Rheumatology Advance Access originally published online on July 16, 2009
Rheumatology 2009 48(10):1222-1226; doi:10.1093/rheumatology/kep204
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Myeloid leukaemia in systemic lupus erythematosus—a nested case–control study based on Swedish registers
1Department of Rheumatology, Mälar Hospital, Eskilstuna, 2Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Karolinska Institutet, Stockholm, 3Department of Genetics and Pathology, Rudbeck Laboratory, Uppsala University, Uppsala, 4Department of Medicine, Division of Haematology and 5Clinical Epidemiology Unit, Department of Medicine, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden.
Correspondence to: Björn Löfström, Department of Rheumatology, Malar Hospital, SE 631 88 Eskilstuna, Sweden. E-mail: bjorn.lofstrom{at}dll.se
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Abstract |
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Objective. To assess the risk factors for leukaemic transformation and myeloid leukaemia in patients with SLE.
Methods. A national SLE cohort identified through SLE discharge diagnoses in the Swedish hospital discharge register during 1964 to 1995 (n = 6438) was linked to the national cancer register. A nested case–control study in SLE patients who developed acute or chronic myeloid leukaemia was performed with SLE patients without malignancy as controls. Medical records from cases and controls were reviewed and bone marrow specimens were re-evaluated. A Medline search of previously published cases of SLE and myeloid leukaemia was performed.
Results. After confirmation of SLE diagnosis according to the ACR criteria, eight patients with SLE and myeloid leukaemia and 18 SLE controls were included in the study. Preceding leucopenia was significantly associated with leukaemia development, whereas other SLE manifestations were not. Two cases had a preceding bone marrow confirming myelodysplastic syndrome (MDS). Only two cases were significantly treated with cyclophosphamide or AZA. A Medline search resulted in only 15 previously published cases of coincident SLE and myeloid leukaemia. Preceding MDS was reported in five of these, whereas only eight had been treated with cytotoxic drugs.
Conclusion. Low-dose chemotherapy was not a major cause of myeloid malignancy in our population-based cohort of SLE patients nor in the reported cases from literature. Leucopenia was a risk factor for myeloid leukaemia development and an MDS was frequently seen. Therefore bone marrow investigation should be considered in SLE patients with long-standing leucopenia and anaemia.
KEY WORDS: Systemic lupus erythematosus, Acute myeloid leukaemia, Myelodysplastic syndrome, Risk factors, Leucopenia, Cytotoxic drugs
Submitted 20 January 2009;
revised version accepted 12 June 2009.
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