Genetic predisposition as well as environmental triggers such as Epstein-Barr virus infection and immunodeficiency syndromes are the most plausible explanations for the high incidence of SLL in the United States.
The diagnosis of SLL can be summarized as a disease that occurs as a malignant lymphoproliferative disorder affecting principally adults from the middle-age population. The disease is typically characterized radiographically by bilateral adenopathy, but rarely by a single extranodal lesion.\n
The management of SLL is unique to the disease itself and depends largely on symptoms, symptoms which vary considerably between individuals. Treatments may include a combination of medical and/or surgical treatments, chemotherapy, and/or radiotherapy. The decision to treat patients with SLL with chemotherapy as the sole management method, or chemotherapy in addition to irradiation, requires careful consideration due to both the high risk of relapse in untreated patients and considerable side effect risks for treatment.
In the United States, there has been considerable variation in reported rates of NHL during the past decade. The high number of cases that do not meet pathologic definition may reflect underdiagnosis and delays in diagnosis.
The constellation of clinical features typical of early small lymphocytic lymphoma is discussed in light of the clinical data, suggesting that the syndrome is likely an early manifestation of a lymphoma that usually involves B cells and is associated with a high proportion of monoclonal IgM-containing B cell lymphomas, whereas IgE-dependent lymphomas such as small lymphocytic IgE-positive lymphomas predominate in the elderly.
Although there are limited numbers of patients with SLL in clinical trials, as of December 2010, a handful of drugs are approved to treat this disease. Current clinical trials are focusing to identify the most effective regimen for a large number of patients with SLL. Because most clinical trials are being phase 2, multicenter studies with large sample sizes are needed.
Survival seems to be better when diagnosed localized vs. when diagnosed on systemic basis, and when diagnosed earlier rather than later than the median time of disease detection. Prognosis is worse in males than females. In view of this and of the lack of other options at present for patients with small lymphocytic lymphoma, the management of this disease should be individualized according to the individual characteristics of patients.
Treatment with xl114 resulted in an excellent and durable remission in patients with SLL with recurrent disease and disease progression after relapse. Xl114 showed potent antitumor activity in a preclinical model of follicular lymphoma.
XL114 did not provide a survival advantage over the placebo in this first-line end of the chemotherapy treatment. Thus, a prospective randomized study with a statistically and clinically relevant number of patients is necessary to confirm these results.
XL114 is effective in treating B-cell malignancies including small lymphocytic lymphoma as a part of a new class of drugs. The treatment response to XL114 is related to the underlying cause: relapsed follicular lymphoma will be sensitive, whereas small lymphocytic lymphoma is resistant to treatment with XL114.