From the Guidelines
Systemic Lupus Erythematosus (SLE) is commonly associated with several hematological disorders, including thrombocytopenia, autoimmune hemolytic anemia, leukopenia, and thrombotic disorders, as reported in the 2019 update of the EULAR recommendations for the management of SLE 1.
Hematological Manifestations
The most frequent hematological manifestations of SLE include:
- Thrombocytopenia, which occurs in about 30% of patients due to antiplatelet antibodies or increased platelet consumption
- Autoimmune hemolytic anemia, resulting from autoantibodies targeting red blood cells
- Leukopenia, especially lymphopenia with counts below 1,500 cells/μL, affecting approximately 50% of SLE patients and increasing infection risk
- Antiphospholipid syndrome, characterized by arterial and venous thrombosis, present in 15-20% of SLE patients due to antiphospholipid antibodies interfering with clotting mechanisms
Treatment and Management
These hematological abnormalities often correlate with disease activity and may require specific treatments beyond standard SLE management, including:
- Corticosteroids
- Immunosuppressants
- Rituximab or intravenous immunoglobulin therapy in severe cases
- Anticoagulation therapy for patients with antiphospholipid syndrome, as recommended in the KDIGO 2024 clinical practice guideline for the management of lupus nephritis 1
Recent Guidelines and Recommendations
The 2019 update of the EULAR recommendations for the management of SLE provides guidance on the treatment of hematological manifestations, including the use of glucocorticoids, immunosuppressive agents, and biologics 1. The KDIGO 2024 clinical practice guideline for the management of lupus nephritis also provides recommendations for the management of thrombotic microangiopathy in patients with lupus nephritis, including the use of plasma exchange, glucocorticoids, and rituximab 1.
From the Research
Hematological Disorders Associated with Systemic Lupus Erythematosus (SLE)
The following hematological disorders are associated with SLE:
- Anemia 2, 3, 4, 5, 6
- Leukopenia 2, 3, 4, 5, 6
- Thrombocytopenia 2, 3, 4, 5, 6
- Pancytopenia 5
- Hemolytic anemia 3, 4, 5, 6
- Immune-mediated bone marrow failure 2
- Excessive peripheral cell destruction 2
- Autoimmune hemolytic anemia 5
- Idiopathic thrombocytopenic purpura 5
- Evans' syndrome 5
Pathophysiology and Diagnostic Approaches
The pathophysiology of hematological disorders in SLE is complex and involves immune-mediated mechanisms, bone marrow failure, and peripheral cell destruction 2, 3, 4, 5, 6. Diagnostic approaches include laboratory tests, such as complete blood counts, bone marrow examinations, and serological tests for autoantibodies 2, 3, 4, 5, 6.
Management Strategies
Management strategies for hematological disorders in SLE include:
- Corticosteroids 2, 3
- Immunosuppressive agents, such as mycophenolate mofetil or azathioprine 2, 3
- Novel therapies, such as thrombopoietin receptor agonists and autologous hematopoietic stem cell transplantation 2
- B-cell depletion for refractory thrombocytopenia 2
- Rituximab and intravenous immunoglobulin for refractory cytopenias 3