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Frequently Asked Questions: |
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| What is CML? |
| What causes Ph+ CML? |
| What is GIST? |
| What causes Kit+ GIST? |
| What is KIT? |
| What is Ph+ ALL? |
| What causes Ph+ ALL? |
| What is MDS/MPD? |
| What causes MDS/MPD? |
| What is ASM? |
| What causes ASM? |
| What is HES and/or CEL? |
| What causes HES/CEL? |
| What is Dermatofibrosarcoma Protuberans (DFSP)? |
| What causes DFSP? |
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What is CML? CML: Chronic Myeloid Leukemia Leukemia is a type of cancer of the blood and the bone marrow (the inner part of bones where blood cells are made). With leukemia, two things happen. First, certain blood cells become abnormal. Second, the body keeps producing large numbers of these abnormal cells. CML is one type of leukemia. "Chronic" means that it is a slower-growing cancer that may take years to progress (unlike acute leukemia, which progresses very quickly). "Myeloid" refers to the type of white blood cell being overproduced. So, CML is a slowly progressing cancer that makes the body produce too many cancerous myeloid white blood cells. There are three phases of CML: the chronic phase, the accelerated phase, and the blast crisis phase. As patients move through these phases, their disease progresses, and they experience more physical symptoms. What causes Ph+ CML? When a person is healthy, signals in the body tell it to produce new blood cells. These signals reach early cells, called stem cells, which mature into various types of blood cells. The signals turn stem cells on and off as necessary to produce the number of blood cells the body needs. When a person has CML, a change takes place in the person's DNA and makes this signal stay on constantly. Since this signal stays on, the body produces more and more abnormal cells. What is GIST? A Gastro-Intestinal Stromal Tumor (GIST) is a rare type of cancer that grows in the connective tissue between muscle layers in the digestive (gastrointestinal, or GI) tract. All GISTs can become cancerous and may spread or grow back (recur) after treatment. The tumors most often grow in the stomach and small intestine. Although less common, GISTs can also be found in the colon, rectum, and esophagus. What causes Kit+ GIST? A protein called "Kit" is thought to be one of the major causes of GIST. Normal cells have a limited life span. The Kit protein is located on the surface of normal cells. It sends a signal inside the cells that tells them to grow only as needed. When Kit becomes abnormal, its signal stays on all the time, causing cells to multiply out of control forming a tumor. Cancerous GIST cells develop a survival "advantage" because they grow faster and live longer than the healthy surrounding cells. The longer the cancerous cells live, the more potential they have to become dangerous, and the more likely they are to spread to other parts of the body. What is Kit? Kit is the name of both the gene on chromosome and the protein it produces that is thought to be a major cause of GIST as well as some cases of ASM. What is Philadelphia Chromosome-positive Acute Lymphoblastic Leukemia (Ph+ ALL)? Acute lymphoblastic leukemia (ALL) is a fast-growing malignant disorder resulting from a proliferation of white blood cells that fail to mature and accumulate in the bone marrow, destroying and replacing cells that produce normal blood cells. Prognosis declines with increasing age and is most unfavorable when the Philadelphia chromosome (Ph) is present. What causes Ph+ ALL? Changes in chromosomes (genetic material) are a major cause of abnormal genetic conditions in humans. If a change involves a translocation, part of a chromosome breaks off and attaches to another chromosome. The Philadelphia chromosome results from a reciprocal translocation between chromosomes 9 and 22 in which a gene segment on chromosome 9 is inserted into one of several regions of a gene on chromosome 22. The new gene resulting from this translocation produces an abnormal protein that adversely impacts normal cellular processes, resulting in an increased proliferation of leukemic cells. What is Myelodysplastic/Myeloproliferative disease (MDS/MPD)? The myelodysplastic syndromes (MDS) are a diverse collection of blood disorders characterized by an ineffective production of blood cells. The myeloproliferative diseases (MPDs) are a group of diseases of the bone marrow in which excess cells are produced. Myelodysplastic/myeloproliferative overlap disorders (MDS/MPD) are malignant stem cell disorders that have both dysplastic and proliferative features at the time of initial presentation, resulting in increased numbers of immature cells in the bone marrow and a decrease in mature, functional cells in the blood. What causes MDS/MPD? While patients with MDS/MPD lack the Philadelphia chromosome that is common to chronic myeloid leukemia (CML), chromosomal damage is common in patients with this disease, including gene rearrangements involving the platelet-derived growth factor receptor gene known as PDGFR. The new gene resulting from this kind of rearrangement produces an abnormal protein that adversely impacts normal cellular processes, causing cells to multiply out of control. What is Aggressive Systemic Mastocytosis (ASM)? Aggressive systemic mastocytosis (ASM) is a myeloproliferative disorder characterized by the abnormal growth, proliferation, and accumulation of mast cells in one or more organs that can, in some cases, lead to organ failure. There is usually severe involvement of the bone marrow, liver, spleen, and lymphatic system. Mast cells are produced in the bone marrow and contain chemical mediators, like histamine, that are released in response to inflammatory and allergenic events. What causes ASM? Changes in chromosomes (genetic material) are a major cause of abnormal genetic conditions in humans. If the change involves a mutation in a gene then the protein produced by the gene will usually not function as intended. If the change involves a deletion within a gene, there is a loss of genetic information, resulting in a shorter than normal protein that will also usually not function as intended. ASM is associated with a specific mutation within the c-Kit gene on chromosome 4 or a deletion within the chromosome that causes abnormal activation of the platelet-derived growth factor receptor gene, known as PDGFR. This new gene makes an abnormal protein that adversely impacts normal cellular processes, causing cells to multiply out of control. What is Hypereosinophilic Syndrome (HES) and/or Chronic Eosinophilic Leukemia (CEL)? Hypereosinophilic syndrome (HES) is a blood disorder characterized by unexplained, higher than normal levels of eosinophils, a type of white blood cell, and evidence of organ dysfunction due to eosinophilic tissue infiltration. In some cases, it can additionally be characterized by elevated levels of immature mast cells in the bone marrow and mast cell products in the blood. When associated with chromosomal abnormalities, the disease is then called chronic eosinophilic leukemia (CEL). What causes HES/CEL? An overproduction of eosinopihils in the bone marrow with migration into the blood stream and various organs are upstream events associated with the disease. Changes in chromosomes (genetic material) associated with HES/CEL usually involve translocations of the platelet-derived growth factor receptor gene known as PDGFR. They are a major cause of abnormal genetic conditions in humans. If a change involves a translocation, part of a chromosome breaks off and attaches to another chromosome. Adjacent to the PDGFR gene are a number of genes important in the growth and maturation of eosinophils. The normal function of these genes may be adversely impacted by the PDGFR translocation process, causing the unexplained eosinophilia which is observed. What is Dermatofibrosarcoma Protuberans (DFSP)? Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive cutaneous tumor of intermediate malignancy that is characterized by slow, infiltrative growth and a strong tendency towards local recurrence after surgery. What causes DFSP? Changes in chromosomes (genetic material) are a major cause of abnormal genetic conditions in humans. If a change involves a translocation, part of a chromosome breaks off and attaches to another chromosome. In rare cases, the broken parts can attach to themselves forming ring structures. Chromosomal translocations and/or ring chromosomes which fuse the collagen gene on chromosome 17 to the platelet-derived growth factor gene, known as PDGFR, on chromosome 22 are present in nearly all cases of DFSP. Abnormal activation of the platelet-derived growth factor receptor gene produces an abnormal protein that adversely impacts normal cellular processes, causing cells to multiply out of control. Back to top Please see the Important Safety Information and full Prescribing Information. |
RELATED LINKS
About Ph+ CML
About Kit+ GIST
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SAFETY: Important Product Information
Important information GLEEVEC is available only by prescription.
Indication
GLEEVEC® (imatinib mesylate) tablets are indicated for:
Newly diagnosed adult patients with Philadelphia chromosome–positive chronic myeloid leukemia (Ph+ CML) in the chronic phase.
Patients with Ph+ CML in blast crisis (BC), accelerated phase (AP), or in the chronic phase (CP) after failure of interferon-alpha therapy
Adult patients with relapsed or refractory Ph+ acute lymphoblastic leukemia (Ph+ ALL)
Adult patients with myelodysplastic/myeloproliferative diseases (MDS/MPD) associated with PDGFR (platelet-derived growth factor receptor) gene rearrangements
Adult patients with aggressive systemic mastocytosis (ASM) without the D816V c-KIT mutation or with unknown mutational status
Adult patients with hypereosinophilic syndrome (HES) and/or chronic eosinophilic leukemia (CEL) who have the FIP1L1-PDGFRα fusion kinase and for patients with HES and/or CEL who are FIP1L1-PDGFRα fusion kinase–negative or unknown
Adult patients with unresectable, recurrent, and/or metastatic dermatofibrosarcoma protuberans (DFSP)
Patients with KIT (CD117)—positive gastrointestinal stromal tumors (GIST) that cannot be surgically removed, and/or have spread to other parts of the body
Adult patients post-surgery who have had their GISTs completely removed
Who should NOT take GLEEVEC
Women who are or could be pregnant. Fetal harm can occur when administered to pregnant women; therefore, women should not become pregnant, as well as be advised of the potential risk to the unborn child if GLEEVEC is used during pregnancy.
Women who are breast-feeding should not take GLEEVEC because of the potential for serious adverse reactions in nursing infants.
Sexually active females should use adequate birth control while taking GLEEVEC.
Be sure to talk to your doctor and/or nurse about these issues before taking GLEEVEC.
Warnings and precautions
Edema (swelling) and severe fluid retention have occurred. Your doctor will weigh you regularly and manage unexpected weight gain by drug interruption and diuretics.
Cytopenias (reduction or lack of certain cell elements in blood circulation) such as anemia, have occurred. Your doctor will perform complete blood counts weekly for the first month, biweekly for the second month, and periodically thereafter. In most cases, your doctor will reduce or interrupt your GLEEVEC therapy; in rare cases, your doctor may discontinue treatment.
Severe congestive heart failure and left ventricle dysfunction have been reported, particularly in patients with other health issues and risk factors. Patients with heart disease or risk factors will be monitored and treated for the condition.
Severe liver problems (hepatotoxicity) may occur. Your doctor will check your liver function before beginning treatment and continue to monitor liver function as needed.
Bleeding may occur. Severe gastrointestinal (GI) bleeding has been reported in patients with newly diagnosed PH+ CML and KIT+ GIST. GI tumor sites may be the cause of this bleeding in KIT+ GIST.
GI perforation (small holes or tears in the walls of the stomach or intestine), in some cases fatal, has been reported.
In patients with certain conditions associated with high eosinophil levels (eg, HES, MDS/MPD and ASM), beginning Gleevec has been associated with cardiogenic shock/ left ventricle dysfunction
Skin reactions, such as fluid filled blisters, have been reported with the use of GLEEVEC.
Clinical cases of hypothyroidism have been reported in patients taking levothyroxine replacement during treatment with GLEEVEC. Your doctor should closely monitor your TSH levels.
Long-term use may result in potential liver, kidney, and/or heart toxicities; immune system suppression may also result from long-term use.
GLEEVEC can cause fetal harm when administered to a pregnant woman. Women should be aware of the potential harm to the fetus.
Important safety information
The following serious side effects have been reported in patients taking GLEEVEC:
Severe fluid retention, which can cause swelling around the eyes or swelling of the lower legs, lungs, and heart; fatal in rare cases
Low levels of certain blood cells
Heart failure/cardiogenic shock
Liver problems
Skin blistering
Hemorrhage (abnormal bleeding)
Low levels of thyroid hormone
Your doctor will check you closely for any side effects to stop more serious complications from occurring. Patients with heart disease or risk factors for heart failure should also be monitored carefully.
GLEEVEC is sometimes associated with stomach or intestinal irritation. GLEEVEC should be taken with food and a large glass of water to minimize this problem. There have been rare reports, including deaths, of stomach or intestinal perforation (a small hole or tear). Common side effects of GLEEVEC
A majority of patients treated with GLEEVEC experience side effects at some time. Most side effects are mild to moderate in severity. Some common side effects you may experience include:
Fluid retention
Muscle cramps or painAbdominal painVomitingDiarrheaHemorrhage (abnormal bleeding)NauseaFatigueRash
Some mild to moderate side effects can be managed with the help of other medicines and advice from your doctor. However, in some cases, your dose of GLEEVEC may be stopped for awhile or may be changed. Take GLEEVEC exactly as prescribed. Do not change your dose or stop taking GLEEVEC unless you are told to do so by your doctor. If you miss a dose, take your dose as soon as possible, unless it is almost time for your next dose. In this case, your missed dose should not be taken. A double dose should not be taken to make up for any missed dose. You should take GLEEVEC with a meal and a large glass of water. Be sure to inform your doctor if you are or think you may be pregnant. You should not breast-feed while taking GLEEVEC. Do not take any other medications without talking to your doctor or pharmacist first, including over-the-counter medications such as Tylenol® (acetaminophen); herbal products (St. John's Wort, hypericum perforatum); Coumadin® (warfarin sodium); rifampin; erythromycin; and Dilantin® (phenytoin). Taking these with GLEEVEC may affect how they work, or affect how GLEEVEC works. You should also tell your doctor if you are taking or plan to take iron supplements. Patients should also avoid grapefruit juice and other foods which may affect how GLEEVEC works. Tell your doctor if you experience side effects during therapy with GLEEVEC, including fever, shortness of breath, blood in your stools, jaundice (yellowing of the skin and/or eyes), sudden weight gain, symptoms of heart failure, or if you have a history of heart disease or risk factors for heart disease. After GLEEVEC's approval, the following adverse events have been reported in patients treated with GLEEVEC: compression of the heart due to increased fluid, GI perforation (holes in the stomach or intestine), and sudden lung failure. These events, including some fatalities, may or may not have been drug related. Please see the full Prescribing Information. Tylenol (acetaminophen) is a registered trademark of McNeil Consumer & Specialty Pharmaceuticals a division of McNeil PPC, Inc. Coumadin (warfarin sodium) is a registered trademark of Bristol Myers Squibb Company. Dilantin (phenytoin) is a registered trademark of Parke-Davis, a division of Pfizer Inc. |
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There are 2 options for the treatment of Ph+ chronic myeloid leukemia (CML). Click on GLEEVEC.com to continue to learn about GLEEVEC® or click the box below to learn more about a second-line option.
See full Prescribing Information.