A case report of Chronic Hepatitis B atypically presenting as Secondary Evans Syndrome

Medical Science

Evans Syndrome (ES) is a rare autoimmune disorder defined by Robert Evans in 1951 while studying the association between autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP)[1]. It is usually associated with an idiopathic etiology, but sometimes, an underlying autoimmune condition may trigger ES. ES is characterized by the simultaneous or consecutive presence of warm AIHA and ITP, while autoimmune neutropenia is relatively rare. It shows a positive direct anti-human globulin test. This is a type of disorder wherein one's body produces antibodies against RBCs, WBCs, and platelets. The symptoms can, therefore appear in the form of anemia, low platelet counts, or low white blood cells. In the presence of both AIHA and ITP, always rule out thrombotic microangiopathies, anemia from bleeding associated with ITP, vitamin deficiencies, myelodysplastic syndromes, paroxysmal nocturnal hemoglobinuria, and certain conditions like HELLP (Hemolysis, Elevated Liver Enzymes, and Low Platelets) with special attention to pregnant patients as Evans syndrome is a rare diagnosis of exclusion.[4] ES is a chronic disease that quite often follows a variable clinical course. Spontaneous remissions and exacerbations characterize it. Currently, no conclusive data is available to indicate the worldwide prevalence of the disease; however, certain cohort studies report an incidence of 37%-73% of ES in AIHA. [2,3] Incidence of ES varies from 0.68 to 1.84 per 1000000 people with a one-year period prevalence ranging from 3.30 to 21.30 per 1000000 people.5 It shows female dominance and is predominantly seen in children.[5] To make a diagnosis of ES, a high indicator of suspicion and the exclusion of all the differentials are essential. An accurate diagnosis requires the exclusion of Autoimmune lymphoproliferative syndrome (ALPS), Thrombotic thrombocytopenic purpura (TTP), Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APLA), Sjogren syndrome, Common variable immunodeficiency (CVID), IgA deficiency, Lymphomas and Chronic lymphocytic leukemia (CLL) This case report presents a rare instance of chronic hepatitis B presenting itself as secondary Evans Syndrome.

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