Risk of hematologic malignancy may be increased in patients with systemic lupus erythematosus compared with the general population, but outcomes with these cancers are about the same, a small retrospective study suggested.
Patients diagnosed with hematological malignancies before or concurrently with SLE in the study presented with earlier-stage disease and often achieved cancer remission, according to the report inLupus Science and Medicine.
SLE patients have been known since the 1970s to be at higher risk for cancer, especially for non-Hodgkin's lymphoma, which in earlier studies resulted in higher overall mortality among SLE patients.
The study analyzed 45 patients at the University of Michigan Medical Center in Ann Arbor with confirmed SLE and hematological malignancies (HM).
"Our data suggest that SLE features may bring comorbid HM to clinical attention early, possibly resulting in better outcomes," concluded the authors, led by Jason S. Knight, MD of the university's Division of Rheumatology. Previous reports have focused on hematological malignancies diagnosed after SLE rather than before or concurrently, and there has been little data on response to hematological malignancy treatment in these patients.
The majority of the sample had a positive antinuclear antibody test and about half had positive dsDNA antibodies and antiphospholipid antibodies. The most common SLE manifestations were arthritis and hematological abnormalities. Seven patients had co-existing Sjogren's syndrome.
Most patients received prednisone (76%) and antimalarial agents (73%), while about 50% were treated with immunosuppression.
Overall, a history of hematological malignancies did not have a significant impact on SLE management, and patients received aggressive treatments such as cyclophosphamide for lupus nephritis as indicated.
Of the 45 patients 84% were female. The mean age was 39 at SLE diagnosis and about 48 at hematological malignancy diagnosis. For those diagnosed with hematological malignancies after SLE, the mean age was about 36 for SLE and about 52 for hematological malignancies; for those diagnosed before or concurrently, the mean age was about 45 for SLE and 40.5 for hematological malignancies (P=0.06). Of the 45-patient sample, 29 (64%) were diagnosed with hematological malignancies a year or more after SLE diagnosis, and 36% were diagnosed with hematological malignancies before or within 1 year of SLE.
Of the 16 patients diagnosed with hematological malignancies before or concurrently, nine were diagnosed more than 2 years before SLE, and these tended to be in remission before SLE diagnosis. Only one of this group had a relapse of malignancy. Of seven patients diagnosed with both diseases concurrently, six achieved remission or stabilization of their hematological malignancies. In this group the most common malignancy was leukemia/myeloproliferative disorder/myelodysplastic syndrome (31%), followed by indolent and T-cell lymphoma at 19% each.
"We were surprised to find that a third of patients in our series were actually diagnosed with the hematological malignancies before the SLE," Knight told MedPage Today. "While we considered the possibility that the SLE features might be a paraneoplastic syndrome, this was not typically the opinion of the rheumatologists taking care of the patients -- as almost all patients were minimally started on hydroxychloroquine and, in some cases, on more potent medications such as methotrexate."
In Knight's view, hematologists and oncologists should obtain a rheumatology opinion if their hematological malignancy patients develop joint pain, rashes, or other unexpected symptoms.
In the 29 patients diagnosed with hematological malignancies after SLE, the most common disease was diffuse large B-cell lymphoma (DLBCL), affecting 13 patients (45%), followed by indolent lymphoma at 21%.
DLBCL patients typically presented with advanced disease and despite aggressive treatment had poor outcomes. In this group, 10 of 13 had stage IV disease, 11 of 13 had extranodal disease, and seven had an International Prognostic Index score of 3 or 4 poor prognostic factors. Of 11 treated DLBCL patients, six died within 14 months, one had active disease at 11 months' follow-up and four achieved durable remission (28 to 102 months).
"It does not appear that the increased clinical attention these patients received for their SLE resulted in earlier diagnosis or better outcomes," the authors wrote. On the other hand, it did not appear that malignancy outcomes in SLE patients were markedly worse than in other patients with the same conditions.
In this post-SLE cancer group, six other patients had indolent lymphoma, with generally good outcomes and a mean survival of 39 months. Three had Hodgkin's lymphoma, with two achieving durable remission and one dying.
"Similar to the heightened risk of cardiovascular disease in SLE, this increased risk of hematological malignancies is something that clinicians should definitely be aware of and pursue aggressively when symptoms dictate," Knight said.
The link between SLE and hematological malignancies remains uncertain, although causal hypotheses include a common genetic predisposition, chronic stimulation of the immune system, and disproportional immune responses.
"As the etiology of the risk remains unclear, it also makes a lot of sense to minimize other cancer risk factors whenever possible in lupus patients -- for example, smoking and ionizing radiation exposure," said Knight.
Acknowledging the limitations of this small, retrospective study, which precludes any definitive analysis of risk factors, the investigators called for additional population-based research.
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