The metamorphosis : evans syndrome, a rare presentation of systemic lupus erythematosus / Kristine S. Gamponia-Perona and Mildred T. Rondilla.

Includes appendices and bibliographical references.

Abstract: Evans Syndrome was first reported in 1951 by Evan and Duane, and is defined by the rare development of autoimmune hemolytic anemia and immune thrombocytopenia, whether simultaneous or sequential, with a positive Coombs test. In children, it is one of the rare presentations of autoimmune connective tissue disorders. In this article, we report a fifteen-year old girl with Evans Syndrome, which later evolved into Systemic Lupus Erythematosus. She had pale and sallow skin with multiple ecchymosis and petechial rashes on both upper and lower extremities accompanied by headache, lightheadedness, and generalized body weakness for 3 days. Initial investigation showed moderate normocytic hypochromic anemia with reticulocytosis and thrombocytopenia. Direst and indirect coomb's test was positive. Other bleeding parameters were normal. The peripheral blood smear showed normocytic, hypochromic red blood cells with microcytic red cells, elliptocytes, ovalocytes, polychromatophilic cells, and occasional fragmented red cells, and markedly decreased platelets with occasional giant forms. Bone marrow aspiration was normal. Further investigation revealed a positive Anti-Nuclear Antibody. The patient was initially treated as Evans Syndrome with steroids which controlled the symptoms. She was then worked up for Systemic Lupus Erythematosus, and was treated accordingly.

Research - Department of Child Health