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Lymphoma
Lymphoma is a malignancy arising in lymphatic tissue - this includes lymph nodes, lymphoid tissue in the gastrointestinal tract, aerodigestive tract, or in the spleen. While lymphoma originates in the lymphatic tissue, it can spill-over into the bone marrow.1,2
Types of Lymphoma
Lymphoma is broadly classified histologically into Hodgkin's and Non-Hodgkin's lymphoma.
Hodgkin's lymphoma is characterised by the presence of Reed-Sternberg cells. These are binucleate lymphocytes, that look like "Owl's eyes". Non-Hodgkin's lymphoma does not contain these cells.
Non-Hodgkin's lymphoma can then be further classified as low grade or high grade depending on how fast they grow, and by cell type: NK, T or B cell.1,2
High grade lymphomas are aggressive but respond well to treatment such as chemotherapy. Low grade lymphoimas are slower to grow, but are less responsive to therapy. Low grade lymphomas can transform into high grade lymphomas.
Hodgkin's Lymphoma
Clinical Presentation
Patients often present with painless lymphadenopathy, which is asymmetrical. The most common region is the cervical region. Some patients may also have splenomegaly or hepatomegaly. Patients commonly present with B symptoms are systemic features which include fever, night sweats, weight loss, and fatigue.
Pruritus may also be a presenting feature of lymphoma.1,2
Investigations
The diagnosis of Hodgkin's lymphoma requires histological evaluation from a lymph node biopsy (excisional node biopsy is the best modality).1
Staging
The Ann Arbor staging system is used for lymphomas. This comprises of 4 stages. The investigation required is a CT scan.
The staging system also includes an "A" or "B". A means no systemic symptoms, and B means with systemic symptoms (B symptoms). For example, if a patient only had cervical lymph nodes affected, with fever and night sweats, the way to describe the patient's disease stage would be 1B.1,2
Management
Localised disease can be managed with localised radiotherapy.
Widespread disease should be managed with chemotherapy. Examples of chemotherapy drug regimens include ABVD - Adriamycin, Doxorubicin, Bleomycin, Vinblastine, Dacarbazine.1
Rituximab is a monoclonal antibody against CD20, which is expressed on the surface of B cells. This can be used to target B cell lymphomas.
Non-Hodgkin's Lymphoma
Lymphomas that do not have Reed Sternberg cells fall into the category of NHL. These can be further divided into B cell and T cell Lymphomas. 85% of NHL is B cell type, the remaining 15% is T or NK cell type. The table below provides a list of examples falling into each category.1
Clinical Presentation
Like Hodgkin's lymphoma, patients with NHL can present with painless, asymmetrical lymphadenopathy, with or without systemic symptoms (night sweats, fever, weight loss - this is less common in NHL). NHL more commonly affects the bone marrow and can therefore present with symptoms of bone marrow failure (anaemia, thrombocytopenia - purpura/petechiae, and infections due to leucopenia).
There can also be symptoms depending on which part of the body the lymphoma affects. Apart from the bone marrow, the gastrointestinal tract is a frequent extranodal site of disease, and can cause acute abdominal symptoms. Lymphoma from the lymph tissue in the oropharyngeal tract may result in sore throat symptoms. Some lymphomas affect the skin and will present with dermatological lesions (cutaneous T-cell lymphoma).1, 2
Investigations
Staging
The extent of disease is determined by PET-CT scan. The Ann Arbor staging system is used in NHL (same as Hodgkin's lymphoma).
Management
General principles of management include a combination of chemothreapy drugs. Rituximab is used to treat some types of NHL of B cell origin.1
In some cases, autologous stem cell transplantation is used.
In cases of Non-Hodgkin's lymphoma where there is rapid turnover of cells, there is a higher risk of Tumor Lysis Syndrome.2 Prevention includes hydration and Allopurinol. If Tumor Lysis does occur, it is managed with careful monitoring of U&Es, ECG monitoring, hydration, and Rasburicase. Visit the Emergenices in Haematology page for detailed information about Tumor Lysis Syndrome.
References
1. Provan D, Dokal I, Vos J, Baglin T. Oxford Handbook of Clinical Haematology. 4th ed. Oxford: Oxford University Press; 2015
2. Hoffbrand V, Moss P. Hoffbrand's Essential Haematology. Oxford: John Wiley & Sons, Ltd; 2016.
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