From the Guidelines
Evans syndrome is a rare autoimmune disorder characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), as described in the context of ITP management 1. The clinical course of ITP may also be different depending on whether it is primary ITP or occurs in the setting of additional autoimmune cytopenias, such as Evans syndrome, which is a condition where the immune system mistakenly attacks and destroys red blood cells and platelets 1.
- Key characteristics of Evans syndrome include:
- Simultaneous or sequential development of AIHA and ITP
- Autoimmune destruction of red blood cells and platelets
- Symptoms of anemia, such as fatigue, weakness, and pallor, along with easy bruising, petechiae, and bleeding from thrombocytopenia
- Can occur at any age, but is more common in children
- Treatment of Evans syndrome usually involves immunosuppressive therapy, with the goal of managing symptoms and preventing complications, as outlined in the guidelines for ITP management 1. The treatment approach for Evans syndrome typically involves corticosteroids, such as prednisone, as first-line treatment, with second-line options including rituximab, intravenous immunoglobulin, or immunosuppressants like mycophenolate mofetil or cyclosporine 1.
- The condition is often chronic with periods of remission and relapse, requiring long-term monitoring of blood counts and immunosuppression management, similar to the management approach for ITP 1.
- Evans syndrome may also be associated with other autoimmune conditions or underlying disorders, such as systemic lupus erythematosus or common variable immunodeficiency, which can impact treatment decisions and outcomes 1.
From the FDA Drug Label
8 Hematologic Disorders Idiopathic thrombocytopenic purpura in adults Secondary thrombocytopenia in adults Acquired (autoimmune) hemolytic anemia Erythroblastopenia (RBC anemia) Congenital (erythroid) hypoplastic anemia Evans syndrome is not explicitly mentioned in the provided drug label. However, it is related to Hematologic Disorders, specifically a condition characterized by a combination of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura.
- The label does mention Acquired (autoimmune) hemolytic anemia and Idiopathic thrombocytopenic purpura in adults, which are components of Evans syndrome.
- However, the label does not directly address Evans syndrome as a specific condition. 2
From the Research
Definition of Evans Syndrome
- Evans syndrome (ES) is a rare immune disorder characterized by the simultaneous or sequential occurrence of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) [(3,4,5,6,7)].
- It can also include autoimmune neutropenia (AIN) [(4,5,7)].
Clinical Presentation
- The clinical presentation of ES can include fatigue, pallor, jaundice, and mucosal bleeding 7.
- Patients may experience remissions and exacerbations throughout their lifetime, with acute manifestations such as catastrophic bleeding and massive hemolysis 7.
Diagnosis
- The diagnosis of ES requires the presence of both AIHA and ITP, with a positive direct Coombs test [(3,4,5,6,7)].
- Other differential diagnoses, such as thrombotic microangiopathies, anemia due to bleeding, vitamin deficiencies, myelodysplastic syndromes, and paroxysmal nocturnal hemoglobinuria, must be ruled out 5.
Treatment
- The treatment of ES is mostly empirical, with corticosteroids as the first-line therapy [(4,5,6,7)].
- Second-line treatments include rituximab, splenectomy, and supportive therapies [(4,5,6,7)].
- In cases unresponsive to immunosuppressive agents, hematopoietic stem cell transplantation has been successful 7.