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LEUKEMIA KIT LEUKEMIA KIT We created the Leukemia Kit to highlight the key principles to managing the cancer decision-making process. In it, you will find tools designed to present you with insightful information you as a patient or caregiver will find helpful in your own search for the best cancer treatment available. Undoubtedly, many unfamiliar issues surface after a cancer diagnosis–questions and concerns you never imagined you would have to face. It’s OK if you feel overwhelmed, angry or upset. Your situation requires you to make a multitude of tough decisions, often immediately. But you do have the power to make sharp, informed decisions. You have the power to take charge of your situation but to do so, you need to sort through all of the emotions–yours and your loved ones’–assess all of the facts and identify a solution to help you get back on track. As you flip through the following pages, you will find five sections: “Understanding Leukemia,” “Overview of Treatment Options,” “Questions to Ask Your Doctor,” “Selecting Your Treatment Hospital,” and, most importantly, the final piece entitled the “Decision Manual.” The Decision Manual is a worksheet we offer you, to help you gain control and take a more active role in the decisionmaking process. It requires you to begin asking questions–hard questions–that ask what you are looking for in a hospital and a physician, the goals and expectations you bring to the treatment process and the steps you need to take to make your goals a reality. If this sounds different to you, it’s because it is different! We believe you must be a key player and a decision-maker. At the very least, the Leukemia Kit contains useful information about hospitals, treatment options and questions you may use to assess the doctors and hospitals you visit throughout this experience. We wish you the best on your journey ahead and would be happy to hear from you if we can be of service in any way. UNDERSTANDING LEUKEMIA Unlike solid tumors that may develop in the lung or breast, leukemia affects your body’s blood cell-producing tissues. Like all cancers, leukemia originates at the cellular level. Understanding leukemia requires you to familiarize yourself with some basic information regarding how and why leukemia develops in the body. Your body continually produces new blood cells, creating one hundred billion red blood cells and four hundred million white blood cells every hour. Each blood cell performs a variety of different functions vital to your overall health. Four types of cells comprise your blood: red blood cells, white blood cells, lymphocytes and platelets. Sometimes called erythrocytes, red blood cells transport life-giving oxygen from your lungs throughout your body. White blood cells and lymphocytes manufacture antibodies designed to protect your body from bacteria and other foreign substances. Platelets, or thrombocytes, contain clotting agents, preventing bleeding. Each mature blood cell originates from a single parent cell called a stem cell. Ninety-five percent of stem cells begin life in the bone marrow–a spongy tissue that fills the inside of the bones. Some stem cells remain in the marrow until they mature; others migrate to the thymus, a gland in the lymphatic system, before developing into their “adult” cell types. Generating new stem cells to replace old or damaged blood cells allows your body to continually restore itself through this natural maintenance process. But sometimes, the cells that comprise the bloodproducing tissues of the bone marrow mutate, or change, and begin manufacturing blood cells in uncontrollable numbers. This uncontrolled cellular growth is called cancer. A leukemia diagnosis may leave you with many unanswered questions. Uncovering some of these answers begins by learning about the specific type of leukemia diagnosis you receive. Basic differences do exist between the four main types of leukemia–studying these differences may help you gain a better perspective of your current situation. Your Immune System and Leukemia Understanding leukemia begins by examining one of the most amazing systems in your body–the immune system. Consisting of your thymus, spleen, lymph nodes, gastrointestinal tract and bone marrow, the immune system guards against foreign substances, called antigens, and other abnormal cells sometimes produced by your body. Upon detecting an antigen, your immune system responds, deploying white blood cells, or lymphocytes, to mark the antigen with a special protein called an antibody. The antibody works like a fingerprint, identifying the antigen so additional white blood cells can recognize, target and destroy the foreign substance. White blood cells play a vital role in your body’s ability to defeat the bacteria, fungi and other microorganisms you encounter in your home and workplace. Leukemia compromises this natural immune response by altering the normal development of white blood cells. Improperly formed white blood cells look and act differently than healthy white blood cells, limiting the cell’s ability to carry out normal functions. Studying blood samples taken from your peripheral blood stream and your bone marrow allows your Hematological Oncologist–a doctor specializing in treating cancerous diseases of the blood–to gather valuable information regarding the cell type and aggressiveness of the abnormal cells. Leukemia typically affects the two main types of white blood cells–lymphoid cells and myeloid cells. Lymphoid cells, called lymphocytes, primarily mature in your lymph nodes or other tissues of the lymphatic system; myeloid cells represent bacteria-destroying granulocytes and macrophages. These cells mature only in your bone marrow. Determining the aggressiveness of a leukemia cell is a critical element to assessing your situation. Your Hematological Oncologist will obtain a sample of your blood and examine it under a microscope. By magnifying your blood samples, your physician reveals the structure of the abnormal white blood cells, providing a snapshot of the cell’s level of development. Your Hematological Oncologist will classify your leukemia in one of two ways–acute or chronic. Acute leukemic cells fail to mature beyond the earliest phase of blood cell development, called blast phase. A blastic white blood cell lacks the basic functionality of a fully developed white blood cell. An acute diagnosis signifies your bone marrow contains more than 30 percent blast cells. These young, dysfunctional white blood cells multiply rapidly and accumulate in your bone marrow, disrupting your marrow’s ability to produce healthy white blood cells and normal levels of red blood cells and platelets. Unlike the swiftly progressing acute leukemias, some chronic leukemic cells possess the ability to develop into mature, semi-functional white blood cells. However, over time, your bone marrow and spleen may begin producing white blood cells incapable of reaching maturity. Dividing uncontrollably, these blastic white blood cells increase in number and begin accumulating in your bone marrow. The overabundance of blast cells in your marrow may compromise its ability to produce healthy white blood cells and normal levels of red blood cells and platelets. Consequently, your condition rapidly accelerates. Many different types of leukemia exist. A description of the four most common leukemias follows: • Acute Lymphocytic Leukemia (ALL) represents the most common type of leukemia affecting children; however, 4,000 people from all age groups will receive an ALL diagnosis each year. ALL is a rapidly progressing leukemia involving blastic white blood cells that mature in your bone marrow, thymus or lymph nodes. ALL cells can collect in your brain, spinal cord and/or lymph nodes. Apart from a general diagnosis, you may receive an ALL subtype diagnosis. The ALL subtype diagnosis hinges upon the type of white blood cell–T-lymphocyte or Blymphocyte–your blast cells most closely resemble. T-lymphocytes originate in your thymus; B-lymphocytes are produced by your bone marrow. • Acute Myelogenous Leukemia (AML) stands as the most common type of leukemia diagnosed in the United States, with an estimated 10,000 new AML cases diagnosed each year. AML is a rapidly progressing leukemia involving white blood cells produced by your bone marrow. Depending upon the type of white blood cell your disease affects, you may receive one of eight different AML subtype diagnoses. • Chronic Lymphocytic Leukemia (CLL) most often develops in adults over age 60. Representing the second most common type of leukemia, with over 8,000 new cases diagnosed annually, CLL involves an overproduction of white blood cells called lymphocytes. Generally characterized as a slowly progressing form of leukemia, CLL cells accumulate in your bone marrow and lymphatic system. Increasing the proportion of dysfunctional CLL cells in the blood impedes your bone marrow’s ability to produce healthy white blood cells and normal levels of red blood cells and platelets. This speeds the progression of the disease, transforming chronic leukemia into a more aggressive, acute leukemia. • Chronic Myelogenous Leukemia (CML) arises in adults between the ages of 30 and 50. CML develops when your bone marrow cells begin multiplying and dividing outside of your bone marrow tissue. Ninety percent of all CML cases link to a single genetic marker called the Philadelphia Chromosome. The nucleus of every normal cell in your body contains two chromosomes packed with genetic information determining traits like eye color and skin tone. Sometimes, a piece of the first chromosome translocates, or moves, swapping positions with a small piece from the second chromosome. This exchange of genetic information activates the growth of leukemic cells. CML cells multiply slowly in the chronic, or earliest phase of the disease; however, CML quickly progresses as the number of young, undeveloped blast cells increases. If the number of blast cells in your circulating blood stream and bone marrow rises above 30%, your disease more closely resembles acute leukemia. Talking to a doctor about something as serious as cancer can be intimidating, especially if you feel overwhelmed or upset about confronting the disease. Weaving an understanding of your type of leukemia, together with knowledge of how and why the disease developed in your body, can equip you with a greater sense of control when interacting with physicians. An Important Note on Leukemia Diagnosis and Staging Deciding upon a course of treatment may be the hardest, yet most important life choice you make during this time. Making educated treatment decisions begins by learning about the progression of leukemia in your body. Oncologists in the United States currently employ the TNM–”Tumor,” “Node” and “Metastasis”–Staging Method to stage solid tumors. The TNM Staging Method measures disease progression by tracking how far the cancer travels from the primary tumor site, or the tumor’s point of origin. But unlike solid tumors, leukemia cells originate in your bone marrow and metastasize, or spread, throughout the body via your circulating blood stream. This leaves conventional staging methods unsuited to accurately determine the progression of your disease. Properly diagnosing leukemia involves two separate steps: identifying the leukemia cell type and, with this accomplished, assigning a cytological classification. Using a microscope, your Hematologist–a doctor with special training and expertise in analyzing human blood cell structure and treating blood-borne diseases–closely examines a sample of your blood. The Hematologist documents abnormal cell structure and compares levels of mature white blood cells to existing levels of blast cells. While this process helps your physician verify the presence of leukemic cells in your blood, it may not reveal enough evidence to confirm the specific cell type (e.g. lymphocytic or myeloid) affected by the disease. Determining the leukemia cell type may require your Hematologist to perform a bone marrow aspiration and biopsy by withdrawing a small amount of your liquid bone marrow through a fine needle and obtaining a core sample of your bone. Studying the cytology, or the structure and function of your leukemia cells, presents your Hematological Oncologist with the raw information necessary to assign your particular type of leukemia a cytological classification. The cytological classification guides your prognosis, or the physician’s projection of how the disease will progress in your body. Hematological Oncologists utilize cytological classification systems to identify various subtypes of AML, ALL and CLL. Three different types of cytological classification systems exist–the FAB System, Rai Classification and Binet Staging. The following section provides a brief description of the three cytological classification systems. • French-American-British (FAB) System–The FAB System utilizes a subtype classification to categorize the different forms of Acute Lymphocytic Leukemia (ALL) and Acute Myelogenous Leukemia (AML). Studying the genetic markers associated with each type of leukemic cell and the appearance of the cells under a microscope allows the Hematologist to assess cellular differentiation, or the maturity level of the leukemic cells. The FAB system employs eight subtype classifications ranging from M0 to M7 to distinguish the different variations of AML. • Undifferentiated AML (M0) consists of immature, or blastic, bone marrow cells called myeloblasts. Normal myeloblasts mature into granulocytes–a type of white blood cell responsible for destroying bacteria in the bloodstream. Tiny sand-like particles known as granules exist inside of the mature granulocyte. A microscope reveals the presence of granules, helping your Hematologist differentiate the mature granulocytic cell from other mature cells. A M0 subtype indicates the myeloblasts remain undifferentiated and, therefore, lack the granules present in a mature granulocyte. • Myeloblastic Leukemia (M1) consists of abnormal myeloblasts showing some signs of early granulocytic differentiation. • Myeloblastic Leukemia (M2) consists primarily of abnormal early granulocytic cells, called promyelocytes. M2 leukemia cells remain in varying levels of early granulocytic development. • Promyelocytic Leukemia (M3) consists of increasingly mature early granulocytes. M3 leukemia cells contain many granules. • Myelomonocytic Leukemia (M4) consists of differentiated abnormal granulocytes and cells called monocytes. Monocytes produce macrophages, cells responsible for engulfing and digesting antigens like bacteria. In M4 leukemia, abnormal monocytic cells represent more than 20% of all nucleus-containing cells in the bone marrow. • Monocytic Leukemia (M5) consists of two separate forms of AML. Discriminating between the two forms of M5 leukemia hinges upon identifying the level of cellular differentiation displayed by the monocytic cells. The first form of M5 leukemia involves only poorly differentiated monoblasts. The immature monoblasts–precursors to monocytes–contain lacy-appearing genetic material. The second, more differentiated form of M5 leukemia indicates a higher concentration of monoblasts, promonycytes and monocytes in the circulating bloodstream. • Erythroleukemia (M6) consists of abnormal red blood cell-forming cells, called erythrocytes. • Megakaryoblastic Leukemia (M7) consists of abnormal immature giant bone marrow cells called megakaryoblasts and immature immune system cells called lymphoblasts. Three FAB subtype classifications categorize ALL. Assigning an ALL subtype requires an understanding of the leukemia cells’ morphology, or the physical characteristics associated with the ALL cells. The FAB subtypes L1, L2 and L3 help your Hematologist categorize ALL leukemia cells by grading the appearance of the abnormal cell’s nucleus, cytoplasm and overall maturity level. A description of the FAB ALL subtype classifications follows: • L1 classification involves immature immune system cells called lymphoblasts. L1 cells are small and contain a round nucleus and little cytoplasm–a clear substance that surrounds the outside of the nucleus and extends to the cell membrane. • L2 classification involves large, immature lymphoblasts that contain an ample amount of cytoplasm and an irregularly shaped nucleus. L2 cells vary in size and may exhibit one or more enlarged nucleoli–tiny spheres inside the nucleus responsible for producing RNA. • L3 classification involves large, immature lymphoblasts that contain an abundant amount of cytoplasm peppered with tiny sand-like granules and large air or fluid-filled pockets called vacuoles. The nucleus of an L3 cell appears normal in shape. • Rai Classification–Rai Classification grades Chronic Lymphocytic Leukemia (CLL) cells by employing a Roman numeral staging system similar to the traditional TNM Staging Method used to stage non-leukemic cancers. By tracking the build-up of mature lymphocytes in the bloodstream, Rai Classification works by linking increased lymphocyte accumulation to a greater risk category or stage number. The Roman numerals 0, I, II, III and IV represent the various Rai Classification risk categories, with Stages 0 and I representing low risk categories and Stages III and IV representing high risk categories. A description of the five Rai Stage categories follows: • Rai Stage 0 represents a low-risk category characterized by lymphocytosis–a lymphocyte count higher than 15,000 lymphocytes per cubic millimeter of blood. • Rai Stage I represents an intermediate risk category characterized by lymphocytosis plus enlarged lymph nodes, a condition called lymphadenopathy. • Rai Stage II represents an intermediate risk category characterized by lymphocytosis plus evidence of an enlarged liver (hepatomegaly) or an enlarged spleen (splenomegaly). The lymph nodes may or may not be enlarged. • Rai Stage III represents a high-risk category characterized by lymphocytosis plus anemia and a low red blood cell count. The lymph nodes, liver and spleen may or may not be enlarged. • Rai Stage IV represents a high-risk category characterized by lymphocytosis plus a low number of blood platelets, called thrombocytopenia. Binet Staging–Binet Staging represents the second classification system used to categorize Chronic Lymphocytic Leukemia (CLL) cells. Used widely throughout Europe, Binet Staging differentiates CLL leukemia cells by identifying the level of lymphoid tissue involvement. Lymphoid tissues like your lymph nodes and spleen produce lymphocytes. A person diagnosed with CLL experiences a slow increase in the level of lymphocytes circulating in the blood. As the concentration of lymphocytes rises, some lymphoid tissues may exhibit symptoms such as swelling. The Binet Staging system classifies these symptoms into three subtype categories, marked by the letters, “A,” “B” and “C.” A description of the three Binet Staging categories follows: • Binet Stage “A” represents a person with fewer than three areas of lymphoid tissue involvement. • Binet Stage “B” represents a person with more than three areas of lymphoid tissue involvement. • Binet Stage “C” represents a person with anemia who also exhibits a marked decrease in the number of platelets–blood cells responsible for blood clotting–found in the blood. This condition is called thrombocytopenia. Cytological classification systems like FAB, Rai and Binet Staging categorize leukemia cells based upon the visible characteristics of the cancerous cells. Hematologists utilize a separate phase system to track the development of Chronic Myeloid Leukemia (CML). Three phases classify the progression of CML in the body–chronic, accelerated and blastic. A description of the three CML phases follows: • Chronic Phase represents the initial CML development phase in which blast cell counts represent less than 5% of the total number of blood and bone marrow cells in the body. Chronic phase CML is characterized by excessive levels of mature granulocytes in the circulating bloodstream. Ninety percent of people diagnosed with chronic phase CML exhibit splenomegaly, or an enlarged spleen caused by a build-up of white blood cells. • Accelerated Phase represents the advanced CML development phase in which blast cell counts represent from 5% to 30% of the of the total number of blood and bone marrow cells in the body. Accelerated phase CML is characterized by excessive levels of granulocytes and increasing levels of myeloid, or bone marrow, blast cells in the circulating bloodstream. Genetic studies of accelerated phase CML cells reveal a rise in the number of chromosomal abnormalities exhibited in each abnormal cell. • Blastic Phase represents the crisis CML phase in which blast cell counts represent more than 30% of the total number of blood and bone marrow cells in the body. The overflow blast cells accumulate and rapidly overwhelm the body with dysfunctional cells, disrupting the bone marrow’s normal production of healthy red blood cells, white blood cells and platelets. Once CML reaches blast phase, the disease transforms into an acute form of leukemia. Correctly classifying your leukemia is critical because it helps you and your doctor determine the right treatment options for you. One way you can impact the classification process is to seek out second or third medical opinions from different Hematological Oncologists. Like a system of checks-and-balances, obtaining a second or third medical opinion assures you the type of treatment your original Hematological Oncologist recommends receives the support of another expert in the same field. A listing of the tools needed to diagnose and classify leukemia follows: • Physical Exam – A physical exam consists of a one-on-one examination between the patient and physician. Throughout the exam, the physician will ask questions designed to clarify your current level of health and identify any symptoms pointing to potential areas requiring further examination. The physician will examine your abdominal area for evidence of spleen and liver enlargement and may inspect the lymph nodes clustered around your collarbone, groin and underarms by feeling, or palpating, for any unusual swelling. Lymph nodes collect and transport excess fluid, bacteria and cellular waste from the body in a yellowtinted fluid called lymph. Examining the lymph nodes allows the physician to check for evidence of lymph node tenderness or swelling–possible indicators of leukemia. • Blood Tests – Apart from a physical examination, the Hematological Oncologist utilizes an array of blood tests to identify the particular leukemia cell type and, with this accomplished, to quantify the number of leukemia cells present in the blood samples. Information derived from blood tests helps the Hematologist assign a cytological classification and arrive at a prognosis–two guide posts you and your doctor may use to select a treatment option that best fits your needs. The following section highlights some key types of blood tests administered before, during and after leukemia treatment. • Complete Blood Count (CBC) – A complete blood count calculates the quantity, type and form of red blood cells, white blood cells and platelets circulating in the bloodstream. Analyzing the full spectrum of the blood and its components requires Hematologists to employ a number of tests. A brief description of the eight tests comprising a CBC analysis follows: • White Blood Cell (WBC) Count – A white blood cell count measures the number of WBCs present in a onemicroliter drop of blood. A “normal” white blood cell count may range from 4,100 to 10,900 WBCs per microliter of blood. Exercise habits, stress level and disease status can fluctuate these numbers. • Red Blood Cell (RBC) Count – A red blood cell count measures the number of RBCs present in a one-microliter drop of blood. Red blood cell counts vary with a patients age and sex. Men typically exhibit from 4.5 to 6.2 million RBCs per microliter of blood, whereas women normally range from 4.2 to 5.4 million RBCs per microliter of blood. • Platelet Count–A platelet count measures the number of platelets, or thrombocytes, present in a one-microliter drop of blood. A “normal” platelet count may range from 150,000 to 400,000 platelets per microliter of blood. • White Blood Cell Differential – A white blood cell differential measures the percentage of the five major types of WBCs–neutrophils, lymphocytes, monocytes, eosinophils and basophils–present in a one-microliter drop of blood. The percentages represent the volume of a specific type of WBC in the blood sample. Neutrophils represent the bulk of the WBC army, comprising 50 to 60% of the body’s total number of WBCs. • Hematocrit (HCT) Assay – Hematocrit assay measures the percentage of RBCs present in a one-microliter drop of blood. The percentage represents the volume of RBCs in the blood sample. • Hemoglobin (Hgb)Testing – Hemoglobin is an iron-rich protein lining the surface of red blood cells. Hemoglobin testing assesses the body’s ability to effectively transport oxygen from the lungs throughout the body by measuring the level of hemoglobin per deciliter (100 milliliters) of blood. The “normal” level of hemoglobin varies between men and women. Generally, men register 14 to 18 grams of hemoglobin per deciliter of blood; women usually measure between 12 to 16 grams of hemoglobin per deciliter of blood. • Red Blood Cell Indices – Red blood cell indices (plural for “index”) reflect three core measurements indicative of RBC functionality: ■ Mean Corpuscular Volume (MCV) measures the size of the red blood cells in the sample. ■ Mean Corpuscular Hemoglobin (MCH) measures the hemoglobin content present in the average RBC. ■ Mean Corpuscular Hemoglobin Concentration (MCHC) measures the overall concentration of hemoglobin in the blood sample. • Blood Morphology and Staining – Blood morphology and staining illustrates cell shape and structure, as well as the appearance of the nucleus under a microscope. Applying a special stain to the blood sample allows the Hematologist to note any abnormalities or deficiencies exhibited by the cells. • Bone Marrow Tests – Obtaining small samples of the spongy, blood-cell producing bone marrow provides the Hematological Oncologist and Pathologist with valuable insight into the marrow’s cellular composition. Studying the size, shape and granule content of the marrow cells under a microscope reveals crucial information necessary for a complete and accurate diagnosis. Two tests collect bone marrow samples for analysis: • Bone Marrow Aspiration – Bone marrow aspiration employs a fine needle attached to a syringe to suction, or aspirate, a small, teaspoon-sized sample of liquid bone marrow from the hipbone. • Bone Marrow Biopsy – Bone marrow biopsy, sometimes called a core biopsy, utilizes a biopsy needle to obtain a cylindrical core sample of marrow tissue from the hipbone. Apart from viewing the bone marrow samples under a microscope, the Hematological Oncologist should conduct additional testing to precisely distinguish the leukemia cell type. • Flow Cytometry – Flow cytometry measures the internal composition, shape and size of bone marrow cells traveling in a stream of liquid called a flow. Before introducing the marrow cells into the flow, cells receive a special antibody stain, or labeling, designed to distinguish the bone marrow’s cellular characteristics once the sample undergoes laser light exposure. The energy from the laser light causes the marrow cells to fluoresce, or glow. Sophisticated computer programs capture and analyze how the light emits and scatters from the energized cells, allowing the Hematologist to count and categorize normal and abnormal cells present in the marrow sample. • Immunophenotyping – Immunophenotyping utilizes monoclonal antibody (MOAB) technology to classify specific leukemia cell types. MOAB technology works by manufacturing large quantities of laboratory-produced antibodies designed to recognize and attach to specific leukemia cell lines. Information obtained via immunophenotyping details chromosomal characteristics the Hematological Oncologist may use to properly diagnose certain leukemias and devise a more targeted treatment strategy for the patient. • Diagnostic Tests – Diagnostic tests provide images of the human body. X-rays, magnets, radioisotopes, special video equipment or actual tissue samples present physicians with an “inside” view of the intricate structures that make up the body. • Chest x-ray – A chest x-ray employs conventional x-rays to produce a two-dimensional picture of the chest cavity on x-ray film. Viewing the x-ray film sometimes allows the Hematological Oncologist to identify fluid build-up, enlarged lymph nodes or other symptoms associated with leukemia. Using chest x-rays during and after treatment presents oncologists with a pictorial history that charts your progression through the treatment process. • Computerized Tomography (CT) Scans – Computerized tomography scans utilize x-rays to create crosssectional images of the body. Computerized tomography works by fusing x-ray technology with sophisticated computer imaging systems. Recent advances in CT scanning-speed and imaging capabilities produce precise four-dimensional images in a fraction of the time older CT machines require. • Positron Emission Tomography (PET) Scans – Positron emission tomography differentiates normal cells from rapidly dividing cancer cells by measuring cellular activity. Injecting a small amount of a sugar-bound radioisotope into the patient’s vein allows the PET scan to distinguish between normal and abnormal cellular activity by recording how the different cells burn sugar. Rapidly dividing cancer cells burn sugar at a faster rate than normal cells, distinguishing the cancer cells from healthy tissue. Very few facilities offer this technology – you might wish to consider this when pursuing treatment options or second opinions. OVERVIEW of TREATMENT OPTIONS Today, more than ever, you have access to an array of leukemia treatment options. The sheer number of available options makes understanding the basic treatments an extremely important component of your decision-making process. Exploring this wide range of treatment options requires a general understanding of three traditional treatment modalities–surgery, radiation therapy and chemotherapy–and a fourth group of promising emerging therapies includes immunotherapy and bone marrow transplantation. Here is some basic information about the four treatment categories. Keep in mind, selecting a treatment is not only important but a highly personal decision. Taking extra time to review treatment options with family members or other close friends may help you feel more comfortable and confident before proceeding with treatment. • Surgery – Surgery is the oldest and, perhaps, the most widely practiced form of cancer treatment. Although surgery is rare, side effects that arise as a result of leukemia can require surgical intervention. Before pursuing surgical treatment, you should always obtain a second medical opinion from a different surgical specialist. Surgery is permanent – therefore, it is critical for care providers to conduct thorough laboratory and diagnostic work to ensure the accuracy and effectiveness of the surgery. A brief listing of surgical treatment options for leukemia follow: • Splenectomy – Splenectomy is an inpatient procedure consisting of the surgical removal of an abnormally swollen, or distended, spleen. Normally, the spleen filters and eliminates any microorganisms and wastes from the blood and stores a reserve of red blood cells that it releases during times of emotional stress and physical exertion to boost the level of oxygen-carrying cells in the bloodstream. When a person has leukemia, the spleen attempts to collect and store large amounts of leukemic cells, causing the organ to enlarge. In its enlarged state, the spleen may press against the stomach and/or diaphragm, impeding breathing and digestion. • Radiation Therapy – External beam radiation therapy works by utilizing high-powered x-rays, gamma rays or electron beam radiation to target and destroy rapidly dividing leukemia cells in your body. You may receive radiation therapy as part of a preparative regimen used to ready your body for a bone marrow or stem cell transplant. Recent technological advances in diagnostic imaging machinery allow Radiation Oncologists–doctors who specialize in the planning and delivery of radiation therapy–to deliver precise beams of radiation right where you need it most. Differences do exist in the quality of radiation equipment; therefore, you should always look for a treatment facility with the latest diagnostic equipment and radiation machinery. Radiation therapy is often used in conjunction with surgery and/or chemotherapy. A brief listing of radiation therapy options follows: • 3-D Conformal Radiation Therapy – 3-D conformal radiation therapy is an external form of radiation therapy utilizing computed tomography (CT) planning to image and reconstruct the tumor and surrounding normal tissues in three dimensions using a computer program. This technology allows the radiation oncologist to conform the radiation beam(s) to specific target areas. Because the radiation beams are precisely focused, your nearby normal tissue is spared. • Intensity Modulated Radiation Therapy (IMRT) – IMRT represents an advanced form of external 3-D conformal radiation therapy. Employing a powerful computer program to plan the precise dose of radiation in three dimensions, Radiation Oncologists may vary the intensity and conformance of pencil-thin radiation beams onto specific cancerous sites. Our cancer experts tell us they are able to use higher radiation doses than traditional methods would allow in these areas, and yet spare more of the surrounding healthy tissue, compared to standard radiation therapy. • Chemotherapy – Chemotherapy is a broad term relating to a group of medications designed to damage a cancer cell’s ability to grow. Medical Oncologists–doctors who specialize in treating cancer with different types of drugs and chemotherapy–oversee this aspect of cancer treatment. You may receive chemotherapy orally or through an intravenous (IV) administration. Chemotherapy may be administered throughout your treatment process. Sometimes, neoadjuvant chemotherapy–chemotherapy administered prior to a primary treatment like surgery–can increase the effectiveness of the primary treatment. Likewise, chemotherapy administered after a primary treatment, called adjuvant chemotherapy, can reduce the likelihood of tumor spread or cancer recurrence. Unlike radiation therapy, conventional chemotherapy is a systemic treatment carried throughout your entire body by the bloodstream. New medications help to control side effects and, with the proper comprehensive team of experts, the side effects can usually be managed and minimized. Chemotherapy is often used in conjunction with surgery and/or radiation therapy. Today, you and your doctors may choose from an array of chemotherapies. Each unique case requires the Oncologist to identify the most effective form of chemotherapy available to treat your particular form of leukemia. Chemotherapy can also be delivered differently to enhance effectiveness: • Fractionated Dose Chemotherapy – This method differs from conventional chemotherapy in that the total dose of your chemotherapy is broken into smaller amounts and administered over several days, rather than a single larger dose. This helps you by maximizing the dose intensity and exposing leukemia cells within your body to the drugs for a longer period of time, while reducing some of the unpleasant side effects of chemotherapy. • Emerging Therapies – In the hands of a skilled physician, emerging therapies represent promising new treatment options available in select hospitals across the country. We label these therapies “emerging” because researchers continually uncover new and innovative treatment protocol that harness the latest technological advances and the natural power of the body’s immune system to help the body fight back against leukemia. Immunotherapies, including bone marrow transplantation (BMT), non-myeloablative stem cell transplantation (NST) and donor lymphocyte infusion (DLI), illustrate some of the more prominent emerging therapies now available to you. A listing of emerging therapies follows: • Allogeneic Bone Marrow Transplantation (BMT) – Allogeneic Bone Marrow Transplantation utilizes stem cells obtained from a genetically-matched family member to re-infuse leukemia patients with healthy stem cells. Identifying a stem cell donor requires a team effort from the patient and those family members (usually a brother or sister) who may qualify as potential stem cell donors. Ensuring the proper genetic match between the patient’s stem cells and the donor’s stem cells is a critical qualifying step that must occur before a patient can receive an allogeneic transplant. By genetically matching the stem cells, the Hematological Oncologist attempts to find a donor with a similar human leukocyte antigen, or HLA match. The surface of every white blood cell in your body contains this HLA protein. Like a fingerprint, the HLA protein distinguishes your white blood cells from the white blood cells of another person. This distinction helps your white blood cells discriminate between “friendly” cells and “foreign” matter like bacteria and viruses. Typically, an HLA match occurs in 30 to 35 percent of patients and siblings. If the HLA-match is not precise, one of two scenarios may occur: the patient’s immune system will recognize the donor lymphocytes as “foreign” and attack the donor cells; or, the donor lymphocytes will recognize the patient’s cells as “foreign.” In this scenario, the donor lymphocytes mount an offensive against the host’s body in a response called Graft Versus Host Disease (GVHD). Both situations can result in a failed transplant. If an HLA-matched donor is secured, the patient receives higher-than-conventional doses of chemotherapy and/or total body irradiation to eradicate the leukemia cells present in the bloodstream and bone marrow. Apart from killing leukemia cells, these powerful treatments eliminate healthy cells, including healthy bone marrow cells. Without the bone marrow to generate stem cells, the body cannot produce the white blood cells responsible for immune function. Thus, the patient requires donor lymphocytes to “jump start” the production of white blood cells and help restore the body’s immune system to normal levels. Collecting enough donor lymphocytes for an effective bone marrow transplant requires the donor to undergo a stem cell harvest procedure. Usually, the Hematological Oncologist can obtain an adequate amount of stem cells by performing a peripheral blood stem cell transplant, or PBSCT. The PBSCT works much like a blood donation; however, since the circulating blood stream contains much lower levels of stem cells than the bone marrow, HLA-matched donors receive a colony-stimulating factor prior to the PBSCT to boost the number of stem cells present in the circulating blood. During the procedure, the donor’s blood passes through an aphaeresis machine specially designed to filter the stem cells from the blood and return the remaining blood product back to the donor’s body. Sometimes, the PBSCT does not produce the desired amount of stem cell product. If this occurs, the Hematological Oncologist may utilize a bone marrow aspiration to obtain additional stem cells from the donor’s marrow-rich hipbones and pelvis. This procedure, conducted under an operative setting, employs a fine needle inserted into the donors hipbone and/or pelvis to withdraw from 5 to 10 percent of the bone marrow. The marrow product is carefully processed to remove any unwanted red blood cells or tiny bone fragments before administering the harvested stem cells to the patient. The actual “bone marrow transplant” does not require any incisions. Rather, the patient receives the stem cell transplant via a one-to-two-hour-long stem cell infusion. Once inside the patient, the donor stem cells take from two-to-four weeks to migrate to the bone marrow and begin producing healthy white blood cells–a process called engraftment. • Autologous Bone Marrow Transplantation (BMT) – Autologous bone marrow transplantation utilizes stem cells obtained directly from the patient’s circulating blood stream. Like an allogeneic transplant, autologous bone marrow transplants work by utilizing higher-than-conventional doses of chemotherapy and/or total body irradiation to destroy rapidly dividing leukemia cells in the bloodstream. Since chemotherapy and total body irradiation are systemic therapies, these treatments affect other healthy cells in the body, including the bone marrow–the spongy tissue located inside of the bones responsible for producing stem cells. Stem cells are important because they produce red blood cells (responsible for transporting oxygen), white blood cells and lymphocytes (responsible for defending against infection) and platelets, which prevent bleeding. Prior to receiving high-dose chemotherapy and or radiation, the patient undergoes an autologous stem cell harvest, meaning stem cells are collected directly from your own blood stream and immediately frozen. After completing chemotherapy and/or radiation, you then receive an infusion containing your harvested, healthy stem cells. This enables your body to begin manufacturing its own blood cells. • Donor Lymphocyte Infusion (DLI) – Donor Lymphocyte Infusions work by infusing a donor’s white blood cells, or lymphocytes, into the patient with the hope that the donor lymphocytes will search, find, attack and kill cancerous cells. Donor Lymphocyte Infusion is not a stand-alone therapy; however, when used in conjunction with autologous and allogeneic transplants, DLI stands as a new option both for patients experiencing relapse and for patients receiving a mini-transplant. Since DLI does not require high dose chemotherapy or radiation prior to the infusion, a less intensive immuno-suppressive regimen is required, making this procedure an option for patients unable to tolerate high dose chemotherapy or radiation treatments. • Non-Myeoablative Stem Cell Transplantation (Mini-Transplant) – Mini-Transplants work in combination with Donor Lymphocyte Infusion to suppress the patient’s immune system to a level that tolerates the donor stem cells. Combining new immuno-suppressive agents with or without lower doses of chemotherapy and/or radiation effectively prevents the patient’s immune system from rejecting the donated cells, thereby allowing the donor stem cells to settle and begin producing donor-derived cells. These donor-derived cells include lymphocytes that seek and destroy cancer cells in the patient’s body. Special Services Apart from the four main treatment modalities, you should also consider pain management and palliative care services. • Palliative Care –Palliative Care is a specialized form of medicine focused upon alleviating pain, nausea or any number of other side effects you may experience during treatment. Few hospitals offer a dedicated Palliative Care Department–but regardless of this trend, effectively managing your pain is necessary for optimal treatment. Unmanaged pain may interfere with your sleep patterns, appetite and treatment schedule. You should inquire about the Palliative Care or Pain Management programs available in any hospitals you consider for treatment.