Evans Syndrome Treatment & Management

  • Author: Prasad Mathew, MBBS, DCh; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Nov 19, 2009
 

Medical Care

  • The management of Evans syndrome is challenging. Although almost all patients require therapy at some time during the course of the disease, the search for a consistent, effective, and nontoxic therapy continues. Medical therapy continues to be the mainstay. Response to therapy varies even within the same individual, and the disease is characterized by periods of remission and exacerbation. No randomized trials have been conducted in patients with Evans syndrome, and the evidence for treatment is based on case reports, case series, and retrospective studies.
  • Prednisone therapy, the most commonly used first-line therapy, often effectively controls acute episodes, although relapses may be frequent when patients are weaned off prednisone.
  • Intravenous immunoglobulin (IVIG) may help patients who depend on steroids.[18, 19]
  • Other therapies effective in small series include danazol, cyclosporine, azathioprine, cyclophosphamide, and vincristine.
  • Recently, rituximab (a chimeric human/mouse monoclonal antibody which targets CD20 on B lymphocytes) has been used in the management of refractory patients.[20, 21, 22, 23, 24, 25] Responses have varied. Norton and Roberts recently reviewed the use of rituximab in 18 patients (aged 0.3-65 y).[26] The results were encouraging, with sustained complete remission of as long as 17 months. In a recent pediatric prospective series by Rao et al, 2 patients with Evans syndrome were treated with rituximab; one had a partial response, and one had no response.[25] Complications associated with rituximab in these studies have been minimal; the most common is infusion-associated reaction.
  • In an acute setting, blood transfusions and/or platelet transfusions may be required to alleviate symptoms, although their use should be minimized.
  • Additional therapies include splenectomy, alemtuzumab administration, and hematopoietic stem cell transplantation.
    • The role of splenectomy is not clearly established, but it may improve blood counts and reduce steroid dependence; relapses are common and, in most cases, occur within 1-2 months postsplenectomy.
    • Children with Evans syndrome appear to have a higher risk of postsplenectomy sepsis, especially children with pancytopenia.
    • Autologous and allogeneic stem cell transplantation have been used in a small number of patients (14 patients aged 5-52 y), with mixed results.[27]
    • Alemtuzumab is a humanized IgG monoclonal antibody specific for the CD52 antigen present on T cells and B cells. Willis et al reported its use in 3 patients with Evans syndrome; although a response was seen in 2 patients, both relapsed in 3 months.[28]
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Surgical Care

Consider splenectomy in refractory cases.

  • Splenectomy may improve CBC counts and decrease the steroid requirement, although relapses are common.
  • According to a national survey, splenectomy provided a reported duration of response that ranged from 1 week to 5 years; however, the median response duration was just one month.[10]
  • The risk of infection after splenectomy appears to be increased in children with pancytopenia.
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Consultations

  • Pediatric hematologist
  • Pediatric oncologist
  • Rheumatologist
  • Immunologist
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Diet

  • Dietary restrictions are not usually required.
  • Patients receiving steroid therapy should have some restrictions on their salt, sugar, and fluid intake to prevent excessive fluid retention.
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Activity

  • Restrict activities based on patient tolerance and the degree of anemia and bruising.
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Proceed to Medication
 
 
Contributor Information and Disclosures
Author

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

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 Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Gary D Crouch, MD  Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, 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

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  14. 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.

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