Hodgkin Lymphoma

WHAT IS HODGKIN LYMPHOMA?

Adult Hodgkin's lymphoma is a disease in which malignant (cancer) cells form in the lymph system.

The lymph system is made up of the following:

Lymph: Colorless, watery fluid that travels through the lymph system and carries white blood cells called lymphocytes. Lymphocytes protect the body against infections and the growth of tumors.
Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.
Lymph nodes: Small, bean-shaped structures that filter substances in lymph and help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the underarm, pelvis, neck, abdomen, and groin.
Spleen: An organ that produces lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach.
Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.
Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils produce lymphocytes.
Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow produces white blood cells, red blood cells, and platelets.
Because lymph tissue is found throughout the body, Hodgkin's lymphoma can begin in almost any part of the body and spread to almost any tissue or organ in the body.

Lymphomas are divided into 2 general types: Hodgkin's lymphoma and non-Hodgkin's lymphoma.

Hodgkin's lymphoma can occur in both adults and children; however, treatment for adults may be different than treatment for children. Hodgkin's lymphoma may also occur in patients who have acquired immunodeficiency syndrome (AIDS); these patients require special treatment.

There are 5 different types of Hodgkin's lymphoma. These 5 types are based on the way they look under a microscope.

Nodular sclerosing Hodgkin's lymphoma.
Mixed cellularity Hodgkin's lymphoma.
Lymphocyte depletion Hodgkin's lymphoma.
Lymphocyte-rich classical Hodgkin's lymphoma.
Nodular lymphocyte-predominant Hodgkin's lymphoma.
The incidence of Hodgkin lymphoma is consistently lower than that of non-Hodgkin lymphoma and has decreased over the past 30 years.In USA, about 62,250 Americans were diagnosed with lymphoma in 2004 (7,880 cases of Hodgkin lymphoma and 54,370 cases of non-Hodgkin lymphoma).



WHAT ARE THE RISK FACTORS OF HODGKIN LYMPHOMA?

The cause of this type of lymphoma is unknown. Many studies of environmental, especially occupational, linkages have been conducted with ambiguous results. The Epstein-Barr virus has been associated with a proportion of cases, but its precise role in the predisposition or onset of the disease is still under study. Hodgkin lymphoma and non-Hodgkin lymphoma occur with greater frequency among persons infected with the human immunodeficiency virus (HIV), although the virus itself is not considered to be the direct cause of Hodgkin lymphoma.

Age and gender can affect the risk of developing adult Hodgkin's lymphoma. Incidence rates higher in adolescents and young adults.



HOW TO DETECT HODGKIN LYMPHOMA?

The following symptoms may be caused by adult Hodgkin's lymphoma and suggest this disease.

Painless, swollen lymph nodes in the neck, underarm, or groin.
Fevers (unexplained).
Drenching night sweats.
Weight loss (unexplained).
Itchy skin.
Tiredness.
Symptoms which suggest diagnosis of this disease are briefly listed below table.

A =
no B symptoms

B =
presence of at least one of these


1)
unexplained weight loss > 10% baseline during 6 months prior to staging


2)
recurrent unexplained fever > 38oC


3)
recurrent night sweats


Examination of thewhole body and lymph nodes is very important to detect (find) and diagnose adult Hodgkin's lymphoma.

The following tests and procedures may be used:

Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's past illnesses and treatments will also be taken.
Complete blood count: A procedure in which a sample of blood is drawn and checked for the following: The number of red blood cells, white blood cells, and platelets. The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. The portion of the sample made up of red blood cells.
Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.
Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it.
Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done: Excisional biopsy: The removal of an entire lymph node. Incisional biopsy or core biopsy: The removal of part of a lymph node. Needle biopsy or fine-needle aspiration: The removal of a sample of tissue from a lymph node with a needle.
Immunophenotyping: A test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out if malignant lymphocytes (cancer) began from the B lymphocytes or the T lymphocytes.
Stages of Adult Hodgkin's Lymphoma

After adult Hodgkin's lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.

The following tests and procedures may be used in the staging process: CT scan (CAT scan), PET scan (positron emission tomography scan), bone marrow biopsy, laparotomy, chest x-ray, needle or surgical biopsy, and thoracentesis.

Stages of adult Hodgkin's lymphoma may include A, B, E, and S.

A: The patient has no symptoms.
B: The patient has symptoms such as fever, weight loss, or night sweats.
E: "E" stands for extranodal and means the cancer is found in an organ or tissue other than the lymph nodes or extends to tissues beyond, but near, the major lymphatic areas.
S: "S" stands for spleen and means the cancer is found in the spleen.
The following stages are also used for adult Hodgkin's lymphoma:

Stage I

Stage I: Cancer is found in a single group of lymph nodes.
Stage IE: Cancer is found in one area or organ other than the lymph nodes.
Stage II

Stage II: Cancer is found in two or more lymph node groups on the same side of the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).
Stage IIE: Cancer is found in one area or organ other than the lymph nodes and in the lymph nodes near that area or organ, and may have spread to other lymph node groups on the same side of the diaphragm.
Stage III

Stage III: Cancer is found in lymph node groups on both sides of the diaphragm.
Stage IIIE: Cancer is found in lymph node groups on both sides of the diaphragm and in a nearby area or organ other than the lymph nodes.
Stage IIIS: Cancer is found in lymph node groups on both sides of the diaphragm and in the spleen.
Stage IIIS+E: Cancer is found in lymph node groups on both sides of the diaphragm, in a nearby area or organ, and in the spleen.
Stage III is also divided into stage III(1) and stage III(2):

Stage III(1): Cancer is limited to the upper abdomen above the renal vein.
Stage III(2): Cancer is found in lymph nodes in the pelvis and/or near the heart.
Stage IV: the cancer either:

is found throughout at least one organ other than the lymph nodes and may be in lymph nodes near those organs; or
is found in one organ other than the lymph nodes and has spread to lymph nodes far away from that organ.
Recurrent Adult Hodgkin's Lymphoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the lymph system or in other parts of the body.

The staging of Hodgkin lymphoma may be briefly summarized in below table.

Stage
Involvement

1
Single lymph node region (1) or one extralymphatic site (1E).

2
Two or more lymph node regions, same side of the diaphragm (2) or local extralymphatic extension plus one or more lymph node regions same side of the diaphragm (2E).

3
Lymph node regions on both sides of the diaphragm (3) which may be accompanied by local extralymphatic extension (3E).

4
Diffuse involvement of one or more extralymphatic organs or sites.






HOW TO TREAT HODGKIN LYMPHOMA?

There are different types of treatment for patients with adult Hodgkin's lymphoma.

Different types of treatment are available for patients with adult Hodgkin's lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials.

Patients with Hodgkin's lymphoma should have their treatment planned by a team of doctors with expertise in treating lymphomas.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Combination chemotherapy is treatment with more than one anticancer drug.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Surgery

Laparotomy is a procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken for biopsy. If cancer is found, the tissue or organ is removed during the laparotomy.

High-dose chemotherapy and radiation therapy with stem cell transplantation

High-dose chemotherapy and radiation therapy with stem cell transplantation is a method of giving high doses of chemotherapy and radiation therapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

Treatment Options by Stage

Stage I Adult Hodgkin's Lymphoma

Treatment of stage I depends on whether the patient has stage IA (without symptoms) or stage IB (with symptoms) and where the cancer is.

Stage IA If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may include the following:
Combination chemotherapy with or without radiation therapy. Radiation therapy to lymph nodes in the mantle field (neck, chest, and armpits), including those near the aorta, with radiation therapy to the spleen if laparotomy is not done. Radiation therapy to the mantle field, with or without laparotomy.
If the cancer is above the diaphragm and does involve a large part of the chest, treatment may include the following: Combination chemotherapy and radiation therapy to the mantle field (neck, chest, and armpits).
If the cancer is below the diaphragm, treatment may include the following: Radiation therapy to the groin. Combination chemotherapy with radiation therapy to involved areas.
Stage IB. Treatment of stage IB is usually combination chemotherapy with or without radiation therapy.
Stage II Adult Hodgkin's Lymphoma

Treatment of stage II depends on whether the patient has stage IIA (without symptoms) or stage IIB (with symptoms) and where the cancer is.

Stage IIA If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may include the following: Combination chemotherapy with or without radiation therapy. Radiation therapy to lymph nodes in the mantle field (neck, chest, and armpits), including those near the aorta, with radiation to the spleen if laparotomy is not done. Radiation therapy to the mantle field with or without laparotomy.
If the cancer is above the diaphragm and does involve a large part of the chest, treatment may include the following: Combination chemotherapy and radiation therapy to the mantle field.
Stage IIB. Treatment of stage IIB may include the following: Combination chemotherapy with or without radiation therapy. A clinical trial of combination chemotherapy with or without radiation therapy.
Stage III Adult Hodgkin's Lymphoma

Treatment of stage III depends on whether the patient has stage IIIA (without symptoms) or stage IIIB (with symptoms) and where the cancer is.

Stage IIIA If the cancer does not involve a large part of the chest, treatment may include the following: Combination chemotherapy with or without radiation therapy.
If the cancer does involve a large part of the chest, treatment may include the following: Combination chemotherapy with radiation therapy.
Stage IIIB. Treatment of stage IIIB may include the following: Combination chemotherapy with or without radiation therapy.
Stage IV Adult Hodgkin's Lymphoma

Treatment of stage IV may include the following:

Combination chemotherapy.
Combination chemotherapy with or without radiation therapy.
Chemotherapy and stem cell transplantation.
Recurrent Adult Hodgkin's Lymphoma

Treatment of recurrent adult Hodgkin's lymphoma may include the following:

Combination chemotherapy.
Combination chemotherapy followed by high-dose chemotherapy and stem cell transplantation with or without radiation therapy.
Radiation therapy with or without chemotherapy.
High-dose chemotherapy and stem cell transplantation.


HOW TO ESTIMATE THE PROGNOSIS OF HODGKIN LYMPHOMA?

Certain factors which affect prognosis (chance of recovery) are on the following:

The patient's symptoms.
The stage of the cancer.
The type of Hodgkin's lymphoma.
Blood test results.
The patient's age, gender, and general health.
Whether the cancer is recurrent or progressive.
Adult Hodgkin's lymphoma can usually be cured if found and treated early.

The five-year relative survival rate for patients with Hodgkin Lymphoma has doubled from 40 percent in whites in 1960 to more than In the United States in 2004, there are 123,181 people living with Hodgkin lymphoma (active disease or in remission) and 346,749 people living with non-Hodgkin lymphoma, for a total of 469,930 members of the U.S. population who are living with lymphoma.

Five-year relative survival is 96 percent for Hodgkin lymphoma in people under 20 years of age.

Recently, criteria which can identify patients with high risk advanced Hodgkin's lymphoma became available. Each of the following factors has an equivalent negative impact on survival.

Age>45 years
Gender male
Stage IV
Hemoglobin<105 g/L
Albumin, serum<40 g/L
WBC>15.0 x 109/L
Lymphocytes count <0.6 x 109/L or percent <8% of WBC
A special attention should be paid the patients with high risk lymphoma.