Hodgkin Lymphoma Patient & Family Handbook

A Handbook for Families

Hodgkin Lymphoma

ONCOLOGY SERIES

A Handbook for Families

Hodgkin Lymphoma

ONCOLOGY SERIES

Hodgkin Lymphoma A HANDBOOK FOR FAMILIES

Author Susanne B. Conley, MSN RN AOCNS CPON®

Contributor Amy L. Billett, MD

Parent Reviewer Jerry Loew

Content Reviewer Steering Council

This handbook is published by the Association of Pediatric Hematology/Oncology Nurses (APHON) for educa- tional purposes only. The material has been developed by sources believed to be reliable. The material is not intended to represent the only acceptable or safe treatment of Hodgkin lymphoma. Under certain circumstances or conditions, additional or different treatment may be required. As new research and clinical experience expand the sources of information available concerning the treatment of Hodgkin lymphoma, adjustments in treatment and drug therapy may be required. APHON makes no warranty, guarantee, or other representation, express or implied, concerning the validity or sufficiency of the treatments or related information contained in this handbook. APHON provides this handbook for educational use. Users may copy and distribute this material in unadapted form only, for noncommercial use, and with attribution given to APHON.

n WHAT IS HODGKIN LYMPHOMA? Hodgkin lymphoma, also known as Hodgkin’s disease, is a cancer of the lymphatic system. The lymphatic system is part of the body’s immune system, which protects the body from infection and disease. The lymphatic system is made up of a network of tissues and organs, including lymph nodes, tonsils, bone marrow, the spleen, and the thymus. Lymph tissue also is found in other parts of the body including the gut, skin, and brain. The lymphatic system circulates watery fluid called lymph throughout the body. This lymph fluid includes a type of white blood cells called lymphocytes that help the immune system filter viruses, bacteria, and other foreign substances out of the body. Because lymph tissue is found throughout the body, Hodgkin lymphoma can develop almost anywhere and spread to almost any tissue or organ. Researchers have determined it is a cancer that develops from an abnor- mal lymphocyte (i.e., white blood cell) that divides too rapidly and grows without order or control. The diagnosis of Hodgkin lymphoma depends on the presence of a particular kind of abnormal blood cells called Hodgkin Reed-Sternberg cells. These large, abnormal lymphocytes are seen when the involved tissue from a person with Hodgkin lymphoma is examined under a microscope.

The Lymphatic System

n WHAT ARE SOME SYMPTOMS OF HODGKIN LYMPHOMA? Most patients with Hodgkin lymphoma experience swollen lymph nodes that usually are painless, firm, and movable. These nodes most commonly are found in the neck and chest and less commonly under the arms or in the groin. Other symptoms of Hodgkin lymphoma include • fever • night sweats • weight loss for no known reason • fatigue • difficulty breathing • itchy skin. n WHAT CAUSES HODGKIN LYMPHOMA? The causes of Hodgkin lymphoma are unknown. Individuals are at greater risk of developing Hodgkin lymphoma when they have the following: • Epstein-Barr virus (EBV), which is the virus that causes infectious mononucleosis, often called “mono” (how - ever, most people who have an EBV infection never develop Hodgkin lymphoma) • human immunodeficiency virus (HIV) • a family history of Hodgkin lymphoma (although hereditary cases are very rare). n WHO GETS HODGKIN LYMPHOMA? Hodgkin lymphoma is a rare disease, accounting for about 5% of childhood cancers in the United States. It rarely occurs in patients younger than 5 years, but the risk of developing Hodgkin lymphoma increases in the second decade of life, making it one of the most common cancers in teenagers (usually between the ages of 15 and 19 years) and young adults. n IS HODGKIN LYMPHOMA INHERITED? Though rare, some cases of Hodgkin lymphoma have been reported within families of certain ethnic groups. This leads to the premise that there may be some genetic predisposition that can increase the risk of developing Hodgkin lymphoma. There is a slightly greater chance that Hodgkin lymphoma will occur in siblings and cousins of those who have or have had the disease. Although the risk is about two to five times higher for siblings com- pared with nonsiblings, the likelihood that a sibling will get Hodgkin lymphoma is still very low.

n WHAT IS METASTASIS? Metastasis refers to the spread of a tumor from its original location (i.e., primary site) to other parts of the body. Hodgkin lymphoma often spreads from one lymph node to another and also can spread to organs outside the lymphatic system. If a biopsy reveals Hodgkin lymphoma, additional tests will be performed to find out if the cancer has spread to other parts of the body. n WHAT IS STAGING? Staging is the process of determining the location and extent of a disease at the time of diagnosis. The information gathered from the staging process determines the extent of the involvement of the disease and helps guide treatment for that disease. There are four stages in Hodgkin lymphoma which are determined by the locations of the lymph node chains that have tumors. A higher stage number indicates that the disease has spread further throughout the body. Hodgkin Lymphoma Staging Within each stage, the letters "E" and "S" are used to further classify the disease. "E" indicates that cancer has extended into an organ or tissue that is not part of the lymphatic system but is next to an involved lymph node. "S" indicates that cancer is found in the spleen. STAGE I • Stage I: Cancer is found in one group of lymph nodes. • Stage IE: Cancer is found in one group of lymph nodes and has extended to a nearby organ or tissue that is not part of the lymphatic system. STAGE II • Stage II: Cancer is found in two or more lymph node groups on the same side of the diaphragm (the breathing muscle that separates the chest and belly areas). • Stage IIE: Cancer is found in two or more lymph-node groups on the same side of the diaphragm and has extended from one of those lymph nodes to a nearby organ or tissue that is not part of the lymphatic system. STAGE III • Stage III: Cancer is found on both sides of the diaphragm. • Stage IIIE: Cancer is found on both sides of the diaphragm and has extended from one of these lymph nodes to a nearby organ or tissue that is not part of the lymphatic system. • Stage IIIS: Cancer is found on both sides of the diaphragm and in the spleen. • Stage IIIE+S: Cancer is found on both sides of the diaphragm and in the spleen and has extended from one of these lymph-node groups to a nearby organ or tissue that is not part of the lymphatic system. STAGE IV • Stage IV: Cancer is found throughout one or more organs that are not part of the lymphatic system (e.g., the lungs, bones, or bone marrow) and may be in lymph nodes that are near or far from those organs. Each stage is further classified as either "A" or "B." "A" means the child has no specific symptoms. "B" indicates the child has experienced specific symptoms prior to diagnosis. These specific "B" symptoms include an unexplained fever that exceeds 100.4 ºF/38.0 ºC, drenching night sweats, or weight loss of at least 10% of the individual’s body weight during a 6-month period.

Risk Groups In addition to staging, Hodgkin lymphoma is divided into risk groups based on information obtained during staging, the size and location of the tumor, and whether the patient has “B” symptoms. Once the risk group is determined, the treatment team will use this information to decide which type of treatment is needed.

n WHICH TESTS AND PROCEDURES WILL MY CHILD NEED? Several tests are necessary to diagnose Hodgkin lymphoma and determine the extent of your child’s disease. The lymph nodes, chest, and abdomen will be examined. An enlarged spleen or liver may indicate Hodgkin lymphoma is present in those organs. The presence of small, soft lymph nodes in children may be misleading because only lymph nodes that have been increasing in size or that are significantly enlarged are a concern. To determine the best treatment possible, it is very important to identify the exact type of Hodgkin lymphoma and where it is located in the body. Your child’s physician will decide which tests and procedures are needed.

Most Commonly Performed Tests and Procedures

TISSUE BIOPSY The removal of all or part of a lymph node or other tissue is necessary to diagnose Hodgkin lymphoma. A lymph node tissue biopsy can be performed either in an operating room or in the hospital’s radiology department. Your child’s comfort and anxiety level always will be considered when planning procedures that involve needles. Once the biopsy sample is obtained, a pathologist will examine the tissue under a microscope to look for cancer cells, specifically Hodgkin Reed-Sternberg cells, which are common abnormal lymphocytes in Hodgkin lymphoma.

X-RAY EXAMINATION A chest X-ray, which takes a picture of the organs and bones inside the chest, may be necessary to determine whether Hodgkin lymphoma is present in the upper middle area of the chest (the mediastinum). This is the area just above the heart but below the neck, and it is a common site for Hodgkin lymphoma. A chest X-ray also can help determine if the lungs are affected. CT SCAN A computerized axial tomography (CT) scan is a computer-assisted X-ray that provides very detailed images of areas inside the body from different angles. The scan is painless and quick, but your child must be able to lie completely still during the scan. Younger children may require sedation to help them lie still. Your child may not be allowed to eat or drink for several hours before the exam. To make the CT images clearer, it also may be nec - essary for your child to have a small amount of dye injected into a vein or to drink a liquid containing a flavorless dye. Typically, side effects do not occur from either type of dye, although allergic reactions are possible. These scans are obtained at the time of diagnosis and at different times during treatment to determine how your child is responding to it.

PET SCAN A positron emission tomography (PET) scan creates images of the body that show the size and shape of organs and tumors. PET scans are like CT and magnetic resonance imaging (MRI) scans, except they also can show chemical and functional changes within the body. A PET scan has three steps: injection of a radioactive mate- rial; a waiting period, usually 30–60 minutes; and scanning by the PET machine. During the waiting period and the scanning, your child must be able to lie completely still. The scanning process is painless; however, some children may require sedation to help them lie still. Like the CT scan, a PET scan should be obtained at diagnosis and is used to evaluate your child’s response as treatment progresses. BONE MARROW BIOPSY AND ASPIRATION Some children may need a bone marrow biopsy and aspiration, which is a procedure that involves collecting a sample of bone marrow. Bone marrow is found in the hollow part of the bone and is where white blood cells (infection fighters), red blood cells (oxygen carriers), and platelets (cells that help blood clot properly) are made. Once the bone marrow sample is collected, it is examined under a microscope by a pathologist to see whether Hodgkin lymphoma is present. Children usually require sedation or anesthesia during this procedure because it is performed with a biopsy needle.

Other Tests and Procedures that May Be Performed

MRI An MRI is a scan that produces very exact pictures of organs and tumors inside the body. During an MRI scan, your child will lie on a table that is moved into a tube-like machine that surrounds him or her with a magnetic field. The scan is painless; however, the machine makes a loud banging noise that may frighten some children. Your child must be able to lie completely still during the scan. If your child is unable to remain still, sedation may be necessary. BLOOD TESTS Blood tests are performed to monitor your child’s blood cells, body salts, and certain proteins or compounds of interest in the blood. These tests primarily are used to monitor your child’s response to treatment and any possible side effects of therapy. A complete blood count helps detect decreases in the number of red blood cells, white blood cells, or platelets in the blood. Blood chemistry tests, such as tests that check the individual’s blood urea nitrogen and creatinine levels, are performed to detect changes in kidney function. Other tests, such as those monitoring the individual’s levels of alanine aminotransferase, aspartate

aminotransferase, and bilirubin, may be necessary to assess liver function. Blood tests, such as those monitor- ing the individual’s rates of erythrocyte sedimentation and C-reactive protein—which sometimes are elevated in cases of Hodgkin lymphoma—may be used to measure the individual’s response to treatment. During your child’s first visit to the hospital or clinic, your treatment team may request blood tests to rule out other diseases, but these tests are not diagnostic for Hodgkin lymphoma.

VENOUS ACCESS DEVICE (VAD) Chemotherapy usually is needed to treat Hodgkin lymphoma, and there are several line options to help with your child’s treatment. Because treatment for Hodgkin lymphoma often is brief, a temporary catheter called a peripherally inserted central catheter may be used. Sometimes, a surgeon will insert a small plastic tube called a venous access line or device (i.e., a port) into a large blood vessel, usually under your child’s collar bone. These VADs can be used for blood tests and to give medications, chemotherapy, blood products, and nutritional sup- port when needed. You and your child’s treatment team will decide whether a VAD is necessary. A VAD usually is left in place for the duration of treatment and then removed after the completion of therapy.

n HOW IS HODGKIN LYMPHOMA TREATED? Hodgkin lymphoma is one of the most treatable cancers, with a long-term survival rate of more than 90% after treatment. The goal of treatment for Hodgkin lymphoma is to cure the disease while minimizing any treatment-related side effects or adverse reactions. Chemotherapy and radiation therapy are the two most common therapies used to treat Hodgkin lymphoma.

Chemotherapy Chemotherapy is medicine that helps kill cancer cells and prevents the cancer from spreading. Because no single chemotherapy medicine can control the disease by itself, a variety of medications are given in combination to kill the cancer cells. The exact combination of chemotherapy agents your child receives will depend on the stage of the disease and other treatment considerations. Many chemotherapy medications are administered through a vein or VAD, although some are given by mouth. Most chemotherapy treatments are administered in an outpatient clinic; however, some treatments may require hospitalization. Close monitoring of your child will be a priority when they receive chemotherapy. Your child’s treatment team will explain the possible side effects of the specific chemotherapy medicines your child receives. Radiation Therapy Radiation therapy uses a special type of X-ray treatment that kills or damages cancer cells and may be given before or after chemotherapy. When radiation is used to treat Hodgkin lymphoma, it typically is given after the completion of chemotherapy. Because radiation can result in harm to normal tissues and organs, even long after completion of the therapy, the focus has been on avoiding radiation as much as possible in the treatment of Hodgkin lymphoma. The use of PET scans has enabled treatment providers to identify children who respond well to the first two cycles (1 month each cycle) of chemotherapy treatment. Current research has shown that this group of patients may be cured with only chemotherapy (i.e., they do not receive any radiation) or with small doses of radiation in addition to chemotherapy. However, this is still under investigation and may not be the standard practice where your child is receiving treatment. Children who do not respond well to 2 months of chemotherapy may go on to receive radiation. If radiation therapy is necessary for your child, the radiation therapy doctor will explain to you exactly how the radiation will be given and how long the treatments will last. Most children experience very few side effects while they are receiving radiation therapy. However, some children may feel more tired, have a decreased appetite, or experience redness or darkness of the treated skin. The side effects most likely to occur typically depend on the area of the body being treated. In general, most side effects stop after treatment; however, side effects also can occur after treatment stops. Your child’s treatment team will explain any possible late effects in detail.

n HOW LONG WILL MY CHILD’S THERAPY LAST? The length of your child’s therapy will depend on the stage of the disease and its risk group. Usually, treatment is given for 2–6 months, but it can take longer depending on the specific treatment plan. The duration of treatment may be modified based on your child’s response to treatment after 4–8 weeks.

n WHAT IF THE CANCER RECURS? It is possible for Hodgkin lymphoma to recur, which is called a recurrence or relapse. If your child has a relapse, his or her treatment may involve additional chemotherapy, radiation therapy, newer agents like immunotherapy or targeted therapy, or stem cell transplant. Immunotherapy The immune system is the body’s natural defense against infection and disease. Immunomodulators are drugs that modify different parts of that system to boost its function, stop cancer cells from increasing in number, and stop the growth of new blood vessels that provide nutrients to the cancer. This new form of treatment may be used to treat classical Hodgkin lymphoma that was not cured by first-time treatment, and your treatment team may consider using it if additional chemotherapy fails. Because this type of treatment still is new, it usually is administered as part of a clinical trial. We will discuss what this means in the next section on page 8. Targeted Therapy Like immunotherapy, targeted therapy also is a new form of treatment. This class of drugs stops the action of molecules that help cancer cells grow by targeting these molecules on the surface of cancer cells. Targeted therapy is not yet widely used to treat Hodgkin lymphoma, but it may be a treatment option if the cancer recurs. The most common targeted therapy used in Hodgkin lymphoma is a drug called brentuximab. This drug binds to a specific protein on the Hodgkin-Reed Sternberg cell to help destroy it. Stem Cell Transplant Blood stem cells are cells that eventually develop into mature blood cells. The body makes stem cells in the bone marrow, which is the richest source of these cells. Stem cells also are present in the blood stream, although in small quantities, and their amount can be increased with certain drugs. In the treatment of cancer, stem cells are used to restore blood and immune cell formation after intense chemotherapy, radiation therapy, or both have been administered. The goal of stem cell transplant in the treatment of Hodgkin lymphoma is to replace the healthy bone marrow stem cells that are destroyed during a very intense form of chemotherapy called high-dose chemotherapy. Unlike the chemotherapy used for first-time treatment of cancer, this high-dose chemotherapy kills off cancer cells and wipes out the stem cells in the bone marrow, meaning they no longer are able to produce blood cells. During a stem cell transplant, stem cells are collected from a patient with Hodgkin lymphoma before high-dose chemotherapy is administered. After the high-dose chemotherapy, the patient’s collected stem cells are replaced through infusion so that they are able to function again.

n WHAT NEW METHODS OF TREATMENT ARE AVAILABLE? Hodgkin lymphoma has been studied more than any other type of lymphoma. With the many rapid advances in diagnosis and treatment, high survival rates have been reported in children with Hodgkin lymphoma using different treatment strategies. Children with stage I or II disease have a 95% survival rate. Children with stage III and IV disease typically have a survival rate near 90%. Most advances in the treatment of childhood cancer have been made through a process known as clinical trials. During clinical trials, the best-known (or standard) treatment for a cancer is compared with a new (or experimental) treatment that is believed to be at least as good as, and possibly better than, the standard treat - ment. Clinical trials enable doctors to determine whether new treatments are safe and effective. Clinical trials for Hodgkin lymphoma have focused on decreasing long-term side effects by reducing or eliminating radiation therapy and tailoring chemotherapy treatments. Participation in clinical trials is voluntary, and potential candidates must meet specific qualifications. Because clinical trials involve new treatment plans that are experimental, all of the possible risks of the treatment cannot be known ahead of time and unknown side effects may occur. However, children who participate in clinical trials can be among the first to benefit from new treatment approaches. Before making a decision about your child’s participation in a clinical trial, you should discuss all of the potential risks and benefits with your child’s treat- ment team. Additional information about clinical trials is available in the free booklet Taking Part in Clinical Trials: What Cancer Patients Need to Know by the National Cancer Institute (Publication No. 98-4250). To obtain the booklet and other useful information about childhood cancer, call 800.4CANCER (800.422.6237). The booklet also can be downloaded from the National Cancer Institute website at www.cancer.gov/clinicaltrials/learning. n WHAT ARE THE POTENTIAL LATE EFFECTS OF TREATMENT? Like all patients treated with chemotherapy and radiation, survivors of Hodgkin lymphoma may be at risk for developing side effects months or even years after treatment ends. Children who received radiation to the chest area as part of their cancer treatment are at risk for breast and cardiac complications. Other late effects may include delayed puberty in males, infertility, thyroid or lung problems, an increased risk of developing another cancer, delayed growth and development, and bone-health issues. Cancer survivors should seek appropriate cancer-related follow-up care to help them monitor their overall health after treatment ends. Many cancer centers throughout the country include survivor clinics that offer access to experts who can address a wide range of follow-up concerns. For more information about the specific long-term side effects of cancer treatment, visit curesearch.org.

n HOW CAN I WORK WITH MY CHILD’S TREATMENT TEAM? Your child’s care and treatment requires a team ap - proach. As a parent or guardian, you are an integral part of the team and your input is important. Because you know your child better than anyone else, the treatment team will need your help with managing your child’s disease. It is important to communicate openly with your child’s treatment team. Be sure to question your child’s oncology provider or nurse whenever there is anything you are not sure about. It often helps to write down your questions when you think of them. Examples

of some questions to ask include the following: • Has the cancer spread beyond the primary site? • What treatment options are available? • What treatment do you recommend and why? • What are the risks or side effects of the recommended treatment? • What should we do to prepare for treatment? • What is my child’s outlook for survival? • What are the chances of a recurrence? • If my child’s cancer recurs, are other treatment options available?

Use this space to write down some additional questions. _____________________________________________________________________ _____________________________________________________________________ _____________________________________________________________________ _____________________________________________________________________ _____________________________________________________________________ _____________________________________________________________________ _____________________________________________________________________ n ARE MY FEELINGS NORMAL? Learning that your child has cancer can be shocking and overwhelming. At first, you may not believe it or may hope the diagnosis is wrong. However, the changes you see in your child and the experience of being in the hospital and beginning treatment no doubt will confirm the reality of your child’s situation. Many family mem- bers feel they are somehow responsible for the child’s disease or feel guilty that they were not able to detect it

sooner. Remember that this disease was not caused or triggered by anything anyone did to the child, anything the child ate, or anything that happened during pregnancy. In addition to shock and guilt, you and your family may feel anger and sadness. Even the youngest members of your family are likely to be affected. These feelings are normal, and each family member will express them in different ways and at different times. It can be very difficult to feel so many strong emotions all at once. Talking honestly with each other about your feelings, reactions, and questions will help everyone in the family. It may seem difficult to talk to friends, family, or even medical staff, but expressing your feelings will help you cope with this situation. Your child also will benefit from family members continuing to show their care through support and communication.

n HOW CAN I HELP MY CHILD? You will notice frequent changes in your child during his or her treatment for Hodgkin lymphoma. These changes or symptoms may make you feel even more helpless. It is im- portant to remember that despite changes on the outside, your child is still the same person on the inside. Hair loss and other changes in physical appearance are temporary and often bother adults more than the child or his or her siblings and friends. All of your feelings about what your child is going through during treatment for cancer must be balanced by remembering that treatment provides an op- portunity to cure the disease so your child can go on to live a full and meaningful life. It is important to reinforce to your

child that nothing he or she did or said caused this disease. Telling your child that your angry or sad feelings are directed at the cancer and not at him or her will help preserve honesty and closeness in your relationship. Like you, your child will need someone with whom to share feelings. Do not hesitate to ask your child to express his or her feelings and do not be afraid to explain what is happening and why. In spite of the disease, your child is still developing and learning. All children, both sick and well, need love, attention, discipline, limits, and the opportunity to learn new skills and try new activities. As you learn about your child’s special needs, it is important to remember that he or she also has all of the needs and rights of any other growing, developing person. Many patients with Hodgkin lymphoma are adolescents, which presents special considerations. Do not avoid using direct terms and explanations with your child. Children will tolerate treatment better if they understand it and are allowed to be active decision makers whenever possible. The same is true for parents and family members.

n IS MY CHILD’S DIET DURING TREATMENT IMPORTANT? Yes. We know from research that well-nourished children tolerate therapy better and have fewer treatment delays from illness. It may be difficult for your child to resume normal eating habits while receiving therapy, so you will need to be flexible and creative. Your child’s food preferences and tastes may change throughout therapy. Often, numerous small meals are better tolerated than three large ones. Children usually are more interested in eating foods they helped prepare. It is important to include your child in the social activity of fam- ily meals even if he or she does not eat full meals. Remember, nobody wins food fights—it is best not to force your child to eat. Make sure foods that are high in protein and carbohydrates are readily available. Your treatment team should approve multivitamins, herbs, supplements, and all other medi- cines before you give them to your child because they may cause an interaction with your child’s chemotherapy. A dietitian trained in the calorie and energy needs of children with cancer can offer you guidance. Your child’s treatment team also can intervene if there is a nutritional problem.

n CAN MY CHILD ATTEND SCHOOL DURING TREATMENT? Most children who are being treated for Hodgkin lymphoma can attend school. However, some children may not tolerate chemotherapy and radiation as well as others and may need to miss school on occasion. You should discuss school attendance with your child’s oncology team. School is important because it helps children and adolescents maintain social contact with their peers. It is important for your child to return to school as soon as he or she is medically able to do so.

Suggested Resources www.curesearch.org National Childhood Cancer Foundation and the Children’s Oncology Group www.cancer.gov National Cancer Institute www.cancer.gov/clinicaltrials/learning Webpage from which the "Taking Part in Clinical Trials: What Cancer Patients Need to Know" booklet can be downloaded.

NOTES

Association of Pediatric Hematology/Oncology Nurses

8735 W. Higgins Road, Suite 300 Chicago, IL 60631 847.375.4724 • Fax 847.375.6478

info@aphon.org www.aphon.org

Manual para la familia

Linfoma de Hodgkin

Serie oncologia

Manual para la familia

Linfoma de Hodgkin

Serie oncologia

Linfoma de Hodgkin MANUAL PARA LA FAMILIA

Autora Susanne B. Conley, MSN RN AOCNS CPON®

Colaboradora Amy L. Billett, MD

Revisor por parte de los padres Jerry Loew

Revisor del contenido Steering Council

Este manual ha sido publicado por Association of Pediatric Hematology/Oncology Nurses (APHON) con fines educativos únicamente. El contenido ha sido desarrollado por fuentes confiables y no pretende ser el único tratamiento aceptable o seguro para el linfoma de Hodgkin. Bajo ciertas circunstancias o condiciones, se puede requerir un tratamiento adicional o diferente. Es posible que a medida que nuevas investigaciones y experi- encias clínicas amplíen las fuentes de información sobre el tratamiento del linfoma de Hodgkin, sea necesario hacer ajustes en el tratamiento y la terapia con fármacos. APHON no garantiza ni asegura ninguna declaración, expresa o implícita, con respecto a la validez o suficiencia de los tratamientos o a la información relacionada contenida en este manual. APHON proporciona este manual para uso educativo. Los usuarios sólo podrán copiar y distribuir este material en su forma no modificada, para uso no comercial y con atribución a APHON.

n ¿QUÉ ES EL LINFOMA DE HODGKIN? El linfoma de Hodgkin, también conocido como enfermedad de Hodgkin, es un cáncer del sistema linfático. El sistema linfático es parte del sistema inmunitario del cuerpo que es el que lo protege de infecciones y enferme- dades. El sistema linfático está formado por una red de tejidos y órganos que incluye los ganglios linfáticos, las amígdalas, la médula ósea, el bazo y el timo. El tejido linfático también se encuentra en otras partes del cuerpo, como el intestino, la piel y el cerebro. El sistema linfático promueve la circulación por todo el cuerpo de un líquido acuoso llamado linfa. Este líquido linfa, incluye un tipo de glóbulos blancos llamados linfocitos que ayudan al sistema inmunitario a filtrar virus, bacterias y otras sustancias extrañas al cuerpo. Ya que el tejido linfático se encuentra en todo el cuerpo, el linfoma de Hodgkin puede desarrollarse en casi cualquier lugar y extenderse a casi cualquier tejido u órgano. Es un cáncer que se desarrolla a partir de un linfo- cito anormal (glóbulo blanco) que se divide demasiado rápido y crece sin orden ni control. El diagnóstico de linfoma de Hodgkin depende de la presencia de un tipo particular de células sanguíneas anor- males llamadas células de Reed-Sternberg. Estos linfocitos grandes y anormales se observan cuando se examina con un microscopio el tejido afectado de una persona con linfoma de Hodgkin.

El sistema linfático

n ¿CUÁLES SON ALGUNOS SÍNTOMAS DEL LINFOMA DE HODGKIN? La mayoría de los pacientes con linfoma de Hodgkin presentan ganglios linfáticos inflamados que generalmente son indoloros, firmes y móviles. Estos ganglios se encuentran con mayor frecuencia en el cuello y el pecho y con menor frecuencia debajo de los brazos o en la ingle. Otros síntomas del linfoma de Hodgkin incluyen: • Fiebre • Sudores nocturnos • Pérdida de peso sin razón aparente • Fatiga • Dificultad para respirara • Picazón en la piel n ¿QUÉ CAUSA EL LINFOMA DE HODGKIN? Se desconocen cuáles son las causas del linfoma de Hodgkin. Las personas tienen mayor riesgo de desarrollar linfoma de Hodgkin cuando tienen lo siguiente: • El virus de Epstein-Barr (EBV), causante de la mononucleosis infecciosa, a menudo llamada «mono» (aunque, la mayoría de las personas con infección por EBV nunca desarrollan linfoma de Hodgkin) • El virus de inmunodeficiencia humana (HIV) • Antecedentes familiares de linfoma de Hodgkin (aunque los casos hereditarios son muy raros). n ¿QUIÉN PUEDE CONTRAER EL LINFOMA DE HODGKIN? El linfoma de Hodgkin es una enfermedad rara, que representa aproximadamente el 5% de los cánceres infan- tiles en los Estados Unidos. Raramente ocurre en pacientes menores de 5 años, pero el riesgo de desarrollar linfoma de Hodgkin aumenta en la segunda década de la vida, lo que lo convierte en uno de los cánceres más comunes en adolescentes (generalmente entre las edades de 15 y 19 años) y adultos jóvenes. n ¿EL LINFOMA DE HODGKIN SE HEREDA? Aunque es raro, se han reportado algunos casos de linfoma de Hodgkin en familias de ciertos grupos étnicos. Esto sugiere que cierta predisposición genética puede aumentar el riesgo de desarrollarlo. Existe una proba- bilidad un poco mayor de que el linfoma de Hodgkin ocurra en hermanos y primos de aquellos que tienen o han tenido la enfermedad. Aunque el riesgo en hermanos es de más o menos dos a cinco veces mayor que en aquellos que no son hermanos, la probabilidad de que un hermano contraiga linfoma de Hodgkin sigue siendo muy baja.

n ¿QUÉ ES LA METÁSTASIS? La metástasis es la propagación de un tumor desde su ubicación original (el sitio primario) a otras partes del cuerpo. El linfoma de Hodgkin a menudo se propaga de un ganglio linfático a otro y también puede extenderse a otros órganos fuera del sistema linfático. Si una biopsia revela la presencia de linfoma de Hodgkin, se realizarán pruebas adicionales para determinar si el cáncer se ha diseminado a otras partes del cuerpo. n ¿QUÉ ES LA ESTADIFICACIÓN? La estadificación es el proceso que determina la ubicación y alcance de una enfermedad en el momento del diagnóstico. La información obtenida del proceso de estadificación determina el alcance de la enfermedad y ayuda a determinar el tratamiento. Existen cuatro etapas en el linfoma de Hodgkin que están determinadas por la ubicación de las cadenas de ganglios linfáticos que tienen tumores. Un número más alto en la etapa indica que la enfermedad se ha extendido aún más por todo el cuerpo. Estadificación del linfoma de Hodgkin Dentro de cada etapa, las letras “E” y “S” se utilizan para clasificar aún más la enfermedad. La “E” indica que el cáncer se ha extendido a un órgano o tejido que no es parte del sistema linfático, pero que está al lado de un ganglio linfático afectado. La “S” indica que se encontró cáncer en el bazo. ETAPA I • Etapa I: el cáncer se encuentra en un grupo de ganglios linfáticos. • Etapa IE: el cáncer se encuentra en un grupo de ganglios linfáticos y se ha extendido a un órgano o tejido cercano que no forma parte del sistema linfático. ETAPA II • Etapa II: el cáncer se encuentra en dos o más grupos de ganglios linfáticos en el mismo lado del diafrag- ma (el músculo respiratorio que separa las áreas del pecho y el abdomen). • Etapa IIE: el cáncer se encuentra en dos o más grupos de ganglios linfáticos en el mismo lado del diafragma y se ha extendido desde uno de esos ganglios linfáticos hasta un órgano o tejido cercano que no forma parte del sistema linfático. ETAPA III • Etapa III: el cáncer se encuentra en ambos lados del diafragma. • Etapa IIIE: el cáncer se encuentra en ambos lados del diafragma y se ha extendido desde uno de estos ganglios linfáticos hasta un órgano o tejido cercano que no forma parte del sistema linfático. • Etapa IIIS: el cáncer se encuentra en ambos lados del diafragma y en el bazo. • Etapa IIIE+S: el cáncer está en ambos lados del diafragma y el bazo y se ha extendido de uno de estos grupos de ganglios linfáticos a un órgano o tejido cercano que no forma parte del sistema linfático.

ETAPA IV • Etapa IV: el cáncer está en uno o más órganos que no forman parte del sistema linfático (p. ej., pulmones, huesos o médula ósea) y puede estar en los ganglios linfáticos que están cerca o lejos de esos órganos. Cada etapa se clasifica además en “A” o “B”. La “A” significa que no hay síntomas específicos. La “B” indica que el niño ha experimentado síntomas específicos antes del diagnóstico. Estos síntomas específicos de “B” incluyen fiebre inexplicable que excede los 100.4 ºF / 38.0 ºC, sudoración nocturna severa o pérdida de peso de al menos 10% del peso corporal del individuo durante un período de 6 meses. Grupos de riesgo Además de la estadificación, el linfoma de Hodgkin se divide en grupos de riesgo según la información obtenida durante la estadificación, el tamaño y la ubicación del tumor, y si el paciente tiene síntomas “B”. Una vez que se determina el grupo de riesgo, se utiliza esta información para decidir qué tipo de tratamiento se necesita. n ¿QUÉ PRUEBAS Y PROCEDIMIENTOS NECESITARÁ MI HIJO(A)? Se harán varias pruebas a los ganglios linfáticos, tórax y abdomen para diagnosticar el linfoma de Hodgkin y saber su alcance. Un bazo o hígado agrandado indica que el linfoma de Hodgkin está presente en esos órganos. La presencia de ganglios linfáticos pequeños y blandos en niños puede ser engañosa ya que sólo los ganglios linfáticos que han aumentado de tamaño o se han agrandado significativamente son motivo de preocupación. Para determinar el mejor tratamiento posible, se debe identificar el tipo exacto de linfoma de Hodgkin y dónde está ubicado en el cuerpo. El médico de tu hijo(a) decidirá qué pruebas y procedimientos se necesitan.

Pruebas y procedimientos más comunes BIOPSIA DE TEJIDO

Es necesaria la extirpación total o parcial de un ganglio linfático u otro tejido para diagnosticar el linfoma de Hodgkin. La biopsia de tejido de ganglios linfáticos se puede realizar en una sala de operaciones o en el departamento de radiología del hospital. Siempre se tendrá en cuenta el nivel de comodidad y ansiedad de tu hijo(a) al planificar procedimientos que involucren agujas. Teniendo ya la muestra de la biopsia, un patólogo examinará el tejido bajo un microscopio para buscar células cancerosas, específicamente células de Reed-Sternberg, que son linfocitos anormales comunes en el linfoma de Hodgkin.

EXAMEN DE RAYOS X Una radiografía de tórax, que toma una imagen de los órganos y huesos dentro del tórax, puede ser necesaria para determinar si el linfoma de Hodgkin está presente en el área media superior del tórax (el mediastino). Esta es el área justo encima del corazón pero debajo del cuello, y es un sitio común para el linfoma de Hodgkin. Una radiografía de tórax también puede ayudar a determinar si los pulmones están afectados.

TOMOGRAFÍA COMPUTARIZADA (CT) La tomografía axial computarizada (CT) es una radiografía asistida por computadora que produce imágenes muy detalladas de áreas internas del cuerpo desde diferentes ángulos. Es indolora y rápida, pero el niño debe per- manecer completamente quieto. Los más pequeños necesitarán sedación para estar quietos. Es posible que a tu hijo(a) no se le permita comer o beber durante varias horas antes del examen, y para que las imágenes sean más nítidas se le inyectará una pequeña cantidad de tinte en una vena o se le dará a beber un líquido con un tinte sin sabor. Usualmente, ninguno de estos tintes tiene efectos secundarios, pero puede haber reacciones alérgicas. Estas tomografías se realizan al momento del diagnóstico y en diferentes momentos durante el tratamiento para determinar cómo está respondiendo tu hijo(a). TOMOGRAFÍA POR EMISIÓN DE POSITRONES (PET)) La tomografía por emisión de positrones (PET) crea imágenes que muestran el tamaño y la forma de los órganos y los tumores. La PET es como la tomografía computarizada (CT) y la resonancia magnética (MRI), excepto que también muestra cambios químicos y funcionales dentro del cuerpo. El proceso tiene tres pasos: inyección de un material radiactivo; período de espera, generalmente de 30 a 60 minutos, y escaneo con la máquina PET. Duran- te el tiempo entre la espera y el escaneo, tu hijo(a) debe estar completamente quieto(a). El proceso es indoloro, auque algunos niños necesitan sedación para permanecer quietos. Al igual que la CT, se debe obtener una PET en el momento del diagnóstico y para evaluar la respuesta de tu hijo(a) a medida que avanza el tratamiento. BIOPSIA Y ASPIRACIÓN DE MÉDULA ÓSEA Algunos niños pueden necesitar una biopsia y aspiración de médula ósea, que es un procedimiento que consiste en recolectar una muestra de médula ósea. La médula ósea se encuentra en la parte hueca del hueso y es donde se forman los glóbulos blancos (que luchan contra las infecciones), los glóbulos rojos (que transportan oxígeno) y las plaquetas (células que ayudan a la correcta coagulación). Una vez que se recolecta la muestra de médula ósea, un patólogo la examina al microscopio para ver si hay linfoma de Hodgkin. Los niños generalmente requieren sedación o anestesia durante este procedimiento porque se realiza con una aguja de biopsia.

Otras pruebas y procedimientos que pueden realizarse RESONANCIA MAGNÉTICA (MRI) La resonancia magnética (MRI) reproduce imágenes muy exactas de órganos y tumores dentro del cuerpo. Durante este proced- imiento, tu hijo(a) estará acostado(a) sobre una mesa que se mueve hacia una máquina en forma de tubo que lo(a) rodeará con un campo magnético. Aunque es indoloro, la máquina hace un ruido fuerte que puede asustar a algunos niños. Tu hijo(a) debe estar completamente quieto(a) durante el procedimiento, si no puede hacerlo, es posible que lo/la seden. ANÁLISIS DE SANGRE Éstos se realizan para controlar las células sanguíneas, las sales corporales y ciertas proteínas o compuestos en la sangre. Se usan principalmente para controlar la respuesta de tu hijo(a) al trata- miento y los posibles efectos secundarios de la terapia. Un conteo sanguíneo completo ayuda a detectar disminuciones en la cantidad de glóbulos rojos, glóbulos blancos o plaquetas en la sangre. Las

pruebas de química sanguínea, como las que verifican los niveles de nitrógeno ureico en sangre y creatinina, se realizan para detectar cambios en la función renal. Se hacen otras pruebas, como las que controlan los niveles indi- viduales de alanina aminotransferasa, aspartato aminotransferasa y bilirrubina, para evaluar la función hepática. Se pueden hacer análisis de sangre, como los que controlan las tasas de sedimentación de eritrocitos y la proteína C reactiva, que a veces son elevadas en los casos de linfoma de Hodgkin, para medir la respuesta al tratamiento. Durante la primera visita de tu hijo(a) al hospital o clínica, se le pueden hacer análisis de sangre para descartar otras enfermedades, pero estas pruebas no son diagnósticas del linfoma de Hodgkin. DISPOSITIVO DE ACCESO VENOSO (VAD) Generalmente se necesita quimioterapia para tratar el linfoma de Hodgkin, y hay varias opciones. Ya que el tratamiento para el linfoma de Hodgkin a menudo es breve, se puede insertar temporalmente un dispositivo lla- mado catéter central colocado por vía periférica (PICC). Se inserta un pequeño tubo de plástico llamado línea o dispositivo de acceso venoso (i.e., un puerto) en un vaso sanguíneo grande, generalmente debajo de la clavícula de tu hijo(a). Estos VAD se pueden usar para análisis de sangre y para administrar medicamentos, quimioterapia, productos sanguíneos y apoyo nutricional cuando sea necesario. El equipo de tratamiento y tú decidirán si es necesario un VAD, el cual generalmente se deja durante el tratamiento y se elimina al finalizar la terapia.

n ¿CÓMO SE TRATA EL LINFOMA DE HODGKIN? El linfoma de Hodgkin es uno de los cánceres más tratables, con una tasa de supervivencia a largo plazo de más del 90% después del tratamiento. El objetivo del tratamiento para el linfoma de Hodgkin es curar la enfermedad y minimizar los efectos o reacciones secundarios adversos relacionados con el tratamien- to. La quimioterapia y la radioterapia son las dos terapias más comúnmente utilizadas para tratar el linfoma de Hodgkin.

Quimioterapia La quimioterapia son medicamentos que ayudan a eliminar las células cancerosas y evitan que el cáncer se propague. Debido a que un medicamento de quimioterapia no puede controlar la enfermedad por sí solo, se ad- ministra una combinación de varios medicamentos para eliminar las células cancerosas. La combinación exacta de agentes de quimioterapia dependerá de la etapa de la enfermedad y de otras consideraciones. Muchos de estos medicamentos se administran a través de una vena o VAD, aunque algunos se dan vía oral. La mayoría de los tratamientos de quimioterapia se hacen en una clínica ambulatoria, aunque algunos pueden requerir hospi- talización. Monitorear de cerca a tu hijo(a) es una prioridad cuando recibe quimioterapia. El equipo te explicará los posibles efectos secundarios de los medicamentos de quimioterapia específicos que recibe tu hijo(a). Radioterapia La radioterapia utiliza un tipo especial de tratamiento con rayos X que mata o daña las células cancerosas y se puede aplicar antes o después de la quimioterapia. En el linfoma de Hodgkin, generalmente se aplica después de la quimioterapia. Como la radiación puede causar daño a los tejidos y órganos normales, aún mucho después de completar la terapia, se intenta evitar la radiación tanto como sea posible. La tomografía PET permite identificar a los niños que responden bien a los primeros dos ciclos (1 mes cada ciclo) de tratamiento de quimioterapia. Investigaciones han demostrado que este grupo de pacientes puede curarse sólo con quimiote- rapia (i.e, sin recibir radiación) o con pequeñas dosis de radiación además de la quimioterapia. Esto aún se

está investigando y puede que no se practique donde tu hijo(a) recibe tratamiento. Los niños que no responden bien a 2 meses de quimioterapia pueden recibir radiación. Si tu hijo(a) necesita radioterapia, el radioterapeuta te explicará cómo se administrará la radiación y cuánto durarán los tratamientos. En general hay pocos efectos secundarios durante la radioterapia, aunque algunos niños pueden sentir cansansio, menos apetito o presentar enrojecimiento u oscurecimiento en la piel tratada. Los efectos secundarios dependen del área del cuerpo que se está tratando y cesan después del tratamiento. Sin embargo, los efectos secundarios pueden ocurrir después de que se suspende el tratamiento. El equipo de tratamiento de tu hijo(a) te explicará los posibles efectos tardíos en detalle.

n ¿CUÁNTO DURARÁ LA TERAPIA DE MI HIJO(A)? La duración de la terapia dependerá de la etapa de la enfermedad y del grupo de riesgo. Por lo general, el tratamiento se da durante 2 a 6 meses, pero puede tomar más tiempo dependiendo del plan de tratamiento específico. La duración puede cambiar según la respuesta de tu hijo(a) al tratamiento después de 4 a 8 semanas.

n ¿QUÉ PASA SI EL CÁNCER REAPARECE? Es posible que el linfoma de Hodgkin reaparezca, lo que se conoce como recurrencia o recaída. Si tu hijo(a) tiene una recaída, su tratamiento puede incluir quimioterapia adicional, radioterapia, agentes más nuevos como la inmunoterapia o la terapia dirigida o trasplante de células madre. Inmunoterapia El sistema inmunitario es la defensa natural del cuerpo contra infecciones y enfermedades. Los inmunomodu- ladores son medicamentos que modifican diferentes partes de este sistema para aumentar su función, evitar que las células cancerosas aumenten en número y detener el crecimiento de nuevos vasos sanguíneos que nutren al cáncer. Este tratamiento se usa para tratar el linfoma de Hodgkin clásico que no se curó con el primer tratamiento, y se puede considerar usarlo si falla la quimioterapia adicional. Como este tipo de tratamiento aún es nuevo, se administra como parte de un ensayo clínico. De esto hablaremos en la siguiente sección, página 8. Terapia dirigida Al igual que la inmunoterapia, la terapia dirigida es una nueva forma de tratamiento. Este tipo de medicamentos detiene la acción de las moléculas que ayudan al crecimiento de las células cancerosas atacándolas en la super- ficie de las células cancerosas. La terapia dirigida aún no es muy usada, pero puede ser una opción si el cáncer reaparece. El medicamento más usado en esta terapia dirigida para el linfoma de Hodgkin es el Brentuximab que se une a una proteína específica en la célula de Reed-Sternberg para ayudar a destruirla. Trasplante de células madre Las células madre sanguíneas son células que se convierten en células sanguíneas maduras. El cuerpo produce células madre en la médula ósea, que es la fuente más rica de estas células. También están en el torrente san- guíneo en pequeñas cantidades; su cantidad puede aumentar con ciertos medicamentos. En el tratamiento del cáncer, las células madre se utilizan para restaurar la sangre y formar células inmunes después de una quimiote- rapia intensa, radioterapia o ambas.

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