Abstract
Evans Syndrome (ES) is a rare autoimmune disorder that presents with simultaneous or sequential development of autoimmune hemolytic anemia (AIHA), thrombocytopenia, and/or neutropenia. This disease may occur in conjunction with other autoimmune disorders. Primary antiphospholipid syndrome (APS) is a disorder characterized by thrombosis, which can cause life-threatening complications such as fetal demise, strokes, or deep vein thrombosis.A 67-year-old male with type 2 diabetes mellitus, hypertension, and renal insufficiency presented with concomitant ES and APS. His hematological abnormalities began in 2013 after a deep vein thrombosis of the left lower extremity led to a diagnosis of APS and was started on chronic warfarin. In 2014, he was found to have immune thrombocytopenia (ITP) with relapses the following year. Several months later, he was hospitalized and diagnosed with AIHA. In the setting of his previous episodes of ITP and current AIHA, the diagnosis of ES was made. The initial treatment was 100 mg prednisone taper, but rituximab was required to make complete platelet recovery.The severe deterioration and rapid recovery with proper treatment of the patient highlights the importance of a timely diagnosis of ES. Mild thrombocytopenia can be associated with APS; however; severe thrombocytopenia may warrant further investigation for other possible causes. Maintaining ES on the differential diagnosis of patients with APS and thrombocytopenia could enhance health outcomes.
Highlights
Evans syndrome (ES) is a rare autoimmune disorder that presents with simultaneous or sequential development of autoimmune hemolytic anemia (AIHA), thrombocytopenia, or immune-mediated neutropenia [1].Primary antiphospholipid syndrome (APS) is an acquired antibody-mediated disorder characterized by thrombosis causing recurrent fetal demise, stroke, deep vein thrombosis (DVT), or other arterial thrombotic events
Primary APS can be associated with thrombocytopenia and hemolytic anemia, findings required for a diagnosis of ES [2]
Mild thrombocytopenia and hemolytic anemia can be associated with APS, severe thrombocytopenia may warrant further investigation for other possible causes
Summary
Evans syndrome (ES) is a rare autoimmune disorder that presents with simultaneous or sequential development of autoimmune hemolytic anemia (AIHA), thrombocytopenia, or immune-mediated neutropenia [1]. Primary APS can be associated with thrombocytopenia and hemolytic anemia, findings required for a diagnosis of ES [2]. In this case, a diagnosis of ES was initially masked by confirmed APS and thrombocytopenia due to the overlapping presentations of the two syndromes. The patient’s hematological status was further complicated with a diagnosis of immune thrombocytopenia (ITP) after a platelet count of 7 x 109/L and a right lower extremity DVT in 2014; a 40 mg prednisone taper resolved his symptoms. The patient is doing well on warfarin with normal platelet counts and no episodes of thrombosis or anemia
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