From the Research
Leg swelling in autoimmune hemolytic anemia (AIHA) requires prompt medical evaluation and treatment, focusing on addressing the underlying AIHA with corticosteroids, which should improve both the anemia and associated symptoms including edema. The relationship between AIHA and leg swelling is complex, involving several mechanisms such as hemodynamic changes from anemia causing fluid retention, potential kidney involvement affecting fluid balance, and the hypercoagulable state induced by red blood cell destruction and inflammatory processes 1.
Key Considerations
- AIHA is a rare disease characterized by increased destruction of erythrocytes by autoantibodies, with or without complement activation 1.
- The direct antiglobulin test (DAT) or Coombs test is the cornerstone of AIHA diagnosis, as it is positive with anti-IgG in warm AIHA, and with anti-C3d/IgM antisera plus high titer cold agglutinins in cold agglutinin disease (CAD) 1.
- Treatment of AIHA is quite different depending on the type, with steroids and rituximab being effective in warm AIHA, but having a lower response rate and duration in CAD 1, 2.
- For immediate management of leg swelling, elevation of the legs, compression stockings, and limiting salt intake can help reduce fluid retention, while diuretics like furosemide may be prescribed if edema is significant 3, 4, 5.
Management Approach
- Treatment focuses on addressing the underlying AIHA, with corticosteroids (typically prednisone 1-2 mg/kg/day) being the first-line treatment for warm AIHA, and rituximab or other therapies being considered for refractory or relapsed cases 1, 2.
- If deep vein thrombosis (DVT) is suspected, anticoagulation with low molecular weight heparin or direct oral anticoagulants may be necessary after confirmation with ultrasound 3, 4, 5.
- Patients should monitor for worsening swelling, pain, redness, or shortness of breath, which could indicate progression of thrombosis or other complications 3, 4, 5.
Recent Developments
- New drugs are emerging as treatment options for AIHA, including B-cell-directed therapies, plasma cell-directed therapies, complement inhibitors, and phagocytosis inhibition, providing a new perspective for AIHA therapy 1, 2.
- Sutimlimab, a complement inhibitor, has been approved for the treatment of cold agglutinin disease (CAD) 2.