Causes and risk factors
In the West, the incidence of Hodgkin's lymphoma is about 3 new cases a year per 100,000 residents. As for the age of diagnosis, there has been two peaks, at the age of 25 and 65, respectively. Small families and few playmates have been found to be the risk factors that can be interpreted as delayed exposure to common infectious agents. There is a genetic predisposition to the disease and familiarity (4% of all new cases).
The causes are unknown. 30-50% of Hodgkin's lymphoma cases are associated with Epstein-Barr’s virus infection, but the role of the virus in the development of the disease is still unclear. There are no clear associations with any occupational exposure, cigarette smoking, or ionizing radiation. There is a link between HIV and Hodgkin's lymphoma.
Which are the symptoms?
Most patients have swollen lymph nodes (most often laterocervical and supraclavicular) at the beginning of the disease, which do not cause pain. 10% of patients, especially young women, have large mediastinal adenopathy. About a third of patients also report at least one of the following systemic symptoms:
- body temperature above 38°C without infectious causes;
- profuse night sweating;
- unexplained weight loss (>10% in the last 6 months).
Patients often report localized or diffuse itching and more or less severe pain in swollen lymph nodes after drinking alcohol.
How is it diagnosed?
The diagnosis of Hodgkin's lymphoma is histologic and must be made by surgical removal, usually of a lymph node, and analysis by an anatomopathologist of the suspected tissue. There are four variants of classical Hodgkin's lymphoma:
- nodular sclerosis;
- mixed cellularity;
- lymphocyte depletion.
The prognosis and treatment depend on the stage of the disease. Hence, there is a need for accurate diagnosis and procedures to determine the degree of lymphoma at the clinical onset of the disease (staging), using instrumental studies. In particular:
- computed tomography (CT) of the chest, abdomen, and pelvis: this is the single most effective means of evaluating lymph nodes and organs that may be affected by the disease;
- positron emission tomography (PET);
- bone marrow biopsy: allows to diagnose bone marrow infiltration.
How is it treated?
Patients with an early stage disease are treated only with chemotherapy or combined therapy (chemotherapy followed by radiotherapy). Intermediate and advanced stages are treated with combination therapy, although the role of radiotherapy in advanced stages of the disease is debated. Treatment options for patients with disease refractory to standard chemotherapy or recurrent disease include salvage radiotherapy, salvage chemotherapy, and high-dose chemotherapy followed by stem cell transplantation (autologous or matched donor). The San Raffaele Science Institute has extensive experience in stem cell transplantation. New drugs are being tested.