Evans Syndrome – The diagnosis of exclusion

  • Dr. Rutvi Dave, Dave G
Keywords: Evans Syndrome, autoimmune cytopenias, autoimmune thrombocytopenia, hemolytic anemia, immunosupression

Abstract

Evans Syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune
neutropenia. We present a case of woman aged 65 years presenting with Evans Syndrome. This is an uncommon condition where patient presents with combination (either simultaneously or sequentially)
of immune thrombocytopenia (ITP) and autoimmune hemolytic anaemia (AIHA) with a positive direct antiglobulin test (DAT) with the etiology remaining unknown. This condition generally runs a chronic
course and needs life long followup and vigilance. It is characterised by frequent exacerbations and remissions. No sex predilection is known and Evans Syndrome has been described in all ethnic
groups and at all ages. Due to hemolytic anemia and thrombocytopenia with recurrence there is more morbidity. The mortality results in severe cases especially in elderly individuals due to heart failure.
First-line therapy is usually corticosteroids .

References

1. Alice Norton, Irene Roberts, Management of Evans syndrome, British Journal of Haematology,2005 132,, 125–137
2. Evans, R.S., Takahashi, K. & Duane, R.T. (1951) Primary thrombocytopenic purpura and acquired hemolytic anemia. Archives of Internal Medicine, 87, 48–65.
3. Marc Michel,Valérie Chanet, Agnès Dechartres et al, The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases, Blood 2009, October 8, vol. 114 no. 15 3167- 3172
4. Evans RS,Duane RT, Acquired hemolytic anemia; the relation of erythrocyte antibody production to activity of the disease; the significance of thrombocytopenia and leukopenia. Blood1949;4(11):1196-1213.
5. Delezé M, Oria CV, Alarcon-Segovia D. Occurrence of both hemolytic anemia and thrombocytopenic purpura (Evans’ syndrome) in systemic lupus erythematosus. Relationship to antiphospholipid antibodies. J Rheumatol 1988;15(4):611-615.
6. Norton A, Roberts I,. Management of Evans syndrome. Br J Haematol 2005;132(2):125-137.
7. Ng SC, Evans syndrome: a report on 12 patients. Clin Lab Haemat 1992;14(3):189-193.
Published
2019-09-26
How to Cite
Dr. Rutvi Dave, Dave G. (2019). Evans Syndrome – The diagnosis of exclusion. The Indian Practitioner, 67(4), 235-238. Retrieved from https://articles.theindianpractitioner.com/index.php/tip/article/view/845