Evans Syndrome Follow-up

  • Author: Prasad Mathew, MBBS, DCh; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Mar 12, 2012
 

Further Inpatient Care

  • Patients admitted for severe anemia or thrombocytopenia should have respiratory and cardiovascular functions stabilized first; receive blood transfusions, if needed, after consultation with a pediatric hematologist; and then be started on therapy with either intravenous immunoglobulin (IVIG) or steroids.
  • In classic cases (ie, patients who present with either autoimmune hemolytic anemia [AIHA] or immune thrombocytopenia), bone marrow aspiration is not indicated.
    • When a patient has pancytopenia at presentation, or when diagnosis of immune thrombocytopenia is unconfirmed, bone marrow aspiration findings may help exclude an infiltrative pathology or an aplastic marrow.
    • Bone marrow examination may be indicated in unusual cases, in cases refractory to treatment, or in cases when a peripheral blood smear suggests immature myeloid cells.
  • A patient may be discharged if clinically stable (eg, with rising blood counts).
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Further Outpatient Care

  • Weekly follow-up is recommended with blood counts and physical examinations until counts become stable or return to reference ranges.
  • Subsequent follow-up care may be arranged at intervals of 2-4 weeks.
  • More frequent follow-up care may be indicated for patients with clinical or laboratory signs of recurrence (eg, when steroids are tapered).
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Complications

  • A risk of hemorrhage with severe thrombocytopenia is noted.
    • The national survey reported hemorrhage in 29% of patients.[10]
    • This study reported 2 patient deaths from severe GI bleeding and a third death from an acute intracranial bleed.
  • Patients with neutropenia also risk serious infection.
    • The national survey showed invasive infections in 29% of patients, including pneumonia, sepsis, and meningitis with Streptococcus pneumoniae, localized abscess, and osteomyelitis.
    • One patient died from presumed sepsis and liver failure 9 years after splenectomy.
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Prognosis

  • The characteristic clinical course of Evans syndrome has periods of remission and exacerbation, with variable and often disappointing responses to therapy.
  • Recurrences of thrombocytopenia and anemia are common, as are episodes of hemorrhage and serious infections. Evans syndrome sometimes is fatal.
  • Treatment occasionally provides complete resolution. In the national study on Evans syndrome, after a median follow-up of 3 years (range, 4 mo to 18.9 y), 48% had active disease and continued to be treated, 12% had persistent disease but were receiving no treatment, and 33% had no evidence of disease for periods ranging from 1.5-5 years (median, 1 y).[10] Long-term survival data are limited. In patients followed for a median range of 3-8 years, the mortality ranged from 7-36%. Causes of death were mainly due to hemorrhage or sepsis. None of these patients developed any malignancy.
  • Patients rarely do well without treatment.
    • In the national survey, each patient received a median of 5 (range, 1-12) treatment modalities, either in combination or sequentially.[10]
    • Only one patient received no treatment; this patient's hemoglobin levels were 9-13.2 g/dL and platelet counts were 9-208,000/µL during follow-up examinations over 11 years.
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Patient Education

  • Educate patients and their families about the chronic nature of this condition, which can include periods of remission and exacerbation.
  • Explain potential adverse effects of medications, especially long-term steroids, each time a steroid is used to treat an exacerbation.
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Contributor Information and Disclosures
Author

Prasad Mathew, MBBS, DCh  Director, Hemostasis and Hematology Program, Professor of Clinical Pediatrics, University of New Mexico School of Medicine

Prasad Mathew, MBBS, DCh is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Gary R Jones, MD  Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Gary D Crouch, MD  Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

References

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

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

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

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

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

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

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

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

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

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

  11. 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. Nov 2002;23(11):901-9. [Medline].

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

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

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

  15. 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.

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

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

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

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

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

  21. 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. Oct 2011;49(10):622-5. [Medline].

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

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

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

  25. 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. Nov 2003;78(11):1340-6. [Medline].

  26. 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. Sep 15 1997;90(6):2188-95. [Medline].

  27. 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].

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

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

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

  31. 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.

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

  33. 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.

  34. Avcin T, Jazbec J, Kuhar M, et al. Evans syndrome associated with antiphospholipid antibodies. J Pediatr Hematol Oncol. Sep 2003;25(9):755-6. [Medline].

  35. Chen RT, Pless R, DeStafano F. Epidemiology of autoimmune reactions induced by vaccinations. J Autoimmunity. 2001;16:309-318.

  36. Conley ME, Park CL, Douglas SD. Childhood common variable immunodeficiency with autoimmune disease. J Pediatr. Jun 1986;108(6):915-22. [Medline].

  37. Miller BA, Schultz Beardsley D. Autoimmune pancytopenia of childhood associated with multisystem disease manifestations. J Pediatr. Dec 1983;103(6):877-81. [Medline].

  38. Petitt RM. Thrombotic thrombocytopenic purpura: a thirty year review. Semin Thromb Hemost. 1980;6(4):350-5. [Medline].

  39. Rosse WF. Paroxysmal nocturnal hemoglobinuria: the biochemical defects and the clinical syndrome. Blood Rev. Sep 1989;3(3):192-200. [Medline].

  40. Sneller MC, Wang J, Dale JK, et al. Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis. Blood. Feb 15 1997;89(4):1341-8. [Medline].

  41. Tsudo M, Moriguchi T. [Effective cyclosporine therapy to resistant-Evans syndrome]. Rinsho Ketsueki. Nov 1996;37(11):1289-92. [Medline].

 
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