Características clínicas y respuesta al tratamiento en el síndrome de Evans.

BACKGROUND: Evans' syndrome is the association between autoimmune hemolytic anemia and primary immune thrombocytopenia. OBJECTIVES: To describe an Evans' syndrome population's characteristics and its response to treatment. MATERIALS AND METHODS: A retrospective, analytic study including cases diagnosed from January 2008 to December 2018 at Regional Hospital PEMEX, Tamaulipas, Mexico; we evaluated the clinical and biochemical characteristics, as well as their outcomes after treatment. RESULTS: We observed 9 cases with similar clinical and biochemical characteristics, 4 primary and 5 secondary Evans' syndrome. All of them received glucocorticoids as first line of treatment; 6 presented global response; 2 of them complete response, 4 partial response, and 3 no response, regardless the scheme prescribed. An average of 2.78 ± 1.3 lines of treatment were prescribed and four patients required more than three; 3 patients developed systemic autoimmune diseases after mean time of 4.6 years (range: 0.55-9), whose Evans' syndrome had responses after treating the systemic autoimmune disease. CONCLUSIONS: Evans' syndrome is an infrequent entity where an important immunologic imbalance participates and which responses to the first line treatments are torpid with frequent relapses; those patients with more relapses have been associated with the development of systemic autoimmune diseases and most of the cases are secondary, so, we must investigate the background for improving the treatment. [ABSTRACT FROM AUTHOR]