Two cases of sarcoidosis-lymphoma syndrome

Abstract

An association between sarcoidosis and lymphoproliferative diseases (LD), the sarcoidosis-lymphoma syndrome, has been previously described, and may be attributed to the underlying immunological abnormalities that occur during the sarcoidosis disease process. We report two patients who developed Hodgkin's disease and diffuse large B-cell non-Hodgkin's lymphoma (NHL) subsequent to their diagnosis of sarcoidosis after latency periods of 6 years and 18 years respectively. Both patients developed histologically-proven sarcoidosis late in life, at 46 years and 58 years, and had differing clinical courses. The first had radiographically staged II chronic progressive respiratory sarcoidosis (RS) and required long-term methotrexate to control the disease, while the second achieved a spontaneous remission of her stage I intrathoracic RS. After treatment, the patient with Hodgkin's disease remains in remission 2.5 years following six cycles of ABVD protocol chemotherapy and involved-field radiotherapy, while the NHL patient remains in remission at 3 years following six cycles of R-COP protocol chemotherapy. Clinicians should be aware of the potential risks of malignancy, and especially of LD in sarcoidosis patients. They should be alerted to the possibility of additional pathology by any atypical clinical features, and should biopsy new lesions and adenopathy to exclude any coexistent neoplasm.

Population‐based study of autoimmune conditions and the risk of specific lymphoid malignancies

“…A recent meta-analysis reported pooled standardized incidence ratios for NHL of 3.9 in rheumatoid arthritis, 18.8 in Sjögren syndrome and 7.4 in systemic lupus erythematosus(4). Sarcoidosis, a multi-system disorder characterized by granulomatous inflammation, has been linked with NHL in several case reports, suggesting a “sarcoidosis-lymphoma” syndrome(25;26). In keeping with our results, Mellemkjaer et al reported that NHL patients were more likely than controls to have had a hospital discharge for sarcoidosis(8).…”

A patient presenting with generalised lympadenopathy - sarcoidosis, lymphoma or tuberculosis?

“…Since then, a number of cases with co-existing sarcoidosis and lymphoproliferative disorders have been reported in the literature 5. One such report describes the case of a 71-year-old woman presenting with fever of unknown origin due to sarcoidosis-lymphoma syndrome, as active sarcoidosis and B cell lymphoma were found to be concurrently present 6…”

The Hypercalcemic Effect of Vitamin D3 in Sarcoidosis Could Be a Side Effect of an Endogenous Program Organizing Cancer Apoptosis

“…Cutaneous sarcoidosis response in a patient with bladder cancer [24] 2. Two cases of sarcoidosis-lymphoma syndrome [25] 3. Spontaneous resolution of a multiorgan sarcoidosis with malignant lymphoma [26] 4.…”