Understanding Hodgkin Lymphoma

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2 Understanding Hodgkin Lymphoma A Guide For Patients, Survivors, and Loved Ones Fourth Edition This guide is an educational resource compiled by the Lymphoma Research Foundation (LRF) that provides general information on adult Hodgkin lymphoma. Publication of this information is not intended to take the place of medical care or the advice of a patient s doctor. Patients are strongly encouraged to talk to their doctors for complete information on how their disease should be diagnosed, treated, and followed. Before starting treatment, patients should discuss the potential benefits and side effects of cancer therapy. National Headquarters 115 Broadway, Suite 1301 New York, NY (212) phone (212) fax Helpline: (800) ; helpline@lymphoma.org Website: LRF@lymphoma.org This patient guide is supported through unrestricted educational grants from: 2012 Lymphoma Research Foundation. Information contained herein is the property of the Lymphoma Research Foundation (LRF). Any portion may be reprinted or reproduced provided that LRF is acknowledged to be the source.

3 ACKNOWLEDGMENTS The Lymphoma Research Foundation wishes to acknowledge those individuals listed below who have given generously of their time and expertise. We thank them for their contributions, editorial wisdom, and advice, which have truly enhanced this publication. The review committee guided the content and development of this publication. Without their dedication and efforts, this publication would not have been possible. We hope those in the lymphoma community will now be better informed and have a better understanding of their illness because of the gracious efforts of those involved in the planning and execution of this comprehensive disease guide. Review Committee Ranjana Advani, MD, Stanford University Medical Center Nancy L. Bartlett, MD, Washington University School of Medicine Sven de Vos, MD, PhD, UCLA s Jonsson Comprehensive Cancer Center Randy Gascoyne, MD, FRCPC, British Columbia Cancer Agency David J. Straus, MD, Memorial Sloan-Kettering Cancer Center Anas Younes, MD, The University of Texas, MD Anderson Cancer Center ii Understanding Hodgkin Lymphoma

4 TABLE OF C ONTENTS Introduction...1 Part 1 Learning the Basics...2 Chapter 1: Understanding Hodgkin Lymphoma...2 Chapter 2: Seeking Medical Help...12 Chapter 3: Getting a Diagnosis...15 Chapter 4: Work-up Before Treatment Can Begin...21 Part 2 Treatment of Hodgkin Lymphoma...27 Chapter 5: What You Should Know Before Starting Treatment...27 Chapter 6: Treatments for Hodgkin Lymphoma...40 Part 3 Side Effects and Living With Cancer...59 Chapter 7: Common Treatment Side Effects...59 Chapter 8: Managing Your Life During and After Treatment...77 Part 4 Clinical Trials and Advances in Treatment..82 Chapter 9: Overview of Clinical Trials...82 Chapter 10: Advances in Treatment of Patients With Hodgkin Lymphoma...87 About the Lymphoma Research Foundation...89 Understanding Hodgkin Lymphoma iii

5 I NTRODUCTION The purpose of this booklet is to help patients with Hodgkin lymphoma become active participants in their healthcare decisions. Chapters in this book address different issues faced by these patients, including: what to expect during diagnosis, work-up, and treatment; how to cope with treatment side effects; and what questions to ask doctors. 1 Understanding Hodgkin Lymphoma

6 Part 1 Learning the Basics Chapter 1: Understanding Hodgkin Lymphoma Hodgkin lymphoma (HL) is a type of cancer that affects specialized white blood cells called lymphocytes. Lymphocytes work together with other cells in the immune system to defend the body against invasion by bacteria, viruses, parasites, and other foreign substances. Lymphocytes travel around the body in blood vessels and in a separate network of vessels called the lymphatic system. Part 1 The sections in this chapter will explain these and other terms that will help you understand HL and how it affects a person s health. A better understanding of the disease will help patients take a more active role in deciding the course of their treatment. What is cancer? Cancer is a group of diseases that develop when abnormal cells gain the ability to divide abnormally and to spread to areas in the body where they are not normally found. Your body is made up of many different types of specialized cells that are organized in tissues and organs to perform all the different tasks needed to stay healthy. To keep things running smoothly, the cells in your body grow, work, and divide in a very controlled fashion. All of these cells also have a limited lifespan. Normally, a self-destruct mechanism is triggered when a cell becomes too old or when it stops working properly. However, sometimes damage to the genetic material (DNA) of a cell gives it the ability to override this self-destruct mechanism, allowing these abnormal cells to live longer than normal. Unless the body s immune system gets rid of them, these abnormal cells can multiply and cause cancer. Learning the Basics 2

7 HOW CANCER FORMS INSIDE THE BODY Abnormal Cells Evade the Immune System Abnormal Cells Multiply (Cancer) Tumors May Form (Groups of Abnormal Cells) Most cancers are named after the organ or cell type of origin. For example, a cancer that started in the pancreas is called pancreatic cancer and a cancer that started in lymphocytes is called a lymphoma. 3 Understanding Hodgkin Lymphoma

8 Why is there no single cure for cancer? Cancer refers to a large group of very complicated diseases. A tricky aspect of cancer is that there are many different ways for a cell to become abnormal enough to develop into a cancer cell. Because of this, the path taken by a liver cell to become a cancerous liver cell is quite different from that taken by a lymphocyte to become HL. This is why a treatment that works against one type of cancer may not necessarily work against another. There are also smaller, but still important, differences in the cancer cells found in different patients diagnosed with the same type of cancer. Because of this, a treatment that may work very well in one patient may not have the same positive effect in another. These are some of the reasons why most cancers are much harder to treat or prevent than, for example, bacterial or fungal infections, which typically are caused by a single type of organism. Part 1 What is the lymphatic system? As shown in the picture on page 5, the lymphatic system is a circulatory system that is made up of a spidery network of thin tubes called lymph vessels. Similar to blood vessels, lymph vessels branch out into all tissues of the body. While people can clearly see blood vessels, especially at their wrists and on the top of their hand, the lymph vessels are invisible to the naked eye. Learning the Basics 4

9 ANATOMY OF THE IMMUNE SYSTEM The immune system is the body s defense against disease. Tonsil Thymus Lymph nodes Diaphragm Spleen Lymph vessels 5 Understanding Hodgkin Lymphoma

10 Lymph vessels carry lymph, a type of liquid that contains lymphocytes. Within this huge network of vessels are groups of small, bean-shaped organs called lymph nodes. Thousands of lymph nodes are found throughout the body, including the elbows, neck, armpits, chest, abdomen, and groin. Lymph fluid flows through lymph nodes and specialized lymph tissues such as the spleen, tonsils, bone marrow, and thymus gland. Lymph nodes filter lymph fluid, removing bacteria, viruses, and other foreign substances from the body. If a large number of foreign substances are filtered through a node or series of nodes, swelling may occur and the nodes may become tender to the touch. Most swollen nodes are a reaction to infection and are not cancerous. Part 1 What is a lymphoma? A lymphoma is a cancer that affects lymphocytes, a type of white blood cell. Lymphocytes travel through the blood and lymphatic system to defend the body against foreign invaders like bacteria and viruses. There are 2 major categories of lymphomas: Hodgkin lymphoma (HL) and non-hodgkin lymphoma (NHL). NHL is approximately 8 times more common than HL; according to the American Cancer Society, each year in the United States approximately 9,000 adults are diagnosed with HL and over 70,000 with NHL. Both of these major categories of lymphoma are further subdivided into several types that are different in the way they develop and spread, and in how affected patients are treated. Lymphomas usually develop when a change, or mutation, occurs within a lymphocyte, causing the abnormal cell to replicate faster than, or live longer than, a normal lymphocyte. Like normal lymphocytes, cancerous lymphocytes can travel through the blood and lymphatic system and spread and grow in many parts of the body, including the lymph nodes, spleen, bone marrow, and other organs. In advanced stages, most types of HL are found throughout the body by the time a patient is diagnosed with the disease. Learning the Basics 6

11 What is Hodgkin lymphoma? HL (previously called Hodgkin s disease) is a type of cancer that starts in lymphocytes. HL is named after Dr. Thomas Hodgkin, a British physician who first described the disease in Both children and adults can develop HL; the disease is most common in younger adults aged and older adults aged 55 and older. As a result of advances in the diagnosis and treatment of the disease, over 80% of patients with HL are cured. How does Hodgkin lymphoma develop? HL develops when a lymphocyte (usually a type of lymphocyte called a B cell) becomes abnormal (cancerous). These abnormal cells are called Reed-Sternberg (R-S) cells, named after the 2 scientists, Dorothy Reed and Carl Sternberg, who provided the first definitive microscopic descriptions of HL. Most people with HL have R-S cells, although other abnormal cell types may also be present. R-S cells are the defining cells of HL, although they constitute only a small fraction of the overall tumor mass, and they are not dividing cells. R-S cells produce factors that attract many inflammatory cells that make up most of the overall tumor burden. Due to this circumstance, HL initially was not recognized as a cancer but thought of as an infection. 7 Understanding Hodgkin Lymphoma

12 A REED-STERNBERG CELL Part 1 Reed-Sternberg cell Normal lymphocyte Having R-S cells alone does not necessarily mean that a person has HL. To confirm a diagnosis, the lymphatic tissue sample must also contain other cells and features that are characteristic of HL. To help make the diagnosis, a lymphoma pathologist (a doctor specialized in recognizing lymphoid cancers by examining tissues and organs under a microscope) will use tests that look for cell surface markers (antigens) that identify specific cell types of HL, and may also use more sophisticated molecular tests to help secure a confident diagnosis. HL usually starts in the lymph nodes and may be first noticed in the neck, above or below the collarbone, under the arms, or in the chest. Because lymph tissues all over the body are connected, abnormal (cancerous) lymphocytes can circulate in the lymphatic vessels, causing the lymphoma to spread from one lymph node to another throughout the body. HL can also spread to other areas and organs Learning the Basics 8

13 outside the lymph system. Unlike the more common NHL, HL tends to spread sequentially from one group of lymph nodes to the next, rarely skipping lymph nodes; this is a characteristic attribute of this form of lymphoid cancer. What are the most common types of Hodgkin lymphoma? The different types and subtypes of HL are distinguished according to what they look like under a microscope. The type of tumor a patient has may affect the choice of treatment. The 2 main types of HL are: n Classical HL, which affects 95% of patients diagnosed with HL in developed countries. n Nodular lymphocyte predominant HL. Classical HL (CHL) is subdivided into the following 4 different subtypes: Nodular Sclerosis CHL Mixed Cellularity CHL n This is the most common subtype of HL, affecting between 60-80% of patients with the disease. n Under the microscope, the involved lymph nodes often contain prominent scar tissue leading to the name nodular sclerosis (sclerosis means scarring). n The disease is equally common in men and women, and it usually affects teenagers and young adults. n Most patients are cured with current treatments. n This type of CHL affects 15-30% of patients with HL and is found more commonly in men than women. n Under the microscope, the lymph nodes contain many R-S cells mixed with many other types of cells. n This disease primarily affects older adults. n More extensive disease is usually present by the time patients with this subtype are diagnosed. 9 Understanding Hodgkin Lymphoma

14 Lymphocyte- Rich CHL n This subtype of CHL occurs in 5% of all patients diagnosed with HL. n Under the microscope, this subtype shows many normal lymphocytes and very few abnormal cells and R-S cells. n This disease is usually diagnosed at an early stage in adults and has a low rate of relapse or recurrence. Part 1 Lymphocyte- Depleted CHL n This is the least common form of HL, accounting for fewer than 5% of all patients diagnosed with the disease. n Under the microscope, there are very few normal lymphocytes and many R-S cells. n This is an aggressive lymphoma that is usually not diagnosed until the disease is widespread. Nodular lymphocyte predominant HL is rare, affecting 5-10% of patients diagnosed with HL. It affects more men than women and is usually diagnosed in people under 35. This subtype is often found in the neck lymph nodes, is usually diagnosed at an early stage, and it is not very aggressive (it grows slowly). In many ways, this form of HL is similar to low-grade (indolent) B-cell NHL. Why do some people develop Hodgkin lymphoma? The reasons why people develop HL are not well understood. However, scientists have found that people with certain characteristics have a higher risk of developing HL compared with people who do not have these characteristics. The characteristics that make a person possibly more susceptible to developing any type of disease are called risk factors. Having 1 or more of these risk factors does not mean a person will develop HL. In fact, most people with risk factors never develop the disease and many people diagnosed with HL have never been exposed to clearly identifiable risk factors. Learning the Basics 10

15 Known risk factors for HL include: Age Family History Sex Infection by Certain Viruses Weakened Immune System n People who are years or over 55 years have a higher risk of developing HL than people in other age groups. n People with a family history of the disease, especially among first-degree relatives (parent, brother, or sister), have a higher risk of developing the disease compared with people who do not have first-degree relatives with HL; siblings of patients with HL have a 3- to 7-fold increased risk of developing this disease, and the risk is higher in identical twins. Because HL is a relatively rare cancer, even with a 3- to 7-fold increase in risk, the chance of a patient s family member developing HL is still very low. n Only 1% of patients with HL have a family history of the disease. n Men have a slightly higher risk than women of developing HL, but more women than men have the nodular sclerosis subtype. n People infected with Epstein-Barr virus (EBV; which causes mononucleosis) or the human immunodeficiency virus (HIV; which causes AIDS) have a higher risk of developing HL compared with people who do not have these infections. n People who have a weakened immune system because of an inherited condition or the use of immunosuppressant drugs to prevent organ transplant rejection have a higher risk of developing HL compared with people who have a healthy immune system. HL cannot be caused by injury or by catching it from someone who has the disease. 11 Understanding Hodgkin Lymphoma

16 Part 1 Learning the Basics Chapter 2: Seeking Medical Help This chapter explains the signs and symptoms of HL and discusses how a doctor determines whether or not a person has the disease. Part 1 A symptom is anything unusual in a normal body function, appearance, or sensation that a patient experiences. During a visit with a healthcare practitioner, patients should report all of their symptoms to their doctor or nurse. Symptoms may indicate the presence of lymphoma or another disease. Signs are anything unusual that doctors or nurses notice when they examine their patients. What are the signs and symptoms of Hodgkin lymphoma? The following are signs and symptoms of HL. Keep in mind that 3 out of 4 people (75%) diagnosed with HL do not have any symptoms, and that none of these are specific to HL exclusively. n Lumps under the skin in the neck, under the arm, or in the groin. These lumps are swollen lymph nodes that are usually not tender or painful. Infections, not lymphomas or other cancer, are the most common reasons for swollen lymph nodes; in these cases the swollen lumps should return to normal size within a few weeks or months after the infection goes away. For patients with HL, these lumps persist, although they may fluctuate in size (i.e., get smaller). Learning the Basics 12

17 n Unexplained intermittent fevers that may last for many days or weeks. n Unexplained itching that grows steadily more severe over time and usually affects the trunk, arms, and legs, but it can also be more localized. n Weight loss (usually by more than 10% of a person s normal weight). n Extreme sweating at night enough to drench sheets. n Lack of energy. n Coughing, shortness of breath, or chest discomfort may be signs of HL in the chest. Having one or more of these symptoms does not mean that a person has HL. These symptoms may be caused by an infection or other conditions (including other cancers). When should a patient seek medical attention? Anyone who has persistent symptoms should see a doctor to make sure that lymphoma or another serious condition is not present. A good rule of thumb is to seek medical attention if any of the previously mentioned symptoms last longer than 2 weeks or sooner depending on their severity and impact on a person s daily life. Many of these symptoms can arise from different causes and need not be cancer. There are not any specific tests that doctors can use to routinely screen patients to see if they have HL. During the visit with the doctor, patients should describe all of their symptoms. The doctor will ask detailed questions about medical history and perform a complete physical examination. During the physical examination, the doctor will: n Check for swollen lymph nodes under the chin, in the neck and tonsil area, above the shoulders, on the elbows, in the armpits, and in the groin. n Examine other parts of the body to see if there is swelling or fluid in the chest or abdomen that may be caused by swollen lymph nodes. 13 Understanding Hodgkin Lymphoma

18 n Examine the abdomen to see whether any internal organs are enlarged. n Ask about any pain experienced. n Look for any weakness or paralysis that may be caused by an enlarged lymph node pressing against nerves or the spinal cord. Part 1 If doctors suspect lymphoma after reviewing the symptoms reported and signs they have uncovered during the examination, they will order other tests to confirm the diagnosis. These tests should include a biopsy and may also include blood tests, chest x-rays and other imaging tests, scans, and a bone marrow evaluation. These tests and procedures are discussed in more detail in the next chapter. Learning the Basics 14

19 Part 1 Learning the Basics Chapter 3: Getting a Diagnosis Doctors need the results of different diagnostic tests to accurately determine whether or not a patient has HL. This chapter explains the purpose of these different tests and describes what to expect during and after these procedures. Cautions About Interpreting Diagnostic Reports n Only a biopsy is definitive. n Tests can be reported as normal even though lymphoma may be present. n Tests may be reported as abnormal even though lymphoma is not present. n Other conditions may mimic HL. n The interpretation of tests, such as imaging studies and scans, can be difficult in some situations and needs to be made in the context of the disease and the patient. n Often, follow-up tests are needed to determine the true significance of previous results; additional biopsies may be needed to clarify the results. n Some patients like to review their written scan reports; when doing so, it is important to carefully review the findings with their doctor. How is Hodgkin lymphoma diagnosed? The only way to be absolutely sure of a diagnosis of HL (or any cancer) is for a doctor to perform an excisional biopsy to remove an entire lymph node or an incisional biopsy to remove a portion of the diseased tissue (see next page, What is a biopsy? ). A pathologist will examine slides containing portions of the biopsy sample under a 15 Understanding Hodgkin Lymphoma

20 microscope to see if it contains the characteristic features of Reed- Sternberg (R-S) cells and to see if the cells are arranged in ways typical of HL. A surgical pathologist is a doctor who specializes in the diagnosis of diseases by studying the cells from a patient s blood, body fluids, and tissue samples. A hematopathologist specializes in the pathology of blood cells, and a lymphoma pathologist specializes in the diagnosis and classification of HLs and NHLs. These doctors are trained to recognize different cell types by looking at the shape and size of cells and how they are grouped inside a tissue. Part 1 A core needle biopsy is sometimes used to establish a primary diagnosis, but is not optimal due to the small size of the biopsy sample. In situations where the site of biopsy is difficult to access, a core needle biopsy may be reasonable for diagnostic purposes (see the chart on the next page). In addition to routine pathology analyses, portions of biopsy samples will be used for other tests to confirm the diagnosis and to more exactly identify the specific subtype of HL. This chapter explains how these tests work and what kind of information they provide. What is a biopsy? A biopsy is a procedure in which a piece of tissue from an area of suspected disease is removed from the body and examined under a microscope. The information provided by this tissue sample is crucial to correctly diagnose the disease and decide on the best course of treatment. Learning the Basics 16

21 The following are the 2 main types of biopsies doctors use for the initial diagnosis of patients with a lymphoma: Excisional or Incisional Biopsy Core Needle Biopsy n This is the preferred type of biopsy to establish an initial diagnosis of lymphoma because it allows for the removal of bigger samples than other biopsy procedures. The larger the sample, the more tissue the pathologist can examine, which improves the accuracy of diagnosis. n In this procedure, a surgeon cuts through the skin to remove an entire lymph node (excisional biopsy) or a large portion of tissue (incisional biopsy). n If the lymph node is close to the skin surface, the procedure can be done under local anesthesia to numb the area. If the lymph node is in the chest or abdomen, the patient is sedated and the surgeon removes the tissue, usually by making a small incision above the sternum (breast bone). n This procedure is used when the lymph nodes are deep in the chest or abdomen or in other locations that are difficult to reach with excisional biopsy, or when there is a medical reason for avoiding an excisional or incisional biopsy. n In this procedure, a large needle is inserted into a lymph node suspected to be cancerous and a small tissue sample is withdrawn. n A needle biopsy can be done under local anesthesia and stitches are usually not required. n Sometimes the material collected may not be adequate for diagnosis and a subsequent excisional or incisional biopsy may be necessary. A fine needle aspirate (FNA) biopsy is, as the name implies, a type of biopsy performed with a very thin needle (smaller than that used for a core needle biopsy). Because of the small needle size, the sample will only contain scattered cells without preserving how the cells are actually arranged in the lymph node. This limited information is not enough for a precise diagnosis. A FNA biopsy is most often used to check for return of the disease (relapse) and is virtually never used for the initial diagnosis. 17 Understanding Hodgkin Lymphoma

22 After a tissue sample has been removed, it is examined by a pathologist. If the pathologist s interpretation of the biopsy is uncertain, the results should be reviewed by a hematopathologist or preferably a lymphoma pathologist because a pathologic diagnosis of lymphoma can sometimes be difficult to make. Arming the medical oncologist or hematologist with an accurate diagnosis on which sound treatment decisions can be made is of paramount importance. Part 1 What questions should a patient ask the doctor before having a biopsy or other diagnostic procedures? Patients diagnosed with a complicated disease will be asked to undergo a variety of procedures for the initial diagnosis and work-up before treatment begins, during the course of treatment, and during the follow-up period. Before patients agree to a procedure, they should make sure that they understand the reasons for the procedure and what will be involved. Here is a list of questions patients may want to ask their doctor. n Why is this procedure necessary? n What will the procedure tell us about my condition? n Can the same information be obtained in another way? n What is involved in doing this procedure? n What are the possible risks, complications, and side effects? n Where will I have the procedure done? n Will I have to do anything to prepare for the procedure? n How long will the procedure take? Will I be awake? Will I feel pain? n How long will it take for me to recover from the procedure? n Should anyone else be present when I have the procedure? n Will I need someone to take me home afterward? n When will I know the results? n When will we talk about the results? n What will be my out-of-pocket costs? Learning the Basics 18

23 What is immunophenotyping? Immunophenotyping is a process used during evaluation of biopsy material to distinguish between different types of cells (for example, between normal lymphocytes and lymphoma cells), by detecting specific molecules (cell markers or antigens ) found on the cell surface. These cell markers are detected using special antibodies grown and chemically modified in the laboratory so that they will change color when they stick to their corresponding markers. IMMUNOPHENOTYPING Antibodies change color upon binding to specific cell markers. Antibody Antigen 19 Understanding Hodgkin Lymphoma

24 This color change is studied under a microscope using immunohistochemistry (IHC) analysis. Immunohistochemistry (IHC) n In this test, thin slices of the biopsy sample (or thin layers of fluid or blood) are placed on slides and treated with sets of antibodies that recognize different markers found in different types of lymphoma cells and normal lymphocytes. n The pathologist examines the slides under a microscope to look for the visible color change that happens when the antibody sticks to the marker. n The pathologist identifies and counts the number of cells that change color (meaning that they are positive for the marker) with each of the different antibodies and uses that information to identify the specific type of lymphoma. Part 1 Learning the Basics 20

25 Part 1 Learning the Basics Chapter 4: Work-up Before Treatment Can Begin After the initial diagnosis of HL, the doctor may order other tests such as blood tests, bone marrow biopsy and aspiration, imaging studies (especially x-ray, CT, and PET scans), and heart and lung function tests. This process is often called the work-up. Some of these work-up studies are needed to see if and how much the disease has spread to other parts of the body. Doctors will use these test results to determine the stage of a patient s disease. Other tests will check how the disease has affected a patient s overall health and major organ functions. Together all of these tests will provide the information needed to help patients and their doctors decide on the course of treatment that will give patients the best chance for curing the disease or putting it into a lasting remission. This chapter will help you understand how HL is staged, the reason for the various tests, how these tests work, and what to expect. How is Hodgkin lymphoma staged? Staging is used to describe how widely the lymphoma has spread in patients with HL. As shown in the following figure, there are 4 main stages of lymphoma, designated by the Roman numerals I through IV. This staging system divides the body in half using the diaphragm, the muscle that divides the chest cavity from the abdominal cavity. 21 Understanding Hodgkin Lymphoma

26 STAGING OF HODGKIN LYMPHOMA Stage II: Two or more lymph node regions near each other One or more lymph nodes and one organ near each other Stage III: Two or more lymph node regions in different parts of the body with or without involvement of a nearby organ or the spleen Stage IV: Widespread disease Multiple organs With or without lymph node involvement Your doctor may also add any of the following single letters to the stage: n n n A if patients have not experienced symptoms such as significant weight loss (more than 10% in 6 months), drenching night sweats, or fevers. B if patients have lost weight (more than 10% in 6 months) and have experienced drenching night sweats or fevers. X if patients have a tumor in the chest that according to a chest x-ray is at least one-third as wide as the chest, or if tumors in other areas that are at least 10 cm (4 inches) wide, it is called bulky disease. Patients with bulky disease usually need more intensive treatment than patients without bulky disease. Learning the Basics 22 Part 1 Stage I: Localized disease Single lymph node region Single organ outside lymph nodes

27 Remember that advanced stages of HL are curable, unlike advanced stages of solid tumor cancers, which are more difficult to treat. What is the purpose of blood tests? Doctors will test a patient s blood to measure the quantity of the different types of cells (red blood cells, white blood cells, and platelets). While HL cells are not found in the blood, blood tests will help doctors determine how advanced the disease is and whether a patient will be able to tolerate certain kinds of treatments. The blood tests will most likely include: a complete blood count (CBC), differential (to measure the relative amounts of different types of white blood cells), platelets, erythrocyte sedimentation rate (ESR), alkaline phosphatase and other liver enzymes, and serum lactate dehydrogenase (LDH). Patients with risk factors for human immunodeficiency virus (HIV) will also be tested for HIV. Prior to therapy, the blood will also be tested for exposure to the hepatitis B virus (HBV). If the patient is a woman of childbearing age, her doctor will also do a pregnancy test because some of the possible treatments may hurt a fetus. The results from these tests will help patients and their doctors decide between different types of treatments. Many of these blood tests will be repeated during the course of treatment to check how the treatment is affecting the patient s body functions. What types of imaging tests may be used? The imaging tests usually recommended for a patient with HL are a chest x-ray and PET/CT scans of the neck, chest, abdomen, and pelvis. The purpose of these tests is to help find areas of the body where there may be cancer, to learn how far the cancer has spread, and later on to check how well the treatment is working. Most of these tests are painless, and no anesthetic is required. 23 Understanding Hodgkin Lymphoma

28 X-ray Computer Tomography (CT) Scan Positron Emission Tomography (PET) Scan n X-rays use radiation to take pictures of areas inside the body. The amount of radiation used in most diagnostic tests is so small that it poses little risk to the patient. n Findings on a chest x-ray may indicate whether the disease is bulky. n A CT scan takes x-rays from many different angles around the body. A computer combines the pictures obtained from these different angles to give a detailed image of organs inside the body. n Patients with HL often have CT scans of the neck, chest, abdomen, and pelvis. These tests are useful in determining how many nodes are involved, how large they are, and whether internal organs are affected by the disease. n The amount of radiation exposure during a CT scan varies depending on the area scanned. Most CT scans confer little risk to the patient, although CT scans of the abdomen and pelvis do add a moderate amount of risk (1 in 500-1,000) to the general lifetime risk of cancer. n Before a CT scan, the patient may be asked to drink a contrast liquid and/or get an intravenous injection of a contrast dye that will more clearly outline abnormal areas that may be present in the body. n PET scans are very important for the initial staging of HL. n To perform the test, radioactive sugar is first injected into the body. Increased uptake of the sugar in areas affected by HL are detected using a positron camera. The amount of radiation used for a PET scan is low and poses little risk to a patient. n PET scans can help determine how much disease is present (staging) and how well it has responded to treatment. n While CT scans show the size of a lymph node, PET scans show if the lymph node is active (still has significant disease). Today, CT and PET scans are often combined into 1 test (integrated PET/CT). Part 1 Learning the Basics 24

29 Magnetic Resonance Imaging (MRI) n Like a CT scan, an MRI takes images from different angles around the body, but an MRI does not use x-rays like a CT scan; instead it uses magnets and radiofrequency waves. MRI is not used routinely for staging, but sometimes can provide useful additional information. n An MRI can provide important information about tissues and organs, particularly the nervous system, that is not available from other imaging techniques. To create a clearer picture, a contrast dye may be injected into a patient s vein. n A doctor may order an MRI to get clear images of the bones, brain, and spinal cord to see if the cancer has spread to these areas. What is a MUGA scan? A MUGA scan (multi-gated acquisition scan) is an imaging test that looks at how well the heart muscle is working. A MUGA scan is done to make sure that the patient s body can withstand treatment with certain lymphoma drugs that may damage the heart in rare cases. MUGA scans may be done when patients are resting or exercising, depending on what their doctor wants to assess. A doctor will most likely order a MUGA scan if he or she is considering treating a patient with the drug doxorubicin (Adriamycin). This test is needed to make sure that the heart is functioning normally because doxorubicin may be associated with cardiac toxicity. A 2-dimensional echocardiogram (ECHO) is sometimes used instead of the MUGA scan to test heart function. Why might a patient need to have a lung function test? Lung function tests are done to make sure that the body can withstand treatment with certain lymphoma drugs that may stress a patient s lung function. Lung function tests are usually needed in patients with HL who will be undergoing treatment with a chemotherapy regimen that contains the drug bleomycin (Blenoxane). Bleomycin can cause lung damage. A doctor may order breathing tests before beginning treatment and at other times during treatment to make sure that a patient s lungs are still working properly. 25 Understanding Hodgkin Lymphoma

30 What is a bone marrow biopsy and aspiration? Once the diagnosis of HL is made, the doctor may order a bone marrow biopsy and aspiration to see if the lymphoma has spread to the bone marrow. The bone marrow is the spongy, soft material found inside our bones. Bone marrow biopsy is recommended for patients with symptoms or stage III or IV disease. The information from this procedure will help the doctor stage the disease. Part 1 What happens during bone marrow biopsy and aspiration? n The patient lies on the exam table, either on a side or on the stomach. n For the aspiration part of this procedure, the doctor cleans and numbs the skin over the hip and inserts a thin hollow needle into the bone. n The doctor uses a syringe to remove a small amount of liquid from the bone marrow. Even with the numbing local anesthetic, this procedure can be painful for a few seconds while the marrow is withdrawn. n For the biopsy part of this procedure (which is usually done right after the aspiration), the doctor inserts a slightly larger needle to take out a small piece of bone and marrow. This procedure may cause mild pain or a pressure sensation. The procedure does not require any stitches. n A pathologist looks at the samples under a microscope to see if there are any signs of HL. Patients who are anxious about the test should talk with their doctor and nurse to see whether taking a calming medication before the procedure would be helpful. Learning the Basics 26

31 Part 2 Treatment of Hodgkin Lymphoma Chapter 5: What You Should Know Before Starting Treatment Getting a cancer diagnosis is an overwhelming experience. It is perfectly normal to be shocked by the diagnosis, anxious about the future, and confused about the medical information and decisions that need to be made. This chapter will help you prepare for the start of treatment by explaining the next steps and providing tips for talking with a patient s doctor about any questions and concerns. First Steps to Take After Receiving a Diagnosis n Take care of yourself (eat, sleep, rest, and exercise). PATIENT TIP n Seek the support of family, friends, and others on whom you trust and rely. n Learn about the disease and treatment options. n Find medical care that meets your needs. n Find emotional and social support. n Understand the cost of care and what your insurance will cover. n Maintain a copy of your medical records (paperwork, test results, and your own notes). Who will plan and carry out the treatment? The treatment of patients with HL is usually overseen by a medical oncologist or hematologist who specializes in the treatment of patients with lymphoma. Depending on the patient s healthcare needs, the doctor may refer him or her to, or work with, other specialists such as a radiation oncologist. The patient s healthcare team will also include other healthcare professionals such as an oncology nurse, nurse practitioner, physician assistant, social worker, and registered dietitian. The healthcare team should work together and communicate with you to plan, carry out, and monitor the treatment. 27 Understanding Hodgkin Lymphoma

32 What is a prognosis? Prognosis is the medical term doctors use for predicting how the disease will progress and the likelihood for recovery. This is often one of the first things that patients ask their doctor. Keep in mind that HL is highly curable. About 80% of all patients with HL will be cured of their disease; that number rises to more than 90% in patients with earlystage disease. Prognosis is usually based on information gathered from hundreds or thousands of other patients who have had the same disease. This statistical information provides doctors with a general idea of what to expect when a patient is diagnosed with a specific type of HL, and also gives guidance on the kinds of treatments that have been most successful in treating that cancer. Part 2 Keep in mind that no two patients are alike and that statistics from large groups of people cannot accurately predict what will happen to a specific patient. The doctor most familiar with the patient s situation is in the best position to help interpret these statistics and understand if and how they may apply to a patient s particular situation. Treatment of Hodgkin Lymphoma 28

33 Patients with classical HL are grouped in the following prognostic categories to help doctors decide on the best course of treatment. Prognosis Stage Risk Factors Early Favorable Stage I or II None Early Unfavorable Stage I or II One or more of the following: n A tumor in the chest larger than onethird of the width of the chest according to a chest x-ray or at least 10 cm (bulky disease). n Cancer in an organ outside the lymphatic system but adjacent to a lymph node containing a tumor. n A high sedimentation rate also called erythrocyte sedimentation rate (ESR), which refers to the distance red blood cells travel in 1 hour in a sample of blood as they settle to the bottom of a test tube; inflammation, infection, cancer, rheumatic disease, and diseases of the blood and bone marrow increase the ESR. n Cancer in 3 or more nodal areas. n The presence of B symptoms (fever, weight loss, or night sweats). Advanced Favorable: Stage III or n Male. 0-1 Risk Factors Stage IV n Age 45 years or older. Intermediate: 2-3 Risk Factors Unfavorable: 4 Risk Factors n Stage IV disease. n Low blood albumin (protein) level (below 4). n Low hemoglobin level (below 10.5). n High white blood cell count (15,000 or higher). n Low lymphocyte count (below 600 or less than 8% of the white blood cell count). 29 Understanding Hodgkin Lymphoma

34 How does having a bulky tumor affect the patient s prognosis? Having a bulky or high-bulk tumor means that the patient has a tumor in the chest that is at least one-third as wide as the chest according to a chest x-ray, or a single mass of tumor tissue greater than 10 centimeters (4 inches) located anywhere. Because of the presence of bulky tumor, the patient will have to be treated more aggressively than a patient who has the same stage of disease but no bulky tumor. Often, but not always, radiation therapy is incorporated into the treatment of patients with bulky tumors. While smaller tumors are usually easier to eliminate than larger tumors, patients with bulky HL also have a high chance of being cured. Part 2 How will we decide what is the best treatment? There are many effective treatment options for patients with HL. To identify which treatments may work best, doctors consider the following: n The type of HL. n The extent of the disease (stage, location, and bulkiness). n Results of blood tests and other lab tests. n The presence or absence of lymphoma symptoms. n A patient s overall health. n A patient s age and medical history. n A patient s preferences. A doctor will discuss the risks, benefits, and side effects associated with the different treatment choices applicable to the patient s particular situation. Share questions and concerns with the doctor so that together you can decide which option is best. Use the following questions to help you make an informed decision. Treatment of Hodgkin Lymphoma 30

35 Questions to Ask Before Treatment Begins n What is my exact diagnosis? May I have a copy of the report from the pathologist? n What is the stage of my disease? Where are the tumors? PATIENT TIP n What are my treatment choices? Which do you recommend for me? Why? n Are new treatments being studied? Would a clinical trial be appropriate for me? n Do I need more than one type of treatment? n What is the goal of treatment? n What are the expected benefits of each type of treatment? How will we know if the treatment is working? What tests will I need to have to check if it works? How often will I need to get tested? n What are the risks and possible side effects of each treatment? Can these side effects be prevented or controlled? n What should I do to take care of myself during treatment? n Are there any late or long-term side effects that I should be aware of? n How long will the treatment last? n What are the chances that the treatment will be successful? n How will the treatment affect my normal activities? n How often will I need a checkup? n How much will the treatment cost? Will my insurance cover it? 31 Understanding Hodgkin Lymphoma

36 When should a patient get a second opinion? Before starting any type of treatment, a patient may consider getting a second opinion especially if some characteristics of the diagnosis are complicated or uncertain. The purpose of the second opinion is not to question the doctor s expertise, but to make sure that the suggested treatment plan is reasonable and optimal for the patient s particular case. Do not be concerned that you will offend your doctor by requesting a second opinion. Most doctors will be supportive and helpful if you tell them that you would like to get a second opinion. Ask the doctor if it would be okay to briefly delay the start of treatment to give extra time to get a second opinion. Keep in mind that some insurance programs require second opinions; others may cover it if a patient or doctor requests it. Part 2 PATIENT TIP Getting a Second Opinion n Some hematologists/oncologists/lymphoma specialists associated with medical schools or cancer centers may provide a consultation and be willing to work together with a local oncologist to provide treatment and follow-up care. n As part of the second opinion, another pathologist must review the tissue and blood samples to confirm the diagnosis. Ask your doctor about finding a pathologist with a lot of experience diagnosing patients with lymphoma. n To get a second opinion, you will have to provide the consulting doctor a complete copy of all medical records, original x-rays, pathology materials, scans, and reports. When you set up the appointment, ask their office for a list of all the materials they will need. It may be useful to keep your own copy of all these records in case you have questions or concerns later on. Treatment of Hodgkin Lymphoma 32

37 To identify lymphoma specialists to contact for a second opinion: n Ask your current doctors, family members, other patients, friends, and coworkers. n Contact the patient referral service at your local hospital and at the nearest hospital associated with a medical school; many hospitals have online directories that can be searched to find a specialist in your area. n Visit the American Society of Clinical Oncology (ASCO) at to search their oncologist database. n Visit the American Society of Hematology (ASH) at to search for hematologists with an interest in lymphoma. n Visit the National Cancer Institute (NCI) at to identify the nearest NCI-designated cancer center and call or visit their website to find out about their lymphoma specialists. n Visit the American Board of Medical Specialists (ABMS) at and click on, Is My Doctor Board Certified to find out if doctors are board certified in a particular specialty. How to find an oncologist and treatment center? A patient s primary care doctor will probably have referred them to a specialist likely a medical oncologist, hematologist, or hematologist/ oncologist. Oncologists are physicians who specialize in diagnosing and treating patients with cancer. Hematologists are physicians who specialize in diagnosing and treating patients with disorders of the blood and lymphatic system. Many physicians are experienced in both areas, and there is some overlap. Before agreeing to treatment by a specific specialist and treatment center, make sure that they will be able to meet all medical and personal needs. Patients should feel comfortable with the healthcare team and the quality of the care they provide. 33 Understanding Hodgkin Lymphoma

38 Here are some questions to ask to select the best medical team: n What are the credentials of the specialist, the other members of the medical team, and the hospital or cancer center? n Is the specialist board certified as a medical oncologist and/or hematologist? Has he or she passed qualifying examinations by the American Board of Internal Medicine to certify competency in these specialties? n How much experience do the specialist and treatment center have in treating patients with cancer in general, and HL in particular? n How many patients with this disease are being treated here now? n Does the specialist and/or center participate in clinical trials? n How much time does the specialist spend on research and how much on the treatment of patients with HL? n Is the specialist a member of the American Society of Clinical Oncology and/or the American Society of Hematology? n Does the clinic or center have modern surgical facilities and diagnostic equipment? n Is the specialist or clinic affiliated with any major medical center or medical school? n What arrangements are made for medical coverage after hours and on weekends, in case of an emergency? n Is my health insurance accepted at this center? Will the office file claims for reimbursement and process the paperwork? n What kind of patient resources does the clinic or cancer center have for patients with my disease? Part 2 Patients enrolled in a managed care program may have limited choices. However, patients have the right to choose another healthcare team if they are not entirely satisfied with their first consultation visit. They should talk with other patients about their experience and ask them if they would recommend their specialist and healthcare team. Also, patients should share their concerns with their primary doctor and ask for a referral to a different specialist if they are not satisfied after their first visit. Treatment of Hodgkin Lymphoma 34

39 How to communicate with the healthcare team Patients can ease their anxieties by establishing open, honest communication with their doctor and nurse regarding their diagnosis, and learning about what the prescribed treatment regimen is, how it works, what tests are involved, and what side effects and complications may be associated with it. A good first step is to write down all questions that come to mind. Before meeting with a doctor or nurse, whether for the first time or for follow-up visits, consider organizing these questions to bring to the visit. Put the 2 or 3 most important questions at the top of your list, since time with doctors or nurses may be limited. But make sure that a member of the medical team reads all of your questions, because they may see some that are more important than you realize. Patients should consider having a family member or close friend accompany them to the doctor s office or clinic to help ask questions and understand and remember answers. A companion could also help by taking notes during the visit. Some patients bring a recording device to record the answers. Check with the patient s doctor before recording any conversations. Most oncology nurses are also very well informed about cancer treatments and are a good source of information on a wide range of topics. Oncology social workers are also available to assist with practical and emotional needs from the time of diagnosis and onwards. Although family members are often very concerned about their loved one and want information concerning his or her care, confidentiality rules prohibit doctors from giving out information to anyone without the patient s expressed permission. For efficiency, designate 1 family member as the family contact. The patient must remember to specifically tell the doctor the identity of the primary family contact. 35 Understanding Hodgkin Lymphoma