Working Diagnosis:
Evans syndrome (combined autoimmune hemolytic anemia and autoimmune thrombocytopenia)

Treatment:
The patient initially underwent treatment with high dose prednisone (80mg daily) for two weeks before eventually requiring RBC and platelet transfusions. After non-response to prednisone, the patient underwent four infusions of rituximab spaced one week apart. The patient was also instructed to take one milligram of folic acid daily.

Outcome:
The patient’s hemoglobin and platelets decreased to 5.9 and 7,000, respectively before transfusions and subsequent initiation of rituximab therapy. After initial rituximab treatment, hemoglobin and platelets increased to 8.4 and 142,000, respectively. Hemoglobin levels gradually increased with subsequent rituximab treatments and platelets remained stable. One month after initial rituximab treatment, platelets (166,000) normalized and continue to be normal (188,000) nine months from initiation of therapy. Four months after initial rituximab therapy, hemoglobin (13.9) normalized and continues to be normal (15.0) nine months later.

After the initiation of rituximab, the patient had weekly laboratory evaluations. After an upward trend of hemoglobin and platelets was confirmed, laboratory evaluation frequency was decreased. Hematology suggested that because of the chronic relapsing nature of Evans syndrome, the patient have follow up every six months with laboratory monitoring every two months for one year after rituximab therapy.

Because platelet counts quickly increased to greater than 50,000, the patient’s activity was only restricted based on symptoms due to anemia. As his anemia improved, the patient was able to gradually increase his amount of activity and eventually returned to full participation in recreational sports.

Editor's Comments:
Teaching Points:
1. Case reports suggest that immunizations may trigger the development of this disease in susceptible individuals. Vaccine history is important (reference#2).
2. Specific physical findings can assist in work-up:
•Signs of thrombocytopenia include purpura, petechiae, and ecchymoses.
•Signs of anemia include pallor, fatigue, and light-headedness.
•Jaundice may indicate hemolysis.
•Features of hemolysis include a raised reticulocyte count, increase in unconjugated bilirubin, and decreased haptoglobins (references # 1 and 3).

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

2. Chen RT, Pless R, Destefano F. Epidemiology of autoimmune reactions induced by vaccination. J Autoimmun. 2001 May;16(3):309–318.

3. Mathew P. Evans Syndrome. Accessed from emedicine on July 1, 2013. http://emedicine.medscape.com/article/955266-overview

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