Evans Syndrome Questions & Answers

1. Shaikh H, Mewawalla P. Evans Syndrome. 2018 Jan. [QxMD MEDLINE Link]. [Full Text].

2. Seidel MG. Autoimmune and other cytopenias in primary immunodeficiencies: pathomechanisms, novel differential diagnoses, and treatment. Blood. 2014 Oct 9. 124 (15):2337-44. [QxMD MEDLINE Link]. [Full Text].

3. Jaime-Perez JC, Aguilar-Calderon PE, Salazar-Cavazos L, Gomez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018. 9:171-84. [QxMD MEDLINE Link]. [Full Text].

4. 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. [QxMD MEDLINE Link].

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

6. Mantadakis E, Farmaki E. Natural History, Pathogenesis, and Treatment of Evans Syndrome in Children. J Pediatr Hematol Oncol. 2017 Aug. 39 (6):413-9. [QxMD MEDLINE Link].

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

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

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

10. 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. [QxMD MEDLINE Link].

11. Nakayama H, Kikuchi T, Abe R, et al. [Evans syndrome complicated with multicentric Castleman disease successfully treated with tocilizumab]. Rinsho Ketsueki. 2018. 59 (8):997-1001. [QxMD MEDLINE Link].

12. Muela Molinero A, Ballesteros del Río B, Sandoval Guerra V, Llor Banos J. [Evans syndrome as presentation of multicenter Castleman disease]. Rev Clin Esp. 2003 Dec. 203 (12):616-7. [QxMD MEDLINE Link].

13. Marsh JH, Colbourn DS, Donovan V, Staszewski H. Systemic Castleman's disease in association with Evan's syndrome and vitiligo. Med Pediatr Oncol. 1990. 18 (2):169-72. [QxMD MEDLINE Link].

14. Rothstein H, Pfefferman R, Kusminski G, Hurwitz N, Berrebi A. Evans' syndrome in Castleman's disease: unusual presentation with rectal bleeding from angiodysplasia of cecum. Isr J Med Sci. 1992 Nov. 28 (11):807-9. [QxMD MEDLINE Link].

15. Rivalta B, Zama D, Pancaldi G, et al. Evans Syndrome in Childhood: Long Term Follow-Up and the Evolution in Primary Immunodeficiency or Rheumatological Disease. Front Pediatr. 2019. 7:304. [QxMD MEDLINE Link]. [Full Text].

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

17. Lube GE, Ferriani MP, Campos LM, et al. Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study. Pediatr Blood Cancer. 2016 Jul. 63 (7):1238-43. [QxMD MEDLINE Link].

18. Zhang L, Wu X, Wang L, et al. Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome: A Case-Control, Single Center Study. Medicine (Baltimore). 2016 Apr. 95 (15):e3279. [QxMD MEDLINE Link]. [Full Text].

19. 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. [QxMD MEDLINE Link].

20. 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. [QxMD MEDLINE Link].

21. 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.

22. McLeod AG, Pai M, Carter RF, Squire J, Barr RD. Familial Evans syndrome: a report of an affected sibship. J Pediatr Hematol Oncol. 1999 May-Jun. 21 (3):244-7. [QxMD MEDLINE Link].

23. Ahmed FE, Albakrah MS. Neonatal familial Evans syndrome associated with joint hypermobility and mitral valve regurgitation in three siblings in a Saudi Arab family. Ann Saudi Med. 2009 May-Jun. 29 (3):227-30. [QxMD MEDLINE Link]. [Full Text].

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

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

26. 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. [QxMD MEDLINE Link].

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

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

29. 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. [QxMD MEDLINE Link].

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

31. 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. [QxMD MEDLINE Link].

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

33. 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. [QxMD MEDLINE Link].

34. Vaughn JE, Anwer F, Deeg HJ. Treatment of refractory ITP and Evans syndrome by haematopoietic cell transplantation: is it indicated, and for whom?. Vox Sang. 2016 Jan. 110 (1):5-11. [QxMD MEDLINE Link]. [Full Text].

35. 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. [QxMD MEDLINE Link].

36. 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. [QxMD MEDLINE Link].

37. 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. [QxMD MEDLINE Link].

38. 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. [QxMD MEDLINE Link].

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

40. 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. [QxMD MEDLINE Link].

41. 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. [QxMD MEDLINE Link].

42. 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. [QxMD MEDLINE Link].

43. 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. [QxMD MEDLINE Link].

44. 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]:

45. 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. [QxMD MEDLINE Link].

46. 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.

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

48. Ducassou S, Leverger G, Fernandes H, et al. Benefits of rituximab as a second-line treatment for autoimmune haemolytic anaemia in children: a prospective French cohort study. Br J Haematol. 2017 Jun. 177 (5):751-8. [QxMD MEDLINE Link]. [Full Text].

49. 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.

50. 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. [QxMD MEDLINE Link].

51. 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. [QxMD MEDLINE Link]. [Full Text].

52. Sulpizio ED, Raghunathan V, Shatzel JJ, et al. Long-term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single-center retrospective study. Eur J Haematol. 2020 Jan. 104 (1):55-8. [QxMD MEDLINE Link].

53. Carli G, Visco C, Falisi E, et al. Evans syndrome secondary to chronic lymphocytic leukaemia: presentation, treatment, and outcome. Ann Hematol. 2016 May. 95 (6):863-70. [QxMD MEDLINE Link].

54. Miano M, Ramenghi U, Russo G, et al. Mycophenolate mofetil for the treatment of children with immune thrombocytopenia and Evans syndrome. A retrospective data review from the Italian association of paediatric haematology/oncology. Br J Haematol. 2016 Nov. 175 (3):490-5. [QxMD MEDLINE Link].

Ragini Malika Adams, MD Tashia and John Morgridge Endowed Postdoctoral Fellow, Pediatric Hematology/Oncology and Stem Cell Transplantation, Lucile Packard Children’s Hospital, Stanford University School of Medicine

Ragini Malika Adams, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, Western Society for Pediatric Research

Disclosure: Nothing to disclose.

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, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Michigan State Medical Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

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.

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