Korean J Pediatr Hematol Oncol.  2004 Oct;11(2):236-241.

A Case of Evans Syndrome during Valproate Therapy

• Kim E
• Yim SJ
• Choi SR
• Chung SY
• Kang JH
• Jeong DC
• Kim HK

Abstract

We report the first case of immune mediated thrombocytopenia and concurrent hemolytic anemia (Evans syndrome) that developed during valproic acid (VPA) therapy. The 6 years-old girl had been stable with VPA therapy for seizure disorder for 5 years. She was admitted due to purpura and petechiae on both legs. Her blood test was only significant for thrombocytopenia with normal hemoglobin level. Her thrombocytopenia was not caused by VPA toxicity, because of positive anti-platelet antibody. Thrombocytopenia was improved after steroid and intravenous globulin. However, hemoglobin was shown gradual drop with increased reticulocytes counts, high level of lactic dehydrogenase, and a positive direct Coombs test. She had a complete clinical recovery with negative anti-platelet antibody and direct Coombs test after discontinuation of VPA. VPA has a lot of side effects including hematologic toxicity, although this drug is used for seizure disorders. This report is the first Evans syndrome due to VPA, even though VPA induced pure red cell aplasia with positive anti-nuclear antibody or thrombocytopenia by direct toxicity.

Keyword

Evans syndrome; Valproic acid

MeSH Terms

Anemia, Hemolytic
Child
Coombs Test
Epilepsy
Female
Hematologic Tests
Humans
Leg
Oxidoreductases
Purpura
Red-Cell Aplasia, Pure
Reticulocytes
Thrombocytopenia
Valproic Acid*
Oxidoreductases
Valproic Acid