Chicago researchers looked at three people who developed lymphoma at different stages of their lupus disease to try to find clues on the association between the conditions.
Their study, which involved patients with lupus and non-Hodgkin’s lymphoma, or NHL, included a number of observations from scientific articles on the diseases’ connection.
The research, “SLE and Non-Hodgkin’s Lymphoma: A Case Series and Review of the Literature,” appeared in the journal Case Reports in Rheumatology. SLE refers to systemic lupus erythematosus, the scientific name for lupus.
There are many manifestations of lupus, some arising from the disease itself and some from therapy-induced side effects. Studies indicate that lupus patients have a higher rate of lymphoma than healthy people. That finding led to research on predictors of lymphoma development, lymphoma’s effect on lupus patients’ prognosis, and strategies for treating those with both diseases.
Winston Sequeira, a rheumatologist at Rush University Medical Center in Chicago, and some of his colleagues looked at three lupus patients who developed NHL at different stages of their lupus for clues to the diseases’ association. They were hoping to spot factors that could contribute to the development of lupus and predictors of that development.
Case 1 involved a woman who had had lupus for 10 years and chronic hepatitis B. She developed primary central nervous system lymphoma, or PCNSL.
Doctors gave her radiation therapy and intravenous dexamethasone. Spinal and bone marrow biopsies showed that the treatment reduced her lymphoma.
The medical team then treated her with high-dose methotrexate, Rituxan (rituximab) and intrathecal methotrexate administered to the spinal cord. This three-month chemotherapy combo also improved her condition. Doctors then gave her localized radiation therapy and consolidation chemotherapy with Cytosar-U (cytarabine).
She continues to make progress after the multiple treatment regimens.
Case 2 involved a woman who had had lupus for 19 years and developed a localized central nervous system, or CNS, lymphoma.
Doctors gave her the immunosuppressants Cytoxan (cyclophosphamide) and CellCept (mycophenolate). They added allopurinol to the regimen to treat a complication known as tumor lysis.
They also put her on high-dose intravenous methotrexate for diffuse large B-cell lymphoma, or DLBCL, and Diflucan (fluconazole) for a fungal disease she developed known as cryptococcosis.
Neurological symptoms that she had before her treatment began improving after her cycle of therapy.
Case 3 involved a man with lupus and DLBCL Complications arose during his first chemotherapy cycle. He developed febrile neutropenia, or low counts of immune cells known as neutrophils, and her kidney disease progressed to the point that she had to go on dialysis.
Subsequent chemo cycles led to the lymphoma going into remission.
Scientists say lupus stems from problems regulating the immune system. Some research indicates that immunosupressants increase the risk of a person developing lymphoma, although other studies contradict this finding. Research has also suggested an association between immunosupressants and other types of cancer, although the connection is more pronounced in NHL.
PCNSL carries a worse prognosis than other lymphomas. Immunodeficiency and autoimmune diseases are associated with the onset of PCNSL, studies have shown. Case 1 supported previous studies’ conclusion that hepatitis B increases the risk of someone developing PCNSL, the Chicago researchers said.
Cases 2 and 3 supported researchers’ previous findings that DLBCL is the most common type of non-Hodgkin’s lymphoma that people with lupus develop.
There are three types of DLBCL. Each develops differently, and patients’ responses to treatment and prognosis differ.
Some researchers have suggested a link between the Epstein Barr virus and lupus patients’ development of lymphoma. The prognosis for non-Hodgkin’s lymphoma in lupus patients “is heavily weighted upon the stage of the disease, with a higher stage predicting” a worse outcome, the Chicago researchers noted.
The standard treatment regimen for DLBCL usually leads to remission of the lymphoma, the researchers said. But it is ineffective in PCNSL because it has a difficult time penetrating the blood-brain barrier, they added.
As in Case 1, intrathecal chemotherapy with methotrexate and Rituxan is the standard regimen for treating PCSNL, the team said. But “the potential of stem cell transplantation in serving a potentially curative role not only in the management of DLBCL but also in inducing prolonged serological remissions of autoimmune disease should be explored in future studies,” they wrote.