Hematologic manifestations of systemic lupus erythematosus
- Peter H Schur, MD
Peter H Schur, MD
- Editor-in-Chief — Rheumatology
- Section Editor — Basic Science
- Professor of Medicine
- Harvard Medical School
- Nancy Berliner, MD
Nancy Berliner, MD
- Professor of Medicine
- Harvard Medical School
- Chief, Division of Hematology
- Brigham and Women's Hospital
- Section Editor
- David S Pisetsky, MD, PhD
David S Pisetsky, MD, PhD
- Section Editor — Lupus
- Professor of Medicine and Immunology
- Duke University Medical Center
Hematologic abnormalities are common in systemic lupus erythematosus (SLE), both at the time of diagnosis and throughout the course of the disease. The major hematologic manifestations of SLE include anemia, leukopenia, thrombocytopenia, lymphadenopathy, and/or splenomegaly. These abnormalities may be a manifestation of SLE, related to another concomitant disease, and/or caused by an SLE treatment. Alterations in hemostasis have also been observed in SLE patients and are likely associated with autoantibodies. Autoantibodies may be prothrombotic (eg, antiphospholipid antibodies [aPL]) or, conversely, may inhibit the function of clotting factors and increase the risk of serious bleeding.
This topic review will provide an overview of the hematologic manifestations of SLE. The clinical manifestations, diagnosis, and an overview of the management of SLE in children and adults are discussed separately. (See "Overview of the clinical manifestations of systemic lupus erythematosus in adults" and "Systemic lupus erythematosus (SLE) in children: Clinical manifestations and diagnosis" and "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults" and "Overview of the management and prognosis of systemic lupus erythematosus in adults".)
Anemia — Anemia is the most common hematologic abnormality in patients with systemic lupus erythematosus (SLE) and affects more than half of all patients [1-4].
Causes of anemia — Multiple mechanisms may contribute to the development of anemia in patients with SLE [1,2,5-11]:
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- - Causes of anemia
- Anemia of chronic disease
- Iron deficiency
- Autoimmune destruction
- - Autoimmune hemolytic anemia
- - Pure red cell aplasia
- Microangiopathic hemolytic anemia
- Pernicious anemia
- - Evaluation of anemia
- - Neutropenia
- - Lymphocytopenia
- - Causes of thrombocytopenia
- - Evaluation and initial management of thrombocytopenia
- - Causes of pancytopenia
- - Evaluation and initial management of pancytopenia
- Antiphospholipid antibodies and antiphospholipid syndrome
- LYMPHADENOPATHY, SPLENOMEGALY, AND HIGH BLOOD CELL COUNTS
- Evaluation of lymphadenopathy, splenomegaly, and leukocytosis
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS