Radiation therapyRadiation therapy (or radiotherapy) is used on painful bony areas, in high disease burdens, or as part of the preparations for a bone marrow transplant (total body irradiation). Radiation in the form of whole-brain radiation is also used for central nervous system prophylaxis, to prevent recurrence of leukemia in the brain. Whole-brain prophylaxis radiation used to be a common method in treatment of children’s ALL. Recent studies showed that CNS chemotherapy provided results as favorable but with less developmental side-effects. As a result, the use of whole-brain radiation has been more limited. Most specialists in adult leukemia have abandoned the use of radiation therapy for CNS prophylaxis, instead using intrathecal chemotherapy. Biological therapyFor some subtypes of relapsed ALL, aiming at biological targets such as the proteasome, in combination with chemotherapy, has given promising results in clinical trials. Selection of biological targets on the basis of their combined effects on the leukemic lymphoblasts can lead to clinical trials for improvement in the effects of ALL treatment. ImmunotherapyModified HIV has been used to re-engineer the immune system involving the CD19 molecule) with some preliminary success in experimental treatments. In the US, the incidence of ALL is roughly 6000 new cases per year (as of 2009), or approximately 1 in 50,000. ALL accounts for approximately 70 percent of all childhood leukemia cases (ages 0 to 19 years), making it the most common type of childhood cancer. It has a peak incident rate of 2–5 years old, decreasing in incidence with increasing age before increasing again at around 50 years old. ALL is slightly more common in males than females. There is an increased incidence in people with Down Syndrome, Fanconi anemia, Bloomsyndrome, Ataxia telangiectasia, X-linked agammaglobulinemia, and Severe combined immunodeficiency.
The aim of remission induction is to rapidly kill most tumor cells and get the patient into remission. This is defined as the presence of less than 5% leukemic blasts in the bone marrow, normal blood cells and absence of tumor cells from blood, and absence of other signs and symptoms of the disease. Central nervous system(CNS) prophylaxis should begin during this phase of treatment and continue during the consolidation/intensification period. The rationale is based on the presence of CNS involvement in 10%-40% of adult patients at diagnosis. Combination of Prednisolone or dexamethasone,vincristine, asparaginase (better tolerance in pediatric patients), and daunorubicin (used in Adult ALL) is used to induce remission.
Intensification uses high doses of intravenous multidrug chemotherapy to further reduce tumor burden. Since ALL cells sometimes penetrate the CNS, most protocols include delivery of chemotherapy into the CNS fluid (termed intrathecal chemotherapy). Some centers deliver the drug through Ommaya reservoir (a device surgically placed under the scalp and used to deliver drugs to the CNS fluid and to extract CNS fluid for various tests). Other centers would perform multiple lumbar punctures as needed for testing and treatment delivery.
Typical intensification protocols use vincristine,cyclophosphamide, cytarabine, daunorubicin, etoposide,thioguanine or mercaptopurine given as blocks in different combinations. For CNS protection, intrathecal methotrexate or cytarabine is usually used combined with or without cranio-spinal irradiation (the use of radiation therapy to the head and spine). Central nervous system relapse is treated with intrathecal administration of hydrocortisone, methotrexate, and cytarabine.
The aim of maintenance therapy is to kill any residual cell that was not killed by remission induction, and intensification regimens. Although such cells are few, they
will cause relapse if not eradicated. For this purpose, daily oral mercaptopurine, once weekly oral methotrexate, once monthly 5-day course of intravenous vincristine and oral corticosteroids are usually used. The length of maintenance therapy is 3 years for boys, 2 years for girls and adults.
As the chemotherapy regimens can be intensive and protracted (often about 2 years in case of the GMALL UKALL, HyperCVAD or CALGB protocols; for ALL about 3 years, 2 months for males on COG protocols; 2 years, 2 months for females - longer for males, as testicles are a potential reservoir), many patients have an intravenous catheter inserted into a large vein (termed a central venous catheter or a Hickman line), or a Portacath, a cone-shaped port with a silicone nose that is surgically planted under the skin, usually near the collar bone, and the most effective product available, due to low infection risks and the long-term viability of a portacath.
The earlier acute lymphocytic leukemia is detected, the more effective the treatment. The aim is to induce a lasting remission, defined as the absence of detectable cancer cells in the body (usually less than 5% blast cells in the bone marrow). Treatment for acute leukemia can include chemotherapy, steroids, radiation therapy, intensive combined treatments (including bone marrow or stem celltransplants), and growth factors. Chemotherapy is the initial treatment of choice. Most ALL patients will receive a combination of different treatments. There are no surgical options, due to the body-wide distribution of the malignant cells. In general, cytotoxic chemotherapy for ALL combines multiple antileukemic drugs in various combinations. Chemotherapy for ALL consists of three phases: remission induction, intensification, and maintenance therapy.
In general, cancer is caused by damage to DNA that leads to uncontrolled cellular growth and spread throughout the body, either by increasing chemical signals that cause growth or by interrupting chemical signals that control growth. Damage can be caused through the formation of fusion genes, as well as the dysregulation of a proto-oncogene via juxtaposition of it to the promoter of another gene, e.g. the T-cell receptor gene. This damage may be caused by environmental factors such as chemicals, drugs or radiation
ALL is associated with exposure to radiation and chemicals in animals and humans. The association of radiation and leukemia in humans has been clearly established in studies of victims of the Chernobyl nuclear reactor and atom bombs in Hiroshima and Nagasaki. In animals, exposure to benzeneand other chemicals can cause leukemia. Epidemiological studies have associated leukemia with workplace exposure to chemicals, but these studies are not as conclusive. Some evidence suggests that secondary leukemia can develop in individuals treated for other cancers with radiation and chemotherapy as a result of that treatment The survival rate has improved from zero four decades ago, to 20-75 percent currently, largely due to clinical trials on new chemotherapeutic agents and improvements in stem cell transplantation (SCT) technology
Ethnicity: Caucasians are more likely to develop acute leukemia than African-Americans, Asians or Hispanics. However, they also tend to have a better prognosis than non-Caucasians. Age at diagnosis: Children between 1–10 years of age are most likely to develop ALL and to be cured of it. Cases in older patients are more likely to result from chromosomal abnormalities (e.g., the Philadelphia chromosome) that make treatment more difficult and prognoses poorer. White blood cell count at diagnosis of less than 50,000/µl
Cancer spread into the Central nervous system (brain or spinal cord) has worse outcomes. Morphological, immunological, and genetic subtypes Patient's response to initial treatment Genetic disorders such as Down's Syndrome
Generalized weakness and fatigue Anemia Breathlessness Frequent or unexplained fever and infection Weight loss and/or loss of appetite Excessive and unexplained bruising Bone pain, joint pain (caused by the spread of "blast" cells to the surface of the bone or into the joint from the marrow cavity) Enlarged lymph nodes,liver and/or spleen Edema (swelling) in the lower limbs and/or abdomen Petechia, which are tiny red spots or lines in the skin due to low platelet levels
Diagnosing ALL begins with a medical history, physical examination, complete blood count, and blood smears. Because the symptoms are so general, many other diseases with similar symptoms must be excluded. Typically, the higher the white blood cell count the worse the prognosis. Blast cells are seen on blood smear in majority of cases (blast cells are precursors (stem cells) to all immune cell lines). A bone marrow biopsy is conclusive proof of ALL. A lumbar puncture (also known as a spinal tap) will tell if the spinal column and brain have been invaded.
Acute refers to the relatively short time course of the disease (being fatal in as little as a few weeks if left untreated) to differentiate it from the very different disease of chronic lymphocytic leukemia, which has a potential time course of many years. It is interchangeably referred to as Lymphocytic or Lymphoblastic. This refers to the cells that are involved, which if they were normal would be referred to as lymphocytes but are seen in this disease in a relatively immature (also termed 'blast') state.
Pathological examination, cytogenetics (in particular the presence of Philadelphia chromosome), and immunophenotyping establish whether myeloblastic (neutrophils, eosinophils, or basophils) or lymphoblastic (B lymphocytes or T lymphocytes) cells are the problem. RNA testing can establish how aggressive the disease is; different mutations have been associated with shorter or longer survival. Immunohistochemical testing may reveal TdT or CALLA antigens on the surface of leukemic cells. TdT is a protein expressed early in the development of pre-T and pre-B cells, whereas CALLA is an antigen found in 80% of ALL cases and also in the "blast crisis" of CML.
Medical imaging (such as ultrasound or CT scanning) can find invasion of other organs commonly the lung, liver, spleen, lymph nodes, brain, kidneys, and reproductive organs.
Initial symptoms are not specific to ALL, but worsen to the point that medical help is sought. They result from the lack of normal and healthy blood cells because they are crowded out by malignant and immature leukocytes (white blood cells). Therefore, people with ALL experience symptoms from malfunctioning of their erythrocytes (red blood cells), leukocytes, and platelets. Laboratory tests that might show abnormalities include blood count tests, renal function tests, electrolyte tests, and liver enzyme tests. The signs and symptoms of ALL are variable but follow from bone marrow replacement and/or organ infiltration.
On Friday, July 13th 2012 we took Mykayla back to the pediatrician. While checking for pneumonia they discovered a basketball sized mass (of lymphoblasts) in her chest. We were sent to Portland Oregon where the nearest children’s hospital is and the very next day she underwent spinal taps and bone marrow biopsies and was subsequently diagnosed with Leukemia. Mykayla’s mass was so large that she was not able to be sedated for risk of death from the pressure on her esophagus and heart. Some of the valves that drain fluid from around your heart were blocked by the mass causing her pericardial effusion (fluid around the heart). Mykayla spent 3 days in the Intensive care unit and had to undergo an entire surgery to place a PICC line with no anesthetic or pain relief at all due to the risk it posed on her condition.
Leukemia is the most common form of childhood cancer.
Mykayla fell ill in May of 2012. She had flu like symptoms such as body aches, fevers, fatigue, cough, and runny nose. She was evaluated by an urgent care office and diagnosed with strep throat. They prescribed antibiotics but she did not get better. She soon developed a red rash that covered her ankles. Her bruises that covered her body were alarming to us… as she was too tired to play outside where she normally got bruises like this. Her tummy began to hurt badly and we took her to her pediatrician. He wanted us to remove dairy from her diet… to see if that solved the issues we were having, but It continued to get worse. She began sweating during the night, so badly that it would soak through her sheets into her mattress. Her breath was shallow… and her skin was pale cool to the touch. Her heart beat was funny, we couldn’t pin-point what exactly was different but the way her pulse looked was different. We feared that we would lose her if they did not find out what was wrong.
Acute lymphoblastic leukemia (ALL) is a form of leukemia, or cancer of the white blood cells characterized by excess lymphoblasts. Malignant, immature white blood cells continuously multiply and are overproduced in the bone marrow. ALL causes damage and death by crowding out normal cells in the bone marrow, and by spreading (infiltrating) to other organs. ALL is most common in childhood with a peak incidence at 2–5 years of age, and another peak in old age. The overall cure rate in children is about 80%, and about 45%-60% of adults have long-term disease-free survival.
Mykayla has T-cell Acute Lymphoblastic Leukemia.
This is a very rare and aggressive form of childhood leukemia; it accounts for 15-18% of childhood leukemia cases. Leukemia is cancer of the blood and bone marrow. Mykayla’s DNA was altered someway and it caused her bone marrow to start producing immature/leukemic white blood cells.These immature white blood cells are known as “lymphoblasts”. Lymphoblasts never die like a normal cell so they build up and accumulate in the chest area causing a mass (this is specific to T-cell phenotype).
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A brave Young girl with a unique & inspiring path through childhood leukemia