Naproxen-Induced Evans Syndrome

Abstract

Evans syndrome is an autoimmune disorder characterized by the simultaneous occurrence of autoimmune hemolytic anemia and immune thrombocytopenic purpura. It can further be classified as primary Evans syndrome when it occurs by itself, or secondary Evans syndrome when it is associated with other autoimmune and lymphoproliferative disorders. Corticosteroids and immunoglobulins are the first-line treatments for primary Evans syndrome, and subsequent options include other immunosuppressive medications. Medical literature provides little information about the triggers of primary Evans syndrome. Knowing such information, however, is essential to recognize, treat and prevent the recurrence of the disease effectively. We report a 68-year-old female who presented with shortness of breath, cough, bruises, scleral icterus, and dark urine after several days of naproxen therapy for pain. Further workup noted direct antiglobulin test positive for IgG, anemia, and thrombocytopenia. Imaging studies showed deep venous thrombosis. She was diagnosed with Evans syndrome and improved following prompt treatment with corticosteroids, anticoagulants, blood transfusion therapies, and discontinuation of naproxen. The prognosis of Evans syndrome is poor, variable, and characterized by relapses. Early diagnosis and treatment are therefore associated with better prognosis. This case is critical because it shines a light on one of the major causes of Evans syndrome, reports a practical approach to treating the condition, and paves the way for future research on Evans syndrome. This case is also the first reported naproxen-induced Evans syndrome in the world's literature.

Keywords: autoimmune hemolytic anemia (aiha); evans’ syndrome; hematology; immune thrombocytopenic purpura; naproxen.

Figure 1. Core biopsy demonstrating hypereosinophilia, and hypercellularity with erythroid and megakaryocytic hyperplasia

Figure 2. Core biopsy demonstrating hypereosinophilia, and hypercellularity with erythroid and megakaryocytic hyperplasia

Figure 3. Peripheral smear showing leukocytosis with hypereosinophilia and erythroblastosis, thrombocytopenia, and macrocytic anemia

Figure 4. Peripheral smear showing leukocytosis with hypereosinophilia and erythroblastosis, thrombocytopenia, and macrocytic anemia

Figure 5. The CTA pulmonary was notable for minimal pulmonary thromboembolism findings with two small adherent filling defects representing thrombi in the right lower lobe pulmonary artery

CTA: Computed tomography angiography