Evans Syndrome Medication

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

Medication Summary

Prednisone (eg, 1-2 mg/kg dose, usually divided twice or three times daily) often effectively controls acute episodes of Evans syndrome. Although some patients are successfully weaned off steroids, relapses may occur when prednisone is tapered or stopped. Use an alternate-day steroid therapy, if possible.

Patients with persistent immune cytopenia and those who require prolonged or high doses of steroids may benefit from intravenous immunoglobulin (IVIG), at doses such as 1-2 g/kg/d for 1-2 days. Their thrombocytopenia is more likely than their hemolysis to respond. Long-term control of thrombocytopenia is reportedly achieved with interval doses of IVIG.

Other therapies reported in a national survey by Mathew et al included immunomodulating agents (eg, cyclosporine, danazol, azathioprine, cyclophosphamide, vincristine).[10] Plasmapheresis was performed in 3 patients. One patient had transient responses, at best, to receiving the immunosuppressive agent tacrolimus (Prograf, FK506), protein A-Sepharose column plasmapheresis, whole blood exchange transfusion, and total nodal irradiation. Another patient showed some response to treatment with a combination of Rh D immunoglobulin (anti-D), methotrexate, prednisone, and folic acid (responses to these agents varied).

One pilot study showed that a multiagent approach may be effective in patients with Evans syndrome[32] . Transfusions of RBCs and platelets were also used as adjuncts to treatment.

One of the newer agents that has been tried in refractory Evans syndrome is rituximab. However, recent reports suggest that it is being used as a second-line therapy. Rituximab is approved for the treatment of relapsed and refractory B-cell non-Hodgkin lymphoma. Binding of rituximab to cells expressing CD20 results in cell death by means of a combination of mechanisms, including antibody-dependent cell cytotoxicity, complement activation and apoptosis. Hence, it has been used in the treatment of various autoimmune disorders mediated by autoantibodies.

Galor et al reported the successful use of rituximab in a patient with Evans syndrome.[22] Mantadakis et al reported its successful use in a patient with long-lasting Evans syndrome refractory to standard treatments; the patient responded well to Rituximab and then responded again when relapse occurred 7 months later.[23] In contrast, Grossi et al treated 2 patients with rituximab without success.[33] In fact, the patients had a worsening of their anemia. Zecca et al described the weekly use of rituximab in 5 children with Evans syndrome, all of whom were successfully treated.[24] In a retrospective review of patients at the Mayo Clinic, Shanafelt reported responses in one of the cytopenias among 3 patients with Evans syndrome, but not both.[25]

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Glucocorticoids

Class Summary

Initial therapy for Evans syndrome. Elicits anti-inflammatory properties and cause profound and varied metabolic effects. They modify the immune response of the body to diverse stimuli.

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Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

 

Commonly used steroid medication that interferes with macrophage Fc gamma and C3b receptors responsible for destruction of red cells and platelets; may work by decreasing abnormal IgG antibody production that may be responsible for destruction of the cells; can increase vascular stability and ameliorate endothelial abnormalities associated with thrombocytopenia; also helps decrease clearance of opsonized platelets.

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Immune Globulin

Class Summary

This is a purified preparation of gamma globulin. It is derived from large pools of human plasma and is composed of 4 subclasses of antibodies, approximating the distribution of human serum.

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Immune globulin intravenous (Carimune NF, Gammar-P, Gammagard, Iveegam EN)

 

Neutralizes circulating myelin antibodies through antiidiotypic antibodies; down regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; may increase CSF IgG (10%).

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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

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