Systemic lupus erythematosus (SLE) is associated with lymphoproliferative diseases such as Hodgkin’s lymphoma (HL)1. Since there is considerable overlap between the features of SLE and HL there can be a great difficulty in diagnosing HL in the presence of SLE1.
A 35-year-old woman was followed from 2002 for SLE with neuropsychiatric, renal and hematologic involvements. She was treated with only glucocorticoids with favourable outcomes. In April 2006, when she was under steroid treatment of 10 mg/day, she was admitted for cervical mass and weight loss. Physical examination showed a left indolent, fixed and elastic cervical adenopathy. The biological assessment was normal. Computerized tomography of the chest and abdomen showed a left basicervical mass expanded to the anterior and superior mediastinum. Magnetic resonance imaging was suggestive of a thymoma (Figure 1, Figure 2). The cervicotomy showed a supraclavicular mass. Histological and subsequent clonality studies confirmed classical Hodgkin’s lymphoma (HL) of the nodular sclerosing type. Viral serology (for Epstein-Barr, herpes simplex, and herpes zoster) was negative. The diagnosis of Hodgkin’s disease stage Ia was retained and the patient was transferred to hematological department where she was treated by chemotherapy (adriamycin, bleomycin, vinblastine and dacarbazine) with favourable outcomes. She is being currently followed in our department and is in remission of her lupus and Hodgkin's disease.
The relative risk of hematologic malignancy is estimated to be 60% higher in patients with SLE than in the general population, the reason being unknown1. Of all hematologic cases reported in patients with SLE, the most common is non-Hodgkin’s lymphoma followed by Hodgkin’s disease, leukemia, and then multiple myeloma1.
The initial presenting features of SLE and Hodgkin’s disease are similar, with fever, weight loss, and peripheral lymphadenopathy seen in most cases1. Our patient presented with weight loss and cervical adenopathy.
Persistent large lymph nodes not responding to conventional therapy in SLE should be biopsied for alternative diagnosis (i.e., lymphoma)1.
Several conditions and links have been identified that could potentially predispose patients with SLE to cancer (Table 1)2,3.
* Growth and hormonal factors may play a role in autoimmunity as well as in malignancy3.
A side-effect of immunosuppression is a possibility, as is intercurrent viral infection due to, for example, Epstein-Barr, herpes simplex, herpes zoster and polyoma viruses, which are potentially oncogenic. In our case, viral serology was negative.
Patients who have had a renal transplant are known to have an increased risk of cancer4. They are, however, treated with much higher doses of immunosuppressive agents than patients with lupus. In the studies of Petterson et al.5, Abu-Shakra6 and Sultan et al.7, the use of cytotoxic agents was not related to the occurrence of malignancy. Our patient was treated only with corticosteroids. It may be that the disease itself confers an increased risk. Patients with SLE have defects in both their cellular and humoral immune systems.
The mechanisms of hematologic malignancies8 are thought to be related to the following:
Failure or dysregulation of apoptosis as a result of mutated genes in SLE (Fas ligand).
Accumulation of and mutations in B and T lymphocytes in the lymph nodes.
T-cell immunodeficiency, allowing Epstein-Barr virus (EBV)-infected B-cell proliferation.
Exposure to immunosuppressive medications (possible increased risk of EBV infection in patients with SLE)9.
Large multicenter studies are required to adequately address the risk of developing malignancies in large cohorts of patients with SLE and to address issues such as associated risk factors and additional confounding factors, such as deprivation and exposure to therapy. The chance of detection of malignancy may vary due to factors such as access to health services, which vary widely and are not uniformly available, and may therefore underestimate the risk of malignancy.
SLE has been associated with increased frequency of neoplasia, lymphoma, leukaemia and epithelial tumours. Hodgkin’s disease has been occasionally associated with SLE in adults. An awareness of the association of Hodgkin’s disease with SLE and the modes of presentation will help in the early diagnosis and clinical management of such patients.
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