Lymphomas are a group of diseases caused by malignant lymphocytes that accumulate in lymph nodes and cause the characteristic clinical features of lymphadenopathy.
Occasionally, they may spill over into blood (‘leukaemic phase’) or infiltrate organs outside the lymphoid tissue.
The major subdivision of lymphomas is into Hodgkin lymphoma and non -Hodgkin lymphoma and this is based on the histological presence of Reed – Sternberg (RS) cells in Hodgkin lymphoma.
The disease can present at any age but is rare in children and has a peak incidence in young adults. There is an almost 2 : 1 male predominance. The following symptoms are common.
- Most patients present with painless, non – tender, asymmetrical, firm, discrete and rubbery enlargement of superficial lymph nodes. The cervical nodes are involved in 60 – 70% of patients, axillary nodes in approximately 10 – 15% and inguinal nodes in 6 – 12%. In some cases the size of the nodes decreases and increases spontaneously. They may become matted. Typically, the disease is localized, initially to a single peripheral lymph node region and its subsequent progression is by contiguity within the lymphatic system. Retroperitoneal nodes are also often involved but usually only diagnosed by computed tomography (CT) scan.
- Modest splenomegaly occurs during the course of the disease in 50% of patients. Th e liver may also be enlarged because of liver involvement.
- Mediastinal involvement is found in up to 10% of patients at presentation. Th is is a feature of the nodular sclerosing type, particularly in young women. Th ere may be associated pleural effusions or superior vena cava obstruction.
- Cutaneous Hodgkin lymphoma occurs as a late complication in approximately 10% of patients.Other organs (e.g. bone marrow, gastrointestinal tract, bone, lung, spinal cord or brain) may also be involved, even at presentation, but this is unusual.
- Constitutional symptoms are prominent in patients with widespread disease. The following may be seen:
- Fever occurs in approximately 30% of patients and is continuous or cyclic;
- Pruritus, which is often severe, occurs in approximately 25% of cases;
- Alcohol – induced pain in the areas where disease is present occurs in some patients;
- Other constitutional symptoms include weight loss, profuse sweating (especially at night), weakness, fatigue, anorexia and cachexia. Haematological and infectious
complications are discussed below.
The diagnosis is made by histological examination of an excised lymph node. Th e distinctive multinucleate polyploid RS cell is central to the diagnosis of the four classic types and mononuclear Hodgkin cells are also part of the malignant clone. Th ese cells stain with CD30 and CD15 but are usually negative for B – cell antigen expression. Inflammatory components consist of lymphocytes, neutrophils, eosinophils, plasma cells and variable fi brosis. CD68 detects infi ltrating macrophages and, if strongly positive, is an unfavourable feature.
Histological classification is into four classic types and nodular lymphocyte predominant disease, each of which implies a different prognosis. Nodular sclerosis and mixed cellularity are most frequent. Patients with lymphocyte rich histology have the most favourable prognosis of classic Hodgkin lymphoma. Nodular lymphocyte predominant does not show RS cells and has many features of non – Hodgkin lymphoma and may be treated as
- Full blood count
- Bone marrow aspirate and trephine (not routine)
- Liver function
- C – reactive protein
- Chest X – ray
- CT of thorax, abdomen, chest and pelvis
- PET or PET/CT
- Bone scan
Treatment is with radiotherapy, chemotherapy or a combination of both. The choice depends primarily on the stage although histological grading is an additional factor. Semen storage, if appropriate, should be carried out before therapy is begun.
- Lymphomas are a group of diseases caused by malignant lymphocytes that accumulate in lymph nodes and cause lymphadenopathy.
- The major subdivision of lymphomas is into Hodgkin lymphoma and non – Hodgkin lymphoma and this is based on the
presence of Reed – Sternberg cells in Hodgkin lymphoma.
- Reed – Sternberg cells are neoplastic B cells but most cells in the lymph node are reactive infl ammatory cells.
- The usual clinical presentation is with painless asymmetrical lymphadenopathy– most commonly in the neck.
- Constitutional symptoms of fever, weight loss and sweating are prominent in patients with widespread disease.
- Blood tests may show anaemia, neutrophilia and raised erythrocyte sedimentation rate (ESR) or lactic dehydrogenase (LDH).
- Diagnosis is made by histological examination of an excised lymph node and there are four subtypes of disease.
- Staging of the disease is important for determining treatment and prognosis. History, examination, blood tests, CT and
PET scan are typically used.
- Treatment is with radiotherapy, chemotherapy or a combination of both. The choice depends on the stage and grade of the disease.
- The response to treatment can be monitored by CT and PET scans. Disease relapse can be treated with chemotherapy, sometimes with stem cell transplantation.
- The prognosis is excellent and over 85% of patients can expect to be cured. Late side effects of treatment are a concern.