Cancer is an abnormal growth resulting from uncontrolled division of cells. When this occurs in the lymphatic system, it is known as lymphatic cancer or lymphoma.
Presentation
The symptoms of lymphomas are varied. Patients most commonly present with painless swelling in a lymph node group, usually in the neck, axilla or inguinal group of nodes. Some present with abdominal swelling and there could also be pressure symptoms like paresthesia which results from the tumor pressing against a nerve. Other non-specific symptoms include fevers, weight loss, chills, rigors, night sweat, and easy fatigability.
Workup
There is need for laboratory tests, imaging and histologic investigation in patients with lymphoma.
Laboratory tests
- Complete blood count
- Erythrocyte sedimentation rate
- Lactate dehydrogenase level
- Serum creatinine
- Liver function test
- HIV screening
- Hepatitis B and C screening[6].
Imaging
- Plain radiograph
- CT scan
- PET scan which distinguishes between viable and non-viable tumor
Histology
- Excisional lymph node biopsy
- Lumber puncture to rule out CNS involvement
- Bone marrow biopsy [7]
Treatment
Hodgkin's disease is generally considered to be curable. A combination of chemotherapy and radiotherapy is used in the treatment. Radiotherapy types include: Extended-field radiation, involved-field radiation and involved-node radiation.
Drugs used in chemotherapy include vincristine, adriamycin, doxorubicin, etopoxide, cyclophosphamide and many more are used in various combination therapies. The role of surgery is limited in the treatment of lymphomas. It is useful for specific types like gastrointestinal lymphoma [8].
Prognosis
For Non-Hodgkin lymphoma, patients generally have a 5 year survival rate of 63%. The prognosis is dependent on a number of factors like:
- Type of cells
- Size of the tumor
- Stage of the tumor
- Patient’s age
- Patient’s response to treatment
Hodgkin's disease has a 5 year survival rate of 85%. The outcome is dependent on factors like:
- Serum albumin less than 4g/dL
- Hemoglobin less than 10.5g/dL
- Male sex
- Age of patient 45years and above
- Ann Arbor stage IV disease
- WBC count greater than 15,000/mm3
- Absolute lymphocyte count less than 600/mm3
Each of this factors is assigned 1 point and the lower the total score, the better the prognosis [5].
Etiology
The causes of lymphoma are known carcinogens that have also been implicated in other cancers. They include:
- Infections which could be caused by viruses like Epstein-Barr virus, Hepatitis C virus and HIV and bacteria like Helicobacter pylori.
- Chemical pollutants like benzene, pesticides or hair dye.
- Chemotherapy and radiation exposure
- Drugs, especially tricyclic antidepressants (TCAs).
- Heavy metals [2]
- Chromosomal translocations
Epidemiology
Although lymphoma is currently ranked 7th in the leading types of cancers, the incidence is far becoming a major source of concern. The incidence of new cases has almost doubled since the early 70s and for the most part, there is no explanation for the rise although it might be due to new and better ways of making diagnosis.
The Non-Hodgkin type make up over 80% of all forms of lymphomas. Caucasians are at higher risk of developing this form of cancer compared to Blacks and Asians.
The incidence is generally higher in men. They are generally seen in individuals above 50 years although some subtypes are more prevalent in childhood [3].
Pathophysiology
Non-Hodgkin lymphoma results from a continuous clonal expansion of B or T cells and/or NK cells which arise from a buildup of lesions affecting protooncogenes or tumor suppressor genes leading to cell immortalization. Most of them are of B cell origin and their cell sizes and pattern of growth are determinants of tumor aggressiveness.
The presence of RS cells is the hallmark of the Hodgkin's disease. There are four established subtypes namely:
- Lymphocyte-rich Hodgkin lymphoma show typical RS cells.
- Lymphocyte depleted Hodgkin lymphoma is responsible for very few cases of Hodgkin lymphoma. An increased amount of RS cells and unusual sarcomatous modifications are present.
- The most common one is the nodular sclerosing Hodgkin lymphoma and it constitutes 3 or 4 out of every 5 incidence of Hodgkin lymphoma. It is nodular with a thickened capsule and the distinguishing cell type is the lacunar type Reed Sternberg cell.
- The fourth type is the mixed-cellularity type. Here, the RS cells give a classical appearance. Patients usually have advanced stage disease and it is the commonest type seen in patients with HIV infection [4].
A fifth type, which is now regarded as a different entity is the nodular lymphocyte-predominant Hodgkin lymphoma. It constitutes 5% of cases. Typical RS cells, if seen, are usually infrequent. There are instead lymphocytic and histiocytic cells.
Prevention
The best way to prevent this disease is to avoid the risk factors like viruses, chemical pollutants and drugs that have been implicated in its etiology [9].
Summary
Lymphatic cancer is a cancer that affects the lymphatic system. This is the system responsible for immunity. Lymphatic cancer is not a single entity but a collection of many types of cancers. They are broadly divided into two groups: Hodgkin's disease (Hodgkin lymphoma) and Non-Hodgkin lymphoma based on the presence of Reed Sternberg cells (RS cells) [1].
Patient Information
Definition
Lymphatic cancer, also known as lymphoma is a group of cancers that affects the organs responsible for immunity in the body. It is mostly seen in the extremes of age, young children and older adults.
Cause
It could be caused by long-term exposure to poisonous chemicals like benzene and insecticides. It could also be caused by viruses like the HIV virus. Other causes include drugs and heavy metals.
Symptoms
Some of the symptoms include swelling in the neck, swelling under the armpits, in the groin, and in the stomach. Other symptoms are fever, weight loss, pain, sweating at night and getting tired easily.
Diagnosis
The doctor would ask for a lot of investigations including blood tests like complete blood count to know the hemoglobin level and the number of white blood cells and platelets. HIV and hepatitis screening could be ordered as well as specific chemical blood tests. Other imaging techniques like CT scan also help in the diagnosis. A sample of the swelling would be taken to the lab to determine what type of lymphoma it is [10].
Treatment
Treatment of this disease involves radiotherapy and chemotherapy. The chemotherapy can be done on an out-patients basis. Surgery may also be needed for some patients like those who have the mass inside their abdomen.
References
- Tinguely M, Vonlanthen R, Müller E, et al. Hodgkin's disease-like lymphoproliferative disorders in patients with different underlying immunodeficiency states. Mod Pathol 1998; 11:307.
- Nieters A, Rohrmann S, Becker N, et al. Smoking and lymphoma risk in the European prospective investigation into cancer and nutrition. Am J Epidemiol 2008; 167:1081.
- Goldin LR, Björkholm M, Kristinsson SY, et al. Highly increased familial risks for specific lymphoma subtypes. Br J Haematol 2009; 146:91.
- Lukes R, Butler J, Hicks E. Natural history of Hodgkin's disease as related to its pathological picture. Cancer 1966; 19:317.
- von Wasielewski R, Werner M, Fischer R, et al. Lymphocyte-predominant Hodgkin's disease. An immunohistochemical analysis of 208 reviewed Hodgkin's disease cases from the German Hodgkin Study Group. Am J Pathol 1997; 150:793.
- Schmitz R, Hansmann ML, Bohle V, et al. TNFAIP3 (A20) is a tumor suppressor gene in Hodgkin lymphoma and primary mediastinal B cell lymphoma. J Exp Med 2009; 206:981.
- Horning SJ, Hoppe RT, Breslin S, Bartlett NL, Brown BW, Rosenberg SA. Stanford V and radiotherapy for locally extensive and advanced Hodgkin's disease: mature results of a prospective clinical trial. J Clin Oncol. Feb 1 2002;20(3):630-7
- Aparicio J, Segura A, Garcerá S, Oltra A, Santaballa A, Yuste A, et al. ESHAP is an active regimen for relapsing Hodgkin's disease. Ann Oncol. May 1999;10(5):593-5.
- Rummel MJ, et al. Bendamustine plus rituximab is superior in respect to progression free survival and CR rate when compared to CHOP plus rituximab as first-line treatment in patients with advanced follicular, indolent, and mantle cell lymphomas. Blood. 2009;114:405.
- Horvath B, Demeter J, Eros N, et al. Intravascular large B-cell lymphoma: Remission after rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy. J Am Acad Dermatol. Jul 24 2009