Journal of Hematology & Thromboembolic Diseases
Open Access

Analyzing the Hematological Manifestations of Systemic Lupus Erythematosus

Figueiredo Durand^{* *}Correspondence: Figueiredo Durand, Department of Clinical haematology, University of Porto, Porto, Portugal, Email:

Author info »

Description

Systemic Lupus Erythematosus (SLE) is a chronic, autoimmune disorder characterized by widespread inflammation and tissue damage affecting multiple organ systems. The etiology of SLE is complex, involving both genetic predispositions and environmental triggers that lead to an abnormal immune response. This hyperactivity of the immune system results in the production of autoantibodies, the deposition of immune complexes, and widespread inflammation, all of which contribute to tissue damage in various organs.

In addition to these classic organ manifestations, SLE has significant hematological implications. Hematological abnormalities are commonly observed in SLE patients and can affect both blood cell counts and function. These manifestations not only complicate the diagnosis and management of the disease but also play a significant role in its prognosis and response to treatment.

Hematological manifestations of systemic lupus erythematosus

Hematological manifestations include the anemia and anemia of chronic disease in these ways.

Anemia: Anemia is one of the most common hematological manifestations in SLE, affecting approximately 50–70% of patients during the course of the disease. There are several mechanisms underlying anemia in SLE, including:

Anemia of Chronic Disease (ACD): This is the most prevalent form of anemia in SLE. It occurs as a result of systemic inflammation that impairs iron metabolism and erythropoiesis. Inflammatory cytokines, such as interleukin-6 (IL-6) and tumor necrosis factor (TNF), increase hepcidin production, a key regulator of iron homeostasis. Elevated hepcidin levels inhibit the release of iron from stores and reduce iron absorption from the gastrointestinal tract, leading to functional iron deficiency.

Pure Red Cell Aplasia (PRCA): Although rare, PRCA is another form of anemia seen in SLE. In this condition, there is selective failure of red blood cell production in the bone marrow, resulting in a severe decrease in red blood cell count, while other cell lines (white blood cells and platelets) remain unaffected.

Lupus erythematosus in leukopenia

Lupus erythematosus in leukopenia includes several mechanisms:

Leukopenia: Leukopenia, or a reduction in the number of white blood cells (WBCs), particularly neutrophils, is a hallmark hematological feature of SLE, affecting up to 50% of patients. The mechanisms leading to leukopenia in SLE include:

Autoimmune Destruction: In some patients with SLE, autoantibodies may target white blood cells directly, leading to their destruction. This is especially evident in the case of neutropenia, where circulating neutrophils are destroyed by immune complexes or specific autoantibodies.

Bone Marrow Suppression: The systemic inflammation associated with SLE can lead to impaired bone marrow function, resulting in decreased production of white blood cells. This can be further exacerbated by the use of immunosuppressive medications such as corticosteroids and cytotoxic drugs, which are commonly used in SLE treatment.

Coagulation abnormalities

Coagulation abnormalities are an important consideration in the management of SLE, as these abnormalities increase the risk of both bleeding and thrombotic events. Key coagulation abnormalities include:

Antiphospholipid Syndrome (APS): APS is a disorder characterized by the presence of antiphospholipid antibodies, which can interfere with the normal coagulation process, leading to a hypercoagulable state. This is common in SLE and is associated with an increased risk of thrombosis, both venous and arterial, as well as pregnancy complications such as recurrent miscarriage and preeclampsia.

Bleeding Tendencies: Despite the prothrombotic risks, some SLE patients also experience bleeding tendencies, especially in the context of thrombocytopenia or bone marrow suppression. This highlights the complexity of coagulation abnormalities in SLE, where both thrombotic and bleeding risks coexist.