Pediatric Non-Hodgkin’s Lymphoma
Children and adolescents living with Non-Hodgkin’s Lymphoma benefit from a full evaluation before receiving treatment.
Background
Non-Hodgkin Lymphoma Overview
Cancer develops when cells in the body grow out of control. Any cells in your body can become cancer and spread to other areas of the body. Childhood non-Hodgkin lymphoma (NHL) is a particular type of cancer that forms in the lymph system. The lymph system is part of the body’s immune system. The immune system plays an important role in protecting the body from foreign substances, infection, and diseases. The lymphatic system consists of a fluid (lymph), vessels that transport the lymph, and organs that contain lymphoid tissue. The cancerous cell in
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NHL is called a lymphocyte and its normally a cell that helps protect the body from infection and is involved in the immune response. With NHL, these lymphocytes divide rapidly and cause the lymph nodes to become enlarged or other organs in the lymph system and are responsible for the symptoms experienced by children with NHL. Lymphomas are the third most common childhood cancer in the U.S. Around 800 cases of NHL are diagnosed in the US each year and can occur at any age from infancy to adulthood. NHL affects males more than females and is common in white children mostly. (Shankland, Armitage, & Hancock, 2012) Common Types of NHL NHL in children usually falls in one of three types: Lymphoblastic non-Hodgkin lymphoma, Burkitt’s and non Burkitt’s lymphoma, and Large cell or diffuse histiocytic non-Hodgkin lymphoma.
30 percent of the cases are Lymphoblastic non-Hodgkin lymphoma and they usually involves the T-cells, and typically presents with a mass in the chest, swollen lymph node, with or without bone marrow and central nervous system involvement. Burkitt's and non-Burkitt's lymphoma are fast-growing lymphomas in which the cells are undifferentiated and diffuse. This has also been referred to as small noncleaved cells. Burkitt's and non-Burkitt's lymphoma accounts for about 40 percent of the cases. They are usually characterized by a large abdominal tumor and may have bone marrow and central nervous system involvement. Large cell or diffuse histiocytic non-Hodgkin lymphoma. Large cell or diffuse histiocytic non-Hodgkin involves the B-cells or T-cells and accounts for about 25 percent of the cases. Children with this type of non-Hodgkin lymphoma usually have lymphatic system involvement, as well as a non-lymph structure involvement. Anaplastic large cell lymphoma is a type of large cell lymphoma in children. Large cell lymphomas usually do not grow as quickly as other lymphomas in
children. (Dokmanovic, 2013) Risk Factors of NHL in Children The specific cause of non-Hodgkin lymphoma is unclear. It is possible that genetics and exposure to viral infections may increase the risk for developing this malignancy. Non-Hodgkin lymphoma has also been linked to chemotherapy and radiation therapy. Non-Hodgkin may be a second malignancy as a result of the treatment for certain cancers. There has been much investigation into the association of the Epstein-Barr virus that causes the mononucleosis infection, as well as HIV, which causes AIDS. Both of these infectious viruses have been linked to the development of Burkitt's lymphoma. Children and adults with certain hereditary immune system abnormalities have an increased risk of developing non-Hodgkin lymphoma, including patients with ataxia telangiectasia, X-linked lymphoproliferative disease, or Wiskott-Aldrich syndrome. People who have had organ transplants and need to take medicines to suppress their immune systems are also at increased risk. Signs & Symptoms of NHL in Children In many cases, non-Hodgkin lymphoma in children may not cause symptoms until it has grown or spread. Most children have stage III or IV disease at the time of diagnosis because of the sudden onset of symptoms and the fact that these lymphomas tend to grow very quickly. The disease can sometimes progress quickly from a few days to a few weeks. A child can go from otherwise healthy to having multisystem involvement in a short time period. Some children with non-Hodgkin lymphoma have symptoms of an abdominal mass and have complaints of abdominal pain, fever, constipation, and decreased appetite due to the pressure and obstruction a large tumor in this area can cause. Some children with non-Hodgkin lymphoma have symptoms of a mass in their chest and have complaints of respiratory problems, pain with deep breaths (dyspnea), cough, and/or wheezing. Lymphomas in the chest can also press on a main blood vessel (the superior vena cava), which can cause swelling and a bluish coloration in the head and arms. If left unchecked, it can also affect the brain and may even be life-threatening. Symptoms may include: Painless swelling of the lymph nodes in neck, chest, abdomen, underarm, or groin, fever, sore throat, fullness in groin, bone and joint pain, night sweats, fatigue, weight loss or decreased appetite, itching of the skin, or reccuring infections. The symptoms of non-Hodgkin lymphoma may resemble other blood disorders or medical problems. Diagnostic Tests Determining what type of lymphoma a patient has and where it is located in the body is important information for both the physician and the patient. An accurate diagnosis of childhood non-Hodgkin lymphoma (NHL) requires a number of tests. Physicians may use some or all of the following tests as well as the patient’s medical history and results from the physical examination to assess the best course of treatment: blood tests, biopsy, imaging tests, bone marrow examination and lumbar puncture. Staging NHL in Children Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging symptoms that are used for non-Hodgkin lymphoma. Always consult your child's doctor for information on staging. One method of staging non-Hodgkin lymphoma is the following: Stage I: the lymphoma is at one site, either nodal or elsewhere in the body, but not in the chest or abdomen. Stage II: the lymphoma is at two or more sites on the same side of the body, above or below the diaphragm, but not in the chest. Stage III: the lymphoma is in the chest or spine, or it has spread widely in the abdomen, or it is both above and below the diaphragm, but it does not involve bone marrow or the central nervous system. Stage IV: any lymphoma that has bone marrow and/or central nervous system involvement when it is first found. Treatment The initial therapy and intensity of treatment indicated for a patient are based on the subtype and stage of disease. In general, the goal of treatment is to destroy as many lymphoma cells as possible and to induce a “complete remission”; that is, to eliminate all evidence of disease. Patients who go into remission are sometimes cured of their disease. Treatment can also keep NHL in check for many years, even though imaging or other studies show remaining sites of disease. is situation may be referred to as a “partial remission.” In general, chemotherapy and radiation therapy are the two principal forms of treatment for NHL. Although radiation therapy is not often the sole or principal curative therapy, it is an important additional treatment in some cases. Stem cell transplantation and a watch-and-wait strategy are also used to treat some NHL subtypes. Other forms of treatment are emerging, and some are already approved for specific forms of NHL. Many other new therapies are under investigation in clinical trials. Children with non-Hodgkin lymphoma (NHL) may have side effects, also called toxicity, from the cancer treatment they receive. Fortunately, there are many effective ways to make them more tolerable. Parents and patients should ask their healthcare team about possible treatment side effects and inform the physician if the patient experiences any. In many cases, side effects can be lessened with medications or lifestyle changes. (Gera & Saah, 2012) Literature Review Burkhardt and Lenz (2015) The researchers examined the treatment of non-Hodgkin Lymphoma (NHL) in pediatrics and adults. The World Health Organization defines four groups of NHL including 69 subtypes and uncounted numbers of variants of these subtypes. They investigated the relationship between the NHL subtypes and the spectrum of NHL occurring in pediatrics and how it greatly differs from that in adults. It was found that among the NHL patient populations, the overlapping of histological subtypes was extremely limited. In children and adolescents, Burkitt lymphoma and leukemia, lymphoblastic lymphoma (LBL), diffuse large B-cell lymphoma (DLBCL) and ALK positive anaplastic large cell lymphoma (ALCL) cover roughly 90% of all NHL patients. With the exception of DLBCL, all these typical subtypes for pediatrics are found to be rare subtypes in adults. In the realm of NHL diagnosis and staging, it was found that the compared data for pediatrics and adults was often weakened by the use of different staging systems. The St. Jude staging system is used for children and adolescents whereas NHL is adults is staged according the Ann Arbor staging system. The main differences between the two is the definition of advanced stage diseases. The implementation of reference pathology reduced the number of pediatric patients being misdiagnosed and mistreated. This has had a huge impact on clinical relevance being that treatment regimens that are used for pediatric NHL patients differ according to the NHL subtypes. For all the histological subtypes discussed, new drugs are on the way or already being approved. These new drugs can possibly be combined with current standard treatment to improve the outcome. On the other hand, these new drugs might serve as a replacement for more toxic treatment and might even reduce toxicity without impairment of disease-free survival. Researchers found that the process of implementing new drugs into treatment and making drugs available to the patients with NHL varied across the patients age groups substantially. It was also found that the pharmaceutical companies need to strengthen their basis of conduction in the pediatric clinical trial groups. In conclusion, it was found that both pediatric and adult oncologists can benefit from discussions concerning results and experiences in clinical trials as well as respect to critical aspects of infrastructure. Cairo et al. (2012) The researchers analyzed the impact of age and other diagnostic factors on the risk of treatment failure in children and adolescents treated on the French-American-British Mature B-Cell Lymphoma 96 (FAB LMB 96) trial. Adolescents compared to younger children with mature B-cell NHL have been considered to have an inferior prognosis. Mature B-cell non-Hodgkin’s lymphoma (NHL), including Burkitt lymphoma (BL), Burkitt leukemia, diffuse large B-cell lymphoma (DLBCL), and primary mediastinal B-cell lymphoma (PMBL) make up approximately 60% of all malignant NHLs that occur in children and adolescents. It was found that over the past 25 years there has been a reported 99% survival rate in patients with limited-risk, 90% survival rate in patients with intermediate-risk, and an approximate 70% to 80% overall survival rate in children with advanced-risk mature B-cell NHL. Fridrik, Hausmaninger, Lang, Drach, and Krieger (2010)
Hodgkin 's Lymphoma occurs when following a mutation in the lymphocyte DNA. The mutation occurs after birth, meaning that Hodgkin’s disease is not hereditary. The mutated DNA can lead to the uncontrolled growth of cancerous lymphocytes if untreated. The cancerous lymphocytes produce tumor masses in the lymph
Pediatric oncology has been so very rewarding in many ways, but also so very cruel in a few ways. The good days are great, but the sad days are heartbreaking. But beyond the death and the suffering, there is a whole other layer of
Lymphoid tissue is made up of cells called lymphocytes, a type of white blood cell that fights infection. There are 2 major types of lymphocytes: B lymphocytes (B cells) and T lymphocytes (T cells). Normal T cells and B cells have different jobs. But because all the importance of these cells they can travel around the body spreading the cancerous cells. There are four stages for Hodgkin’s lymphoma when testing is completed the doctors can determine what stage the cancer is at and what treatment will be best suited to fighting the disease.
Hodgkin’s lymphoma is a form of cancer in the lymphatic system. This type of lymphoma originates in the white blood cells, and spreads throughout the lymph nodes in the body. The cause of Hodgkin’s lymphoma is unknown, and it is sometimes found by chance. This disease is typically treated with chemotherapy and radiation and, if found in the early stages, the patient has a good chance of being fully cured.
Non-small-cell lung cancer is more common than small-cell lung cancer. It makes up about 80 percent of all diagnosed lung cancers. It is actually four different kinds of lung cancers that grow and respond to treatment in about the same way. These four types of non-small-cell lung cancer are termed squamous cell, adencarcinoma, large cell, and mixed cell. Two other related cancers that are also included in the non-small-cell lung cancers group are carcinoid and mesothelioma. Non-small-cell lung cancer grows more slowly than small-cell lung cancer. Non-small-cell lung cancer tends to grow first in the area where it started then invades the surrounding lung tissue and ribs. It eventually spreads to other areas of the body, mainly bones, brain, and liver.
Diagnosing these cancers is very tricky because they tend to have a lot of the same symptoms, not only to themselves but also to other diseases. Lymphoma is usually misdiagnosed as IBS and as a conciquece of such with the treatment for IBS usually causes the cancer to further spread and develop it's self. (Science Weekly, 2012) Just like in human cancers when finding a lump under the skin on any part of the body it is important to go the the vet and get them checked out. The most comon way of diagnosing cancers is by a biopsy, which is taking a part of flesh from the suspected affected area and testing it for any cancerous cells. Most cancers are misdiagnosed or go undiagnosed completely because their symptoms often resemble other diseases which are easier treated, and more common.
Elphee, E. E. (2008). Understanding the Concept of Uncertainty in Patients With Indolent Lymphoma. Oncology Nursing Forum, 35(3), 449-454.
Steen, Grant R. and Joseph Mirro. Childhood Cancer: A Handbook From St.Jude Children's Research Hospital . Cambridge, MA: Perseus Publisher, 2000.
Kids are meant to be happy, play outside, go to school, and have fun. They aren’t meant to sit in hospitals, losing weight by the pound, carrying around IV poles filled with poison. It’s ridiculous and immature that we don’t have a cure for childhood cancer. The only “treatment” that we have is chemotherapy- a chemical that seems to help fight off cancer. Chemo doesn’t just fight off cancer cells though- it fights off healthy cells in your blood, mouth, digestive system, and hair follicles. The most frustrating thing about childhood cancer is that only 4% of federal funding is exclusively dedicated to childhood cancer research. It is true that more adults get diagnosed with cancer than kids, but does that mean that adults are 96% more important than children? The average age of diagnosis for an adult with cancer is age 67, and the average number of years lost is 15. 15 years are definitely many years, but not that many compared to the average number of years lost for a child- 71. Also, age 67 is a lot older than the average age of diagnoses for a child- age 6. At least the adults get to grow up and have the ability to even have cancer- some of these kids can’t even get through a fifth of their lives.
Non-Hodgkin’s Lymphoma is the classification of any type of malignant lymphoma other than Hodgkin’s Disease, including histiocytic lymphoma and lymphocytic lymphomas.
No matter who it happens to, any type of cancer is heart-breaking. However, one’s heart seems to crack a little bit deeper when you hear a child has been diagnosed. Several forms of cancer can arise during childhood. The most common is acute lymphoblastic/lymphocytic leukemia (ALL). In fact, it is so common between the ages 0-14, that people refer to it as childhood acute lymphoblastic leukemia (Kanwar, 2013). .
How does one see the symptoms for childhood cancer? First one must know that there are many different types. There are forty different types of children’s cancer, including: Leukemia, Lymphoma, Sarcomas, cancers of the nervous system, liver cancers, kidney cancer, and more. Out of these cancers, the two most common childhood cancers are Leukemia, and brain tumors. What is leukemia? It is a cancer in which the bone marrow and other organs that produce blood produce and increased amount of immature or abnormal white blood cells. The symptoms of leukemia are paleness, excessive bruising, pain in the joints, and fatigue. Brain tumors are formed when a massive amount of cells are produced on the brain. The symptoms for this are frequent headaches, vomiting, seizures, decreased coordination, weakness, and problems concerning vision.
You're just going to an ordinary doctors appointment, but you hear news that is devastating. “You have cancer and you need to start chemo immediately if you want any chance of surviving,” says the doctor. You don’t really want to do chemotherapy because of the horrible side effects. That would usually be the end of the discussion, but that's not always the case for minors. Doctors go to court and take you out of your home and put you in foster homes until you get admitted into the hospital. You get taken away from the people you love and care about and get placed in a foster home with no idea what's going on. The court approves the chemo and you have to go to the hospital. You are so upset that you run away but return a week later afraid. Then you wake up strapped to the hospital bed with a port surgically inserted into you. This exact story happened to a girl named Cassandra who is a minor at age 17. She has hodgkin lymphoma and left untreated will kill you. With doing chemo there is an 85% chance of surviving. Chemo is when you put poison in your body to kill cancer cells. Nobody wants poison in your body, sure it kills the cancer but it also kills your organs. There are plenty of other options other than chemo but most doctors are almost convinced that the only way to kill cancer cells is to do chemotherapy.
Hodgkin's and Non-Hodgkin's Lymphoma: Differences? and similarity on MedicineNet.com. Retrieved December 9, 2013, from http://www.cdl.gov http://www.medicinenet.com/script/main/art.asp?articlekey=79261 Turley, Susan M. (2014). The 'Path of Medical language (third ed.). Upper Saddle River, NJ: Pearson. Education & Training, Inc. Zimmerman, K. A. & Co.,
These are tumors that grow more quickly than other