From the Guidelines
Systemic lupus erythematosus (SLE) is commonly associated with several hematological disorders, including thrombocytopenia and autoimmune hemolytic anemia (AIHA), which are the most frequent blood abnormalities necessitating anti-inflammatory/IS treatment in patients with SLE. The most recent and highest quality study, published in 2019, highlights the importance of haematological manifestations in SLE, including thrombocytopenia and AIHA 1.
Key Haematological Disorders
- Thrombocytopenia: characterized by a platelet count below 30,000/mm3, often requiring treatment with moderate to high doses of glucocorticoids (GC) in combination with immunosuppressive (IS) agents, such as azathioprine (AZA), mycophenolate mofetil (MMF), or cyclosporine.
- Autoimmune hemolytic anemia (AIHA): a less common but significant haematological disorder in SLE, treated with similar principles as thrombocytopenia, including GC, IS agents, and rituximab (RTX) in severe cases.
- Leukopenia: common in SLE, but rarely requires treatment; careful work-up is recommended to exclude other causes of leukopenia, especially drug-induced.
Treatment Considerations
- First-line treatment for significant lupus thrombocytopenia consists of moderate to high doses of GC in combination with IS agents, with intravenous immunoglobulin (IVIG) considered in the acute phase or in cases of inadequate response to high-dose GC.
- RTX should be considered in patients with no response to GC or relapses, while cyclophosphamide (CYC) may also be considered in such cases.
- Thrombopoietin agonists or splenectomy should be reserved as last options for treating thrombocytopenia.
- Antiphospholipid syndrome, characterized by arterial and venous thrombosis and pregnancy complications, is present in about 30-40% of SLE patients and requires specific treatment, including anticoagulation therapy 1.
From the Research
Haematological Disorders Associated with Lupus
The following haematological disorders are associated with systemic lupus erythematosus (SLE):
- Anaemia, which can be due to chronic disease, autoimmune haemolytic anaemia, or hypoplastic anaemia 2, 3, 4
- Leucopenia, which can affect both granulocytic and lymphocytic lines and may be caused by autoantibodies, drugs, hypersplenism, or marrow suppression 5, 3
- Lymphopenia, which can lead to increased susceptibility to infection and may require prophylactic therapy to prevent opportunistic infections 5
- Thrombocytopenia, which occurs frequently and is almost invariably autoimmune, and can be regarded as an important prognostic indicator of survival in patients with SLE 2, 5, 3
- Autoimmune haemolytic anaemia (AIHA), which can be refractory to multiple lines of treatment and may require novel therapeutic modalities 5, 6
- Thrombotic thrombocytopenic purpura (TTP), which is not recognised as a criteria for classification of SLE but can occur concurrently with SLE 5
- Myelofibrosis, which is an uncommon yet well-documented manifestation of SLE 5
- Neutropenia, which can lead to morbidity and mortality from increased susceptibility to infection and can be successfully treated with granulocyte colony-stimulating factor 5
Treatment and Management
Treatment and management of these haematological disorders can be challenging and may require a combination of medical, surgical, and biological treatment modalities 5, 6. Glucocorticoids are often used as first-line therapy, but sustained response to therapy is unlikely, and novel therapeutic modalities such as proteasome inhibitor-based combinations may be necessary for refractory cases 5, 6.