Haematological manifestations of lupus
Top Cited Papers
Open Access
- 3 March 2015
- journal article
- review article
- Published by BMJ in Lupus Science & Medicine®
- Vol. 2 (1), e000078
- https://doi.org/10.1136/lupus-2014-000078
Abstract
Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. While related to disease activity, there is no specific therapy for lymphopenia. Severe lymphopenia may require the use of prophylactic therapy to prevent select opportunistic infections. Isolated idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation, haemolytic anaemia, the antiphospholipid syndrome and renal disease. Thrombocytopenia can be regarded as an important prognostic indicator of survival in patients with SLE. Medical, surgical and biological treatment modalities are reviewed for this manifestation. First-line therapy remains glucocorticoids. Through our review, we conclude glucocorticoids do produce a response in majority of patients initially, but sustained response to therapy is unlikely. Glucocorticoids are used as first-line therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line therapy. Rituximab is promising in refractory and non-responding AIHA. TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of TTP and SLE, and a few patients with SLE have concurrent TTP. Myelofibrosis is an uncommon yet well-documented manifestation of SLE. We have compiled the cases that were reported in MEDLINE sources.Keywords
This publication has 176 references indexed in Scilit:
- The interleukin 6 receptor alpha gene polymorphisms are associated with clinical manifestations of systemic lupus erythematosus in KoreansInternational Journal of Immunogenetics, 2013
- Effects of belimumab, a B lymphocyte stimulator-specific inhibitor, on disease activity across multiple organ domains in patients with systemic lupus erythematosus: combined results from two phase III trialsAnnals Of The Rheumatic Diseases, 2012
- Evaluation of TRAF6 in a large multiancestral lupus cohortArthritis & Rheumatism, 2012
- Lymphopenia relating to T-lymphocyte apoptosis in systemic lupus erythematosusClinical Rheumatology, 2011
- Monocyte Chemoattractant Protein-12518 A/G Single Nucleotide Polymorphism Might Be Associated with Renal Disease and Thrombocytopenia of SLEJournal of Biomedicine and Biotechnology, 2010
- Familiality and co‐occurrence of clinical features of systemic lupus erythematosusArthritis & Rheumatism, 2002
- Pathogenetic significance of anti-lymphocyte autoantibodies in systemic lupus erythematosusClinical Immunology and Immunopathology, 1992
- Thrombocytopenia in systemic lupus erythematosus: Association with antiplatelet and anticardiolipin antibodiesClinical Immunology and Immunopathology, 1990
- Systemic tapes erythematosus and myelofibrosisClinical Rheumatology, 1989
- Systemic Lupus Erythematosus with Deficiency of the T4 Epitope on T Helper/Inducer CellsNew England Journal of Medicine, 1985