From the Guidelines
Fulminant autoimmune hemolytic anemia (AIHA) is a severe and life-threatening condition that requires immediate medical intervention with high-dose corticosteroids and possibly additional treatments such as intravenous immunoglobulin, rituximab, and plasma exchange. Fulminant AIHA is characterized by the sudden and massive destruction of red blood cells by the body's own immune system, leading to severe anemia, jaundice, fatigue, and potentially organ dysfunction due to inadequate oxygen delivery. Key features of fulminant AIHA include:
- Sudden onset of severe anemia
- Jaundice
- Fatigue
- Laboratory findings such as low hemoglobin, elevated reticulocyte count, increased lactate dehydrogenase, decreased haptoglobin, and a positive direct Coombs test According to the guidelines for the diagnosis and treatment of chronic lymphocytic leukemia, autoimmune hemolytic anemia should be treated with glucocorticoids as the first line of treatment, and second-line treatment options include splenectomy, intravenous immunoglobulins, and/or immunosuppressive therapy with agents such as cyclosporine A, azathioprine, or low-dose cyclophosphamide 1. In cases of fulminant AIHA, the goal of treatment is to rapidly control the hemolysis and prevent complications such as shock and multi-organ failure. Without prompt treatment, fulminant AIHA can lead to severe morbidity and mortality, making rapid diagnosis and aggressive therapy essential.
From the Research
Definition and Classification of Fulminant Autoimmune Hemolytic Anemia
- Fulminant autoimmune hemolytic anemia (AIHA) is a rare and severe form of AIHA, characterized by a rapid onset of life-threatening anemia 2.
- AIHA is a disorder caused by autoantibodies directed against self-red blood cells, which can be idiopathic or secondary, and classified as warm, cold, or mixed, according to the thermal range of the autoantibody 3, 4, 5.
Clinical Presentation and Management
- Fulminant AIHA can present with severe anemia, jaundice, and multiple organ failure, requiring immediate medical attention 2.
- The treatment of AIHA is still not evidence-based, but corticosteroids are the first-line therapy for warm AIHA, with rituximab and splenectomy being considered as second-line treatments for refractory or relapsed cases 3, 5.
- In severe cases of AIHA, parenteral corticosteroids may be used as a rescue treatment, with a higher response rate compared to oral prednisolone 6.
Prognosis and Outcome
- The prognosis of fulminant AIHA is poor, with a high risk of mortality due to multiple organ failure and sepsis 2.
- Early recognition and treatment of severe AIHA are crucial to improve outcomes, and future research efforts are needed to develop prediction models for severe disease course and to identify effective therapeutic approaches 4.