J Korean Med Sci.  2006 Dec;21(6):1115-1116. 10.3346/jkms.2006.21.6.1115.

A Patient with Mixed Type Evans Syndrome: Efficacy of Rituximab Treatment

• Park CY
• Chung CH

• KMID: 1713130
• DOI: http://doi.org/10.3346/jkms.2006.21.6.1115

Abstract

Mixed type Evans syndrome is a very rare hematologic disease. Although mixed type Evans syndrome may initially respond well to steroids, this disease usually runs a chronic course with intermittent exacerbations. We describe here a 46-yr-old female with the steroid-refractory, mixed type Evans syndrome, and she had a prompt response to rituximab. She was diagnosed as having the mixed type Evans syndrome with the clinical features of symptomatic anemia, jaundice and thrombocytopenia. Prednisone therapy was commenced and her hemoglobin and platelet level returned to the normal. However, after 15 weeks, she relapsed with hemolytic anemia and thrombocytopenia. We started rituximab at the dose of 375 mg/m2 once weekly for a total of 4 doses, which was well-tolerated and this induced the normalization of hemoglobin, bilirubin and lactic dehydrogenase, and there was also a significant increase of the platelet count.

Keyword

Mixed Type Evans Syndrome; Anemia, Hemolytic; Thrombocytopenia; Steroids; rituximab

MeSH Terms

Treatment Outcome
Syndrome
Purpura, Thrombocytopenic, Idiopathic/*drug therapy
Middle Aged
Immunologic Factors/therapeutic use
Humans
Female
Antibodies, Monoclonal/*therapeutic use
Anemia, Hemolytic, Autoimmune/*drug therapy

Figure

• 

  Fig. 1 Schematic representation of the platelet and hemoglobin level in the period from July 2004 until May 2005 (At December 16, 2004 the patient was relapsed). (A) Mixed Evans syndrome was diagnosed and successfully treated with prednisone. (B) After relapse, intravenous infusions of rituximab (375 mg/m2) were given once weekly for 4 consecutive weeks. Until 6 months of rituximab therapy, the patient was stable. Black arrows represent rituximab infusions.

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