Hodgkins Lymphoma is a malignancy of the lymph nodes and the lymphatic system. It is a primary neoplastic disease of the lymphoid B cells which are named Reed-Sternberg cells (Stevens, Lowe ; Scott, 2009). The histological presence is characterised by giant multinucleated cells (Map of Medicine, 2013). Hodgkin’s Lymphoma causes the cells situated in the lymphatic system to abnormally reproduce (lpch.org, 2013).
The change of a normal cell to a malignant one will involve a process that will cause the genes that are involved in normal homeostatic mechanisms to suffer mutational damage. This will result in the activation of genes stimulating proliferation or protection against cell death, the oncogenes, and the inactivation of genes which would normally inhibit proliferation, the tumor suppressor genes (Bertram, 2000).
A B cell is a type of lymphocyte that produces antibodies to fight infection. These are the most commonly used lymphocytes in the bloodstream and they are a crucial element in a strong immune system (Lymphomainfo, 2013).
These are categorised in to two groups based on their clinical and pathological findings. Classic Reed- Sternberg cells are binucleate, which are seen in mixed-cellularity and in nodular sclerosis grade 1 Hodgkin’s Lymphoma. Reed-Sternberg cells which are monoclear may be seen in any type of Hodgkin’s Lymphoma but are mainly encountered in mixed celluarity disease (Stevens, Lowe ; Scott, 2009).
The World Health Organisation recognises two histological types of Hodgkin’s Lymphoma. The nodular lymphocyte and the classic Hodgkin’s Lymphoma. The latter subdivides into four categories called nodular sclerosis, lymphocyte-rich, mixed cellularity and lymphocyte depletion. (Gobbi, ‘et al’, 2013)
Classical Hodgkin Lymphoma is a B-cell malignancy with a distinctive activated B cell phonotype and absence of immunoglobin production (Laskar et al, 2004).
Hodgkin’s Lymphoma accounts for a ratio of 1.2:1 in males especially before puberty (Map of Medicine, 2013). Studies undertaken by scientists have determined that Hodgkin’s lymphoma is caused by a variety of factors which include changes to immunity, infections, genetic factors, chemicals and lifestyle factors. (Cancerinfo.nhs, ) There are certain viruses such as Epstein-Barr Virus (EBV) and the Human Immunodeficiency Virus (HIV) that may increase the risk of developing Hodgkin Lymphoma. Individuals with a weakened immune system after organ transplant or inherited from direct family members. According to the Map of Medicine (2013) the peak age is between 15-54 years and after age 70 years.
Hodgkin’s disease involves a transformed B cell. Disease will present as an enlargement of a single lymph node which may be discovered following an investigation for other symptoms such as weight loss, fever, night sweats or itchy skin. (Stevens, Lowe, Scott, 2009) The symptoms of the tumor mass may include abdominal discomfort or distension, bone pains, neurological symptoms, shortness of breath, coughs and chest pains (Map of Medicine, 2013)
The commonest sites for the disease are the cervical, supraclavicular and the mediastinal lymph nodes, it is stated that sub-diaphragmatic presentations, bone marrow and hepatic involvement are less common. (Gobbi ‘et al’, 2013)
Signs and Symptoms
A symptomatic patient would have swollen lymph nodes in the neck underarm and groin there would be weight loss, itchy skin, weakness, tiredness, coughing and trouble breathing. (cancer.gov ) If a patient presents with a lump that they have had for more than six weeks in the absence of signs of infection they should have a full blood count and should be referred to a specialist in investigation of lymph nodes (Nice.org, 2013).
The prognosis of a patient who has Hodgkin’s Lymphoma is related to the disease stage at presentation. This diagnosis is required to correctly guide the patient along the appropriate therapeutic pathways (Lynch, 2007 pg. 48). However there should be no surgical procedure or biopsy of an enlarged lymph node without prior discussion with an oncologist or haematologist (Nice.org, 2013).
A calculation of the Internal Hodgkin’s Lymphoma Prognostic score is needed and the presence of four out of seven factors will define a poor prognostic category.
1. Age ;45 years
3. Stage IV
5. Albumin ;40
7. WBC ;15×109/l (Yorkshire Cancer Network, 2013)
On presentation of a symptomatic patient the doctor will palpate for enlarged lymph nodes, they will examine all lymph node chains which are the cervical and supraclavicular, inguinal and the axillary. A record of the location, size, texture,
consistency, mobility and tenderness will be reported. The patient’s blood will be examined to see signs for cancer. However a normal result should not be taken as definitive evidence that the patient does not have haematological cancer (The Map of Medicine, 2013).
Pathologists who study the lymphoma specimens should discuss their findings with the Multi-disciplinary team that is responsible for managing the patient. This allows all the clinical, laboratory and further imaging information to be integrated in the MDT meetings. Any treatment should be deferred until definite diagnosis is determined (Nice.org, 2013). However in an audit of reliability of CT scans, 27% of the 124 patient’s awaiting decisions had been offered treatment before the final diagnosis had been determined (Nice.org, 2013)
In accordance with the NICE guidelines there are pathways to follow and Lymphoma staging is carried out with Computed Tomography (Nice, 2012). A CT scan will provide detailed cross-sectional images of the body. The images will show in detail the internal organs. This will determine if there are any enlarged lymph nodes or organs in the body. CT is capable of detecting Hodgkin’s Lymphoma in the thorax, abdomen and pelvic regions. A contrast is used in the procedure to outline abnormal areas of the body. (Cancer.org, 2013)
However problems have occurred in the interpretation of radiological images by local clinicians. An audit was undertaken on CT scans that were referred to an oncology centre for further opinions. The audit revealed that in 34% of cases out of the 124 patients there was a difference in the CT findings. (Nice.org, 2013).
Once they have received the pathological and diagnostic results confirming the diagnosis of Hodgkin’s Lymphoma they will stage the disease according to the Cotswolds revision of Ann Arbors staging systems. (Lakhani, Dilly;Finlayson, 2009 pg. 260)
Nodal area involved
Nodal areas on the same side of the diaphragm involved (no. of involved sites recorded)
Nodal areas on each side of diaphragm
III ¹ upper abdomen
III ² lower abdomen
Visceral involvement. The spleen is part of the reticuloendothelial system. Splenic involvement does not carry the same staging implication as, for instance bone marrow or liver
(Lakhani, Dilly;Finlayson, 2009 pg. 260)
Disease is then sub classified according to the presence or absence of systemic symptoms (eg. Fever, night sweats, weight loss)
Systemic symptoms absent – A
Systemic symptoms present – B
Pet scanning is an imaging modality used in assessing patients with Hodgkin’s Lymphoma. Pet CT is useful in staging, restaging and identifying potential biopsy sites and quantifying the response to therapy. Pet CT is the modality used to identify sites of residual disease after therapy. This is due to it being able to image the whole body and the fact that it gives accurate anatomical distribution of the disease and how it has spread. (Lynch, 2007)
Pet ct has been reported to be better that CT or PET alone in the staging and re-staging of cancer. It provides information on tumor biology which is an important factor for the effective dose delivery in radiotherapy planning. This is due to the biological tumor volume allowing the radiation dose to be modulated in accordance to the distribution of the PET signal intensity within the tumor volume. This results in an effective and efficient delivery of radiation dose and not invading healthy tissue (Carty, Short, Shelly, O’Connell ; Eustace, 2011).
Both chemotherapy and radiotherapy have been used alone and in combination for patients with Hodgkin’s Lymphoma. There are various combination chemotherapies that have been effective. However they have different patterns of acute and late adverse effects. Nice guidelines state that extended field radiotherapy is likely to produce adverse effects in the future such as breast cancer and lung cancer. Therefore limited field radiotherapy is selected to reduce the risk of relapse and the future requirement of intensive treatment. (Nice.org, 2013)
After initial treatment of Hodgkin’s Lymphoma the appearance of a residual mass may occur and CT scans are unable to detect live cancer tissue they just detect an abnormal mass (Texaxoncology, ).
According to Nice guidelines in relation to Hodgkin’s Lymphoma a response assessment should be done with PET CT after two cycles and at the end of therapy for a residual mass (Nice.org, 2013).
A multi-disciplinary team is vital for a patient diagnosed with Hodgkin’s Lymphoma. Patients will benefit from the expertise of a range of specialists for their diagnosis, imaging and further treatment. All care will be given under the recognised guidelines so all the team will be pro-active (Nice.org, 2013).
The haemato-oncology MDT should include Haemato-oncologists, haemato-pathologists, nurses, palliative care specialists, support staff, clinical oncologists, radiologists, and extended MDT may include clinical members of the transplant team, a microbiologist, a pharmacist and a state registered dietician (Map of Medicine, 2013).
The MDT should meet weekly and review diagnosis and establish treatments plans. All these views should incorporate what the patient has requested too such as palliative care and referral to other services (Map of Medicine, 2013).