Leukemia, Chronic Myeloid (CML)

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MedlinePlus: ⁴¹ What is leukemia? Leukemia is a term for cancers of the blood cells. Leukemia starts in blood-forming tissues such as the bone marrow. Your bone marrow makes the cells which will develop into white blood cells, red blood cells, and platelets. Each type of cell has a different job: White blood cells help your body fight infection Red blood cells deliver oxygen from your lungs to your tissues and organs Platelets help form clots to stop bleeding When you have leukemia, your bone marrow makes large numbers of abnormal cells. This problem most often happens with white blood cells. These abnormal cells build up in your bone marrow and blood. They crowd out the healthy blood cells and make it hard for your cells and blood to do their work. What is chronic myeloid leukemia (CML)? Chronic myeloid leukemia (CML) is a type of chronic leukemia. "Chronic" means that the leukemia usually gets worse slowly. In CML, the bone marrow makes abnormal granulocytes (a type of white blood cell). These abnormal cells are also called blasts. When the abnormal cells crowd out the healthy cells, it can lead to infection, anemia, and easy bleeding. The abnormal cells can also spread outside the blood to other parts of the body. CML usually occurs in adults during or after middle age. It is rare in children. What causes chronic myeloid leukemia (CML)? Most people with CML have a genetic change called the Philadelphia chromosome. It's called that because researchers in Philadelphia discovered it. People normally have 23 pairs of chromosomes in each cell. These chromosomes contain your DNA (genetic material). In CML, part of the DNA from one chromosome moves to another chromosome. It combines with some DNA there, which creates a new gene called BCR-ABL. This gene causes your bone marrow to make an abnormal protein. This protein allows the leukemia cells to grow out of control. The Philadelphia chromosome isn't passed from parent to child. It happens during your lifetime. The cause is unknown. Who is at risk for chronic myeloid leukemia (CML)? It is hard to predict who will get CML. There are a few factors that could raise your risk: Age - your risk goes up as you get older Gender - CML is slightly more common in men Exposure to high-dose radiation What are the symptoms of chronic myeloid leukemia (CML)? Sometimes CML does not cause symptoms. If you do have symptoms, they can include: Feeling very tired Weight loss for no known reason Drenching night sweats Fever Pain or a feeling of fullness below the ribs on the left side How is chronic myeloid leukemia (CML) diagnosed? Your health care provider may use many tools to diagnose CML: A physical exam A medical history Blood tests, such as a complete blood count (CBC) with differential and blood chemistry tests. Blood chemistry tests measure different substances in the blood, including electrolytes, fats, proteins, glucose (sugar), and enzymes. Specific blood chemistry tests include a basic metabolic panel (BMP), a comprehensive metabolic panel (CMP), kidney function tests, liver function tests, and an electrolyte panel. Bone marrow tests. There are two main types - bone marrow aspiration and bone marrow biopsy. Both tests involve removing a sample of bone marrow and bone. The samples are sent to a lab for testing. Genetic tests to look for gene and chromosome changes, including tests to look for the Philadelphia chromosome If you are diagnosed with CML, you may have additional tests such as imaging tests to see whether the cancer has spread. What are the phases of chronic myeloid leukemia (CML)? CML has three phases. The phases are based on how much the CML has grown or spread: Chronic phase, where less than 10% of cells in the blood and bone marrow are blast cells (leukemia cells). Most people are diagnosed in this phase, and many do not have symptoms. Standard treatment usually helps in this phase. Accelerated phase, 10% to 19% of the cells in the blood and bone marrow are blast cells. In this phase, people often have symptoms and standard treatment may not be as effective as in the chronic phase. Blastic phase, where 20% or more of the cells in the blood or bone marrow are blast cells. The blast cells have spread to other tissues and organs. If you have tiredness, fever, and an enlarged spleen during the blastic phase, it is called a blast crisis. This phase is harder to treat. What are the treatments for chronic myeloid leukemia (CML)? There are several different treatments for CML: Targeted therapy, which uses drugs or other substances that attack specific cancer cells with less harm to normal cells. For CML, the drugs are tyrosine kinase inhibitors (TKIs). They block tyrosine kinase, which is an enzyme that causes your bone marrow to make too many blasts. Chemotherapy Immunotherapy High-dose chemotherapy with stem cell transplant Donor lymphocyte infusion (DLI). DLI is a treatment that may be used after a stem cell transplant. It involves giving you an infusion (into your bloodstream) of healthy lymphocytes from the stem cell transplant donor. Lymphocytes are a type of white blood cell. These donor lymphocytes may kill the remaining cancer cells. Surgery to remove the spleen (splenectomy) Which treatments you get will depend on which phase you are in, your age, your overall health, and other factors. When the signs and symptoms of CML are reduced or have disappeared, it is called remission. The CML may come back after remission, and you may need more treatment. NIH: National Cancer Institute

MalaCards based summary: Leukemia, Chronic Myeloid, also known as chronic myelogenous leukemia, is related to chronic myelogenous leukemia, bcr-abl1 positive and atypical chronic myeloid leukemia, bcr-abl1 negative, and has symptoms including angina pectoris, chest pain and edema. An important gene associated with Leukemia, Chronic Myeloid is ABL1 (ABL Proto-Oncogene 1, Non-Receptor Tyrosine Kinase), and among its related pathways/superpathways are MAPK Signaling: Mitogens and MAP Kinase Signaling. The drugs Benzocaine and Tannic acid have been mentioned in the context of this disorder. Affiliated tissues include myeloid, bone marrow and bone, and related phenotypes are myeloproliferative disorder and splenomegaly

MedlinePlus Genetics: ⁴² Chronic myeloid leukemia is a slow-growing cancer of the blood-forming tissue (bone marrow). Normal bone marrow produces red blood cells (erythrocytes) that carry oxygen, white blood cells (leukocytes) that protect the body from infection, and platelets (thrombocytes) that are involved in blood clotting. In chronic myeloid leukemia, the bone marrow produces too many white blood cells. Initially, these cells function relatively normally. However, as the condition progresses, immature white blood cells called myeloblasts (or blasts) accumulate in the blood and bone marrow. The overgrowth of myeloblasts impairs development of other blood cells, leading to a shortage of red blood cells (anemia) and platelets.Chronic myeloid leukemia usually begins after age 60. Common features include excessive tiredness (fatigue), fever, and weight loss. Many affected individuals develop an enlarged spleen (splenomegaly), which can cause a feeling of fullness in the abdomen and a loss of appetite. About half of people with chronic myeloid leukemia do not initially have any signs and symptoms and are diagnosed when a blood test is performed for another reason.The condition consists of three phases: the chronic phase, the accelerated phase, and the blast phase (or blast crisis). In the chronic phase, the number of mature white blood cells is elevated, and myeloblasts account for less than 10 percent of blood cells. Signs and symptoms of the condition during this phase are typically mild or absent and worsen slowly. The chronic phase can last from months to years. In the accelerated phase, the number of myeloblasts is slightly higher, making up 10 to 29 percent of blood cells. The signs and symptoms continue to worsen. The accelerated phase usually lasts 4 to 6 months, although it is skipped in some affected individuals. In blast crisis, 30 percent or more of blood or bone marrow cells are myeloblasts. Signs and symptoms are most severe in this phase, including a massively enlarged spleen, bone pain, and weight loss. Serious infections and uncontrolled bleeding can be life-threatening.

UniProtKB/Swiss-Prot ⁷³ Leukemia, chronic myeloid: A clonal myeloproliferative disorder of a pluripotent stem cell with a specific cytogenetic abnormality, the Philadelphia chromosome (Ph), involving myeloid, erythroid, megakaryocytic, B-lymphoid, and sometimes T-lymphoid cells, but not marrow fibroblasts.

Leukemia, chronic myeloid, atypical: A myeloproliferative disorder that shares clinical and laboratory features with chronic myeloid leukemia but lacks the pathognomonic Philadelphia chromosome and the corresponding BCR/ABL1 fusion transcript. Features include myeloid predominance in the bone marrow, myeloid proliferation and low leukocyte alkaline phosphatase value, splenomegaly, hepatomegaly, elevated white blood cell count. Enlarged spleen may also be associated with a hypermetabolic state, fever, weight loss, and chronic fatigue. The enlarged liver may contribute to the patient's weight loss.

GARD: ¹⁹ Chronic myeloid leukaemia (CML) is the most common myeloproliferative disorder accounting for 15-20% of all leukaemia cases.

Orphanet: ⁵⁸ Chronic myeloid leukaemia (CML) is the most common myeloproliferative disorder accounting for 15-20% of all leukaemia cases.

Disease Ontology: ¹¹ A myeloid leukemia that is characterized by over production of white blood cells.

Wikipedia: ⁷⁵ Chronic myelogenous leukemia (CML), also known as chronic myeloid leukemia, is a cancer of the white... more...

Clinical features from OMIM®:

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