Knowledge about chronic granulocytic leukemia

  I. What is slow granulation Chronic granulocytic leukemia (CML) is a group of granulocytes that exhibit uncontrolled growth in the human bone marrow and accumulate in the bloodstream to form. This acquired bone marrow hematopoietic stem cell, or mature granulocyte, undergoes malignant clonal derivation. The disease arises from a chromosomal translocation phenomenon known as the Philadelphia chromosome (i.e., the long arm of chromosome 9 moves to the short arm of chromosome 22.) CML has a characteristic Philadelphia chromosome that allows the fusion of the proto-oncogene c-abl on chromosome 9 with the bcr gene on chromosome 22 to form the bcr-abl chimeric gene, the genetic hallmark of malignant cloning. Since it is a malignant proliferation of pluripotent hematopoietic stem cells, multilineage involvement such as granulocytic, erythrocytic and megakaryocytic lineages can be transformed into lymphocytic leukemia in the acute phase.  The etiology of most patients is unknown, and ionizing radiation is the only clear risk factor for CML. The incidence of CML has been reported to increase sevenfold among the survivors of the nuclear explosion in Japan, and the incidence is highest among young people in this group, especially children under 5 years of age.  Major clinical manifestations Most patients are in the chronic phase at the time of diagnosis, with a slow onset of symptoms and signs that are relatively mild at the beginning. A small number of patients are asymptomatic and the disease is diagnosed only when an elevated white blood cell count is detected during routine blood tests. Severe bone and joint pain, bleeding, unexplained hyperthermia or extramedullary infiltration are mostly seen in the acute stage.  1. Enlarged liver and spleen lymph nodes: Giant spleen, enlarged liver, mildly enlarged lymph nodes, fullness in the upper abdomen or a mass in the left upper abdomen are seen. In about 90% of patients, the spleen is enlarged to varying degrees and can be found under the rib cage, and the giant spleen is hard and often cut. Severe pain in the splenic region or a fricative sound in the splenic region is a sign of splenic infarction. 50% of patients have mild to moderate hepatomegaly. Lymph node enlargement is rare.  2. CNS involvement: retinopathy, optic disc edema, etc.  3. Skin: A few patients have skin infiltrates and skin nodules.  4.Other: pulmonary dysfunction with arthritis and abnormal penile erection may also appear. About 14% of patients are prone to ulcerative disease, mostly due to basophilia. Leukocytosis due to high primitive cell count is common in children with ACML, but the symptoms are mild.  Laboratory tests 1. Peripheral blood picture: mainly leukocytosis, 80% of which are above 100×10/L. Hematocrit is around 80g/L. Thrombocytosis. Granulocytosis can be seen in the classification, including acidophilic and basophilic granulocytosis. The increase in primitive granulocytes is not obvious, and intermediate, late juvenile and mature granulocytes are predominant.  2. Blood examination: leukocyte alkaline phosphatase is reduced.  Bone marrow examination: active proliferation, mainly granulocytosis, primitive granulocytes <10%, mostly intermediate and late granulocytes and rod-shaped nuclei granulocytes:red 10-50:1. Some patients can see bone marrow fibrosis. Bone marrow megakaryocytes were significantly increased, with mature megakaryocytes predominating. Bone marrow culture colony and clump colony are both increased.  4. Chromosomal examination reveals Ph chromosome.  5. Positive bcr-abl fusion gene.  Treatment Bone marrow transplantation is the only treatment method that can cure children with CML. 980 cases of CML were counted by Gluckman in 1990, and the 5-year disease-free survival rate reached 50% after allogeneic bone marrow transplantation.  However, after 2000, the emergence of TKI therapy, imatinib, a drug specifically developed to target the bcr-abl gene, was able to control the protein product of the bcr-abl gene and control the progression of the disease. Now the vast majority of patients are able to obtain long-term survival, now more than 90% of the 10-year survival. If the results are not good, then conversion to second-generation drugs can be used to bring more than half of the patients under control again.  In the era of modern targeted drug therapy, the disease has been transformed from a blood cancer or malignant tumor to a chronic, manageable disease. However, a small percentage of patients may still have instability and progression of the disease, but most of them have the disease under control, just like hypertension and diabetes, and as long as they continue to take medication, the disease will be well controlled and they can have a normal survival and normal work and life.