Abstract
CASE REPORT ON EVANS SYNDROME ASSOCIATED WITH SJOGRENS SYNDROME

Judy James*, Gopika KS, Tonya Clara Thomas, Dhivya Jose, Smitha Mariyam Thomas and Fathimath Dilsha

ABSTRACT

Evans syndrome is a rare and chronic autoimmunedisorder which is characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. It is mainly classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37-73%. People affected with evans syndrome often experience thrombocytopenia and Coomb’s positive hemolytic anemia. The pathophysiology of this disease include deficiencies of CTLA-4, LRBA, and a decreased CD4/CD8 ratio. It is usually treated with corticosteroids and intraveneous IgG. In refractory cases, cyclosporine, rituximab, azathioprine, cyclophosphamide and mycofenolate mofetil have been used. Here we report a case report of Secondary Evans syndrome associated Sjogrens syndrome presented with fever, yellowish discolouration of eyes and shortness of breath. Hence the patient is treated with steroids, calcium supplements, antibiotics. Blood and platelets transfusion is the treatment given to improve symptoms and gain time but its use should be minimized.

Keywords: Evans syndrome, Sjogren syndrome, Thrombocytopenic purpura, Coomb’s test, IVIG(Intraveneous immunoglobulin).


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