015. 3:79. [Medline]. [Full Text].

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