Leukemia is classified into acute and chronic based on degrees of maturation malignant cells in the bone marrow. Leukemia acute marked the disruption that results in increased maturation cells young and going on the failure of the differentiation of blood cells. This causes the disease looks very heavy and causing death within a few months without treatment.
Instead on an increase in leukemia cells mature Chronicle is not controlled, so that the disease seems relatively lighter. Leukemia, chronic at the stadium late can be progressive as acute leukemia.
Leukemia mielositik Chronicle (LMK) is a disease of the ferocity of clonal system hemopoetik pluripotensial hiperproliferasi of the system includes cells, monocytes, granulocytes, system, Eritroid and Megakariosit. Leukemis Mielositik Chronicle is also often called Leukemia Myelogenous Leukemia Granulositik Chronicles, the Chronicle and Chronicle of Myeloid Leukemia.
LMK rarely encountered in children and is estimated to be only a 1-5% of cases of Leukemia. The lowest age that undiagnosed LMK is 3 months.
According to some reports the case of LMK is higher in people who work in units of radiology, people exposed to radiation from the atomic bomb, the sufferer who got radiation therapy for the disease Ankylosing Spondylitis and other diseases. However, only 5-7% of the reported cases of LMK is related to lack of exposure to radiation and it is very rarely about a group of children. On young people, especially those exposed when age under 5 years will improve incident LMK, but not found an increased incidence in infants in the content of his while pregnant. In schematic changes which occur from the time of the initiation of preleukemia and finally to leukemia.
Pathogenesis
LMK is first encountered the ferocity of the diseases related to specific genetic abnormalities in krosomom number 22 (LP ' chromosomes. On more than 90% of patients there is a change of normal bone marrow cells with abnormal chromosome Group G (number 22)-the Philadelphia chromosome Abnormality occurs or Ph. due to the translocation section long arm (q) of chromosome 22 to another chromosome, often chromosome 9 in the "C". This is akuisita abnormality that exists in all cells granulositik, eritroid and megakariositik which are split in the bone marrow and also in cells limposit b. large increase in mass graulosit total body responsible for most of the clinical picture.
Changes of activity of tyrosine kinase which led to the transformation of the provider's underlying incidence of LMK. The onset of the crisis on LMK blastik associated with the emergence of the genes that produce cyklin-dependent kinase-2 inhibitor (CDKN-2) or known as Ph '-2 chromosomes at chromosome number 9, in which a gene has a malignant cell growth enabled properties. In particular the acceleration phase, and blast.
Travel diseases
Travel sickness LMK is divided into 3 phases namely, a phase of chronic, accelerated and blas.
1. phases of the Chronicle
The expansion phase of the Chronicles of the marked the height of the hemopoetik pool with increased formation of mature blood cells, with little functional disorders. Generally cell neoplasm little found in bone marrow, hepar, lien and blood peripheral. Long time phase Chronicle generally 3 years.
On physical examination the patient looked pale, ekimosis, hepatosplenomegaly and sternum pain. Symptoms are related to the degree of leukositosis sometimes (20%) asimptomatis and is found by chance. Leukositosis very heavy (> 500,000/mm3) can be found in children. Examination of blood banks found entire undelete stage differentiation such as myeloblas and promileosit are generally under 15%, as well as not found leukemikus hiatus. Also found an increase in the absolute basophils and eosinophils.
Myelofibrosis is generally rare in the Chronicle, and can be found at 30-40% of sufferers. Abnormalities of granulocytes can be known by the presence of alkaline phosphatase activity of leukocytes decreased (LAP) with examination sitokimia. Diagnosis-Chronicle lekemoid reaction phase of LMK, LMK type juvenil and other myeloproliferatif diseases. On lekemoid, splenomegali are usually not notable, the activity increases, the Ph of LAPS ' chromosome negative, leukositosis and splenomegali are not as powerful as the LMK and involves the organs like the skin and the glands of the spleen. Disease myeloproliferatif is distinguished from the LMK to the examination of granulocytes series Ph ' chromosomes.
Accelerated Phase
After approximately 3 years, LMK Chronicle will be the phase of accelerated with increasing progressives disease. About 5% of cases, sudden changes occurred with the rapid increase in peripheral blood cell blas (crisis blas). Symptoms and signs of a phase of accelerated:
-Heat without causing a clear and progressive splenomegali
-Anemia and trombositopnia after the previous had normal
-Trombositosis > 1000 x 109/L
-> 20% Basophils and myeloblas > 5
-Description of myelodisplasia such as hipogranulasi nuetrofil, micro megakariosit or large mononuclear.
-Fibrosis of collagen on bone marrow
-There are abnormal chromosome such as the new ' Ph-chromosome 2
-Increased uptake timidin by neutrophils
-Increased content of DNA and the decline in the fraction of proliferation.
Phase blas
In this phase of clinical symptoms include anemia, thrombocytopenia and improved blood cell blas on the edge and bone marrow. In the bone marrow is found more than 30% of the cells of the blas which is a sign of the diagnostic phase. Cell blast dominated by myeloid cells but cells eritroid, megakariositik and lymphoblast can be found. Clinical symptoms in this phase is the same as acute leukemia and if cell blas reached more than 100 000 per mm3 then sufferers have a risk of occurrence of hiperleukositosis syndrome. This phase is distinguished with acute leukemia in which splenomegali is not notable, basofilia and the .pH-2 chromosomes.
Complications
Some problems in the handling of LMK:
1. metabolic Problems
Metabolic problems occurred due to the immediacy of sitolisis, which will lead to the occurrence of hiperurikemia, hiperkalemia and hiperfosfatemia. It should be in anticipation of, and in therapy with granting ooze, alkalinisasi and allupurinol.
2. Hiperleukositosis
Extreme increase of leukocytes on LMK can cause complications leukostatik on some organs particularly the brain, lung, retina and penis. Since leukocytes less balanced with erythrocytes will an increase in the viscosity of the blood due to increased fraction of leukocytes. Aterapi includes a provision granting ooze, analgetik, warm compresses, radiotherapy (on the penis or lien) and provision of high-dose chemotherapy (50-74 mg/kgbb/day intravenously).
4. Meningeal Leukemia
Meningeal Leukemia in chronic phase of LMK is often unknown and rarely encountered on stadium blas. The symptoms that are found to be the nerve center of paralysis and udema papil. The Diagnosis is aided by the discovery of cerebrospinal fluid cells blas on. 5. Myelofibrosis
LMK frequently occur together with myelofibrosis and will increase the production of collagen on bone marrow or decline the degradation of collagen.
Therapy
The general aim of the therapy phase chronic sufferers LMK on eliminating the symptoms of the clinic is in a way down the leukositosis and organomegali. Complete remission is the loss of Ph ' + clones and turn cells by normal cells are rare with conventional medicine. However with the technique of transplant sumsumtulang, healing is possible, the goal of therapy in a phase of accelerated LMK and blas is the return to the phase of the Chronicle.
Therapy of chronic phase LMK
The standard treatment is with LMK phase chronic drug single, although most cases are rare healing. With a single drug will happen reduction organomegali and leukocytes in blood banks to be normal but hyperplasia granulocytes and metaplasia Ph ' + in the bone marrow still occur. Drugs are often given as a single drug is:
Busulfan
This drug is used the first time in 1950 in the treatment of LMK, and to present a standard remedy. Is non-specific drug ankilating on cell cycle phase, the work interfere with the function of DNA-and guanine and timidin of malignant cells. The hallmark of this drug has a slow onset and long duration. The dose used is 2-6 mg/kgbbperoral single dose.
The dose was reduced to 50% if the levels of leukocytes reaching 30,000-40,000/mm3 and is stopped if the down reach less or equal to 20,000/mm3.
Prognosis
The average life expectancy of sufferers LMK is 3-4 years from the time a diagnosis is made. Only 30% of these patients survive to 5 years. When you have come to the phase of blas then death will occur after the 1-5 months due to the failure of the bone marrow.
