Hodgkin’s disease
Hodgkin’s disease, also called Hodgkin’s lymphoma, is a type of lymphoma.
LYMPHOMAS
Lymphomas are malignant neoplasms (cancer) of lymphoid tissue.
Lymphoma is a form of cancer that affects the immune system – it is a cancer of immune cells called lymphocytes, a type of white blood cells.
There is abnormal proliferation of lymphatic cells leading to hepatomegaly, splenomegaly and lymphadenitis
Classification of lymphomas
- Hodgkin’s lymphoma
- Non-Hodgkin’s lymphoma
Hodgkin’s Lymphoma
- A malignant proliferation of the lymphoid cells that is characterized histologically by Reed “Sternberg cells.
Hodgkin’s Lymphoma is a malignant disease in which the lymph glands are enlarged and there is an increase of lymphoid tissue in the liver spleen and bone marrow. This disease is fatal if not treated early It was described by a British physician called Thomas Hodgkin in 1832
The exact cause is unknown though it is associated with Epstein Birr Virus (EBV)
- Epstein Birr Virus exposure and infection has been linked to its etiology, especially in the under 15 and over 50 age ranges.
It can occur in both sex and ages but more common in male and in the age of 20-35 & 50-70 years (bimodal age distribution)
There is abnormal/neoplastic proliferation of an atypical form of lymphoid cell eponymously termed as reed-sternberg cell. It predominantly a B- cell disease
Clinical staging of Hodgkin’s lymphoma
Stage 1: involvement of a single lymph node or a single extra nodal site
Stage 11: involvement of two or more lymph node regions on the same site of the diaphragm or localized involvement of an extra nodal site and one or more lymph node regions of the same side of the diaphragm
Stage 111: involvement of lymph node region on both sides of the diaphragm
Stage 111 (1): lymphatic involvement of the upper abdomen in the spleen (ophiceliac and portal node)
Stage 111 (2): lymphatic involvement of the upper abdomen in the spleen (ophiceliac and portal node) and the lower abdominal nodes in the periaortic mesenteric and iliac region
Stage IV: diffuse or dissemination of the disease of one or more extra lymphatic organ or tissue with or without lymph node involvement
i.e H- Hepatic, L- lung, P – pleura, M- marrow, D-dermal, and O – osseous
WHO Classification
- Nodular sclerosing HL, the most common subtype with large tumor nodules
- Mixed cellularity subtype HL is also common subtype
- Lymphocyte rich is a rare subtype
- Lymphocyte depleted is a rare subtype
Clinical features of Hodgkin’s lymphoma
- Enlargement of one or group of nodes which may be discovered following investigation for non specific signs like weight loss (>60% in 6 months), fever and pruritus.
- Enlarged lymph nodes are replaced by firm rubbery (elastic character on palpation) pinkish white tissue
- Involvement of the spleen, liver and bone marrow if not treated
- Lymph node enlargement is insidious
- Involved nodes are painless and rubbery in consistency
- Fever weight loss, weakness, night sweats and anemia may be present in some cases
- Cough and dyspnoea due to enlarged hillar or thoracic lymph nodes
- Chills and tachycardia
- Hepatosplenomegaly
- Abdominal distention and ascites due to enlarged abdominal glands (retroperitoneal nodes)
- Patient complain of pain in the affected lymph node after taking alcohol (cause not known)
- Dysphagia if mediastinal nodes are involved
- Jaundice due to liver involvement
- Bone involvement leads to bone pain
- Paraplegia due spinal cord compression in extra Dural involvement
- Palpable abdominal masses
In advanced stages anemia occur due to decreased erythropoiesis and increase hemolysis
Differential diagnosis
- Non-Hodgkin’s lymphoma
- Lymphadenitis secondary to TB and cat scratch disease
- Pseudo lymphoma caused by phenytoin
- Lymphomatoid granulomatosis
- Sarcoidosis
- HIV disease
- SLE
Investigation done in Hodgkin’s lymphoma
- Lymph node biopsy
- Bone marrow examination
- Peripheral blood film reveals normochromic normocytic anemia, eosinophilia, neutrophilia and lymphopenia
- Raised ESR
- Chest x-ray reveals mediastinal mass
- CT-scan of the chest, abdomen and pelvis to define the extent of the disease
- Lymphangiography
- In TB lymph nodes are large tender, firm and may rapture to for sinuses
Management of Hodgkin’s lymphoma
- Radiation therapy for localized disease
- Short course combination therapy with less extensive radiation
- Radiation is combined with chemotherapy to treat disseminated disease
- Cytotoxic drugs are combined with steroids
- Two regimens are used i.e
- MOPP
Mustin/nitrogen mustard on day 1 and 8
Oncorin/ vincristine day 1 and 8
Procarbazine day 1 and 14
Predisone day I and 14
- ABVD
Adriamycin/ dexorubium day 1 and 15
Bleomycin day 1 and 15
Vinblastin day 1 and 15
Decarbazine day 1 and 15
- Nursing care is based on pancytopenia (A condition in which there is a lower-than-normal number of red and white blood cells and platelets in the blood.) and other drug effects
- Psychological support
- Nutrition support
- Regular hygiene to prevent infections
NON-HODGKIN’S (NON-BURKITT’S) LYMPHOMA (NHL)
This is cancer that originates from the lymphatic system.
Cancer begins in the cell of the immune system. The disease can begin elsewhere in the lymphatic system but more common found in lymph nodes where by lymphocytes mainly B-cells become abnormal. T-cells can also be affected.
The abnormal cell makes copies which also divide continuously. These cells exceed normal life span and can’t offer defense. Extra cells often form a mass called tumor
There are more than 60 specific non Hodgkin’s lymphomas
Staging of Non Hodgkin disease
The stage depends on how many areas are affected and imaging results
Stage I: The lymphoma cells are in one lymph node group such as in the neck or underarm. Or, if the abnormal cells are not in the lymph nodes, they are in only one part of a tissue or organ (such as the lung, but not the liver or bone marrow).
Stage II: The lymphoma cells are in at least two lymph node groups on the same side of either above or below the diaphragm or, the lymphoma cells are in one part of an organ and the lymph nodes near that organ (on the same side of the diaphragm). There may be lymphoma cells in other lymph node groups on the same side of the diaphragm.
Stage III: The lymphoma is in lymph nodes above and below the diaphragm. It also may be found in one part of a tissue or an organ near these lymph node groups.
Stage IV: Lymphoma cells are found in several parts of one or more organs or tissues (in addition to the lymph nodes). Or, it is in the liver, blood, or bone marrow.
Recurrent: The disease returns after treatment.
In addition to these stage numbers, the disease may also described as stage A or B:
A: You have not had weight loss, drenching night sweats, or fevers
B: You have had weight loss, drenching night sweats, or fevers.
Risk Factors for Non-Hodgkin’s lymphoma/ disease
Weakened immune system: The risk of developing lymphoma may be increased by weak immune system due to inherited condition or certain drugs used after an organ transplant Certain infections such as;
- Human immunodeficiency virus (HIV): HIV is the virus that causes People who have HIV infection are at much greater risk of some types of non-Hodgkin lymphoma.
- Epstein-Barr virus (EBV): EBV infection is linked to Burkitt
- Helicobacter pylori: pylori are bacteria that can cause stomach ulcers increase a person’s risk of lymphoma in the stomach lining.
- Human T-cell leukemia/lymphoma virus type 1 (HTLV-1): Infection with HTLV-1 increases a person’s risk of lymphoma and
- Hepatitis C virus: Some studies have found an increased risk of lymphoma in people with hepatitis C More research is needed to understand the role of hepatitis C
Age: Although non-Hodgkin lymphoma can occur in young people, the chance of developing this disease goes up with age. Most people with non-Hodgkin lymphoma are older than 60.
Obesity is other possible risk factor for non-Hodgkin lymphoma.
Occupation: People who work with herbicides or certain other chemicals may be at increased risk of this disease.
Clinical features of Non-Hodgkin’s lymphoma
- Non-Hodgkin’s lymphoma (NHL) is rare before 40 years.
- Lymphadenopathy is common but extra nodal spread occurs early, so first presentation may be in the skin, gut, CNS, or lungs.
- Although often symptomless, systemic symptoms are as for HL.
- Marrow involvement may cause pancytopenia
- Infection is common.
- Progressive non tender lymph node enlargement
- An unexplained weight loss
- Night sweats
- Persistent fever
- Anemia
- Persistent weakness and tiredness
- Hepatomegaly
- Pain swelling and fullness in the abdomen
- Splenomegaly
Diagnosis and Investigation
Physical exam: checking for swollen lymph nodes in the neck, underarms, and groin. Also examine for swollen spleen or liver.
Blood tests: The lab does a complete blood count to check the number of white blood cells. The lab also checks for other cells and substances, such as lactate dehydrogenase (LDH). Lymphoma may cause a high level of LDH.
Chest x-rays: this checks for swollen lymph nodes or other signs of disease in the chest.
Biopsy: A biopsy is taken from lymph node to confirm lymphoma. Removing an entire lymph node is best. The pathologist uses a microscope to check the tissue for lymphoma cells.
Bone marrow biopsy: The doctor uses a thick needle to remove a small sample of bone and bone marrow the hipbone or breast bone. Local anesthesia can help control pain. A pathologist looks for lymphoma cells in the sample.
- LFTs and RFTs
- Serology tests for HIV
CT scan: An x-ray machine linked to a computer takes a series of detailed pictures of the head, neck, chest, abdomen, or pelvis. A patient is given an injection of contrast material. Also may be asked to drink another type of contrast material. The contrast material makes it easier for the doctor to see swollen lymph nodes and other abnormal areas on the x-ray.
MRI pictures of your spinal cord, bone marrow, or brain. MRI uses a powerful magnet linked to a computer. It makes detailed pictures of tissue on a computer screen or film.
Ultrasound: An ultrasound device sends out micro sound waves. A small hand-held device is held against the patient’s body. The waves bounce off nearby tissues, and a computer uses the echoes to create a picture. Tumors may produce echoes that are different from the echoes made by healthy tissues. The picture can show possible tumors.
Spinal tap: CSF is checked for lymphoma cells or other problems.
PET (positron emission tomography) scan: an injection of a small amount of radioactive sugar is given. A machine makes computerized pictures of the sugar being used by cells in the patient’s body. Lymphoma cells use sugar faster than normal cells and areas with lymphoma look brighter on the pictures. The stage is based on where lymphoma cells are found (in the lymph nodes or in other organs or tissues)
Management of Non Hodgkin’s disease
Specialists who treat non-Hodgkin lymphoma include hematologists, medical oncologists, radiation oncologists, oncology nurses and a registered dietitian. The choice of treatment depends mainly on the following:
- The type of non-Hodgkin lymphoma
- Stage of lymphoma
- How quickly the cancer is growing (whether it is indolent or aggressive lymphoma)
- Age of the patient
- Other patient’s health problems
1. Watchful waiting:
- If a patient has indolent non-Hodgkin lymphoma without symptoms, treatment for the cancer is not initiated immediately. The treatment team watches the patient’s health closely so that treatment can start when symptoms begin
- Indolent lymphoma with symptoms needs chemotherapy and biological Radiation therapy may be used for people with Stage I or Stage II lymphoma
- In aggressive lymphoma, the treatment is usually chemotherapy and biological therapy People with lymphoma that comes back after treatment may receive high doses of chemotherapy, radiation therapy, or both, followed by stem cell transplantation
Before treatment starts, health care team should explain possible side effects and ways of managing them to the patient
2. Chemotherapy uses drugs to kill cancer cells throughout the body; drug can be administered by oral route, intravenous or into spinal cord in phases depending on the cancer stage and nature of the drug. Drugs in initial stage cyclophosphamide and chlorambucil
In recurrence CDVP (cyclophosphamide, doxorubicin, vincristine and prednisone) or CVPP (cyclophoshamide, vinchristine, procarbozine and prednisone)
Side effects poor appetite, nausea and vomiting, diarrhea, trouble swallowing, or mouth and lip sores, hair loss, infections, bruise or bleeding easily, skin rashes or blisters, headaches, weakness and tiredness
3. Biological therapy:
- People with certain types of non-Hodgkin lymphoma may have biological therapy. This type of treatment helps the immune system fight cancer.
Monoclonal antibodies i.e interferon, interlukin 2 and tumor necrosis factor (proteins made in the lab that can bind to cancer cell so that they can be destroyed). Patients receive this treatment through a vein at the doctor’s office, clinic, or hospital.
- Flu-like symptoms such as fever, chills, headache, weakness, and nausea may Most side effects are easy to treat. Rarely, a person may have more serious side effects, such as breathing problems, low blood pressure, or severe skin rashes.
4. Radiation therapy/ radiotherapy: uses high-energy rays to kill lymphoma cells. It can shrink tumors and help control Two types of radiation therapy are used for people with lymphoma:
- External radiation: A large machine aims the rays at the part of the body where lymphoma cells have collected. This is local therapy because it affects cells in the treated area only. Most people go to a hospital or clinic for treatment 5 days a week for several
- Systemic radiation: Some people with lymphoma receive an injection of radioactive material that travels throughout the body. The radioactive material is bound to monoclonal antibodies that seek out lymphoma The radiation destroys the lymphoma cells.
- External radiation to abdomen can cause nausea, vomiting, and diarrhea, on chest and neck there may be dry sore throat and difficult in swallowing, the skin may become red, dry, and People who get systemic radiation also may feel very tired, get infections and above signs worsen
5. Stem cell transplantation:
If lymphoma returns after treatment, stem cell transplantation is considered. A transplant of blood-forming stem cells allows a patient to receive high doses of chemotherapy, radiation therapy, or both. The high doses destroy both lymphoma cells and healthy blood cells in the bone marrow. Transplant given through a flexible tube placed in a large vein in the neck or chest area after heavy chemotherapy. New blood cells develop from the transplanted stem cells. The stem cells may come from body of the patient (Autologous stem cell transplantation) or a donor who is a brother, sister or parent (Allogeneic stem cell transplantation) and Syngeneic stem cell transplantation for identical twins
Supportive care aims at controlling pain and other symptoms, to relieve the side effects of therapy and to help the patient cope with the diagnosis. It includes
6. Nutrition: give calories to maintain a good weight, protein to keep promote strength. Eating well may help the patient feel better and have more
7. Activity: Walking, swimming, and other activities can keep the patient strong and Exercise may reduce nausea and pain and make treatment easier to handle. It also can help relieve stress
8. Follow-Up Care: regular checkups after treatment for non-Hodgkin The health team watches patient’s recovery closely and check for recurrence of the lymphoma. Checkups monitors change in health and treatment needs of the patient. Checkups may include a physical exam, lab tests, chest x-rays, and other procedures.
9. Social support: this can be provided by Doctors, nurses, and other members of the health care team who answer many questions about patient’s treatment, working, or other procedures.
Social workers can suggest resources for financial aid, transportation, home care, or emotional support. Support groups like patients or family members meet with other patients or their families to share what they have learned about coping with the disease and the effects of treatment. Groups may offer support in person, over the telephone, or on the Internet. A patient may want to talk with a member of the health care team about finding a support group.
10. Treat treatment side effects appropriately
- Helicobacter pylori is treated with antibiotics
- Surgical: this corrects stricture and obstruction
- Encourage bladder training , habit retraining and intake of oral fluids