Chronic myeloid leukemia typing and clinical performance

During chronic leukemia in children less, or about childhood leukemia 3% to 5%, mainly for chronic myeloid leukemia (chronic myelogenors leukemia, CML).
CML in the infancy of clinical manifestations and adult CML significant difference, it will generally be divided into pediatric CML juvenile and adult type. The literature also divided into baby-type, the family type, type of child care, adult-type IV, of whom family type and the performance of similar infantile, often close relatives only in the pathogenesis.
1. Juvenile chronic myeloid leukemia (juvenilechronic mydogenous leukemia, JCML) almost all of this occurred in children under 5 years old, especially 2-year-old infants under common. The incidence of women than men. Can occur in familial neurofibroma, reproductive or urinary tract malformations in children with mental retardation.
Onset can be acute or ease, often dominated v. respiratory symptoms. Common facial or eczema-like skin rashes, and even for purulent skin rash, can be seen in the skin of coffee spots, skin symptoms can be seen in the leukemia cell infiltration previous months. Lymphadenopathy, or even for suppurative. Sexual hepatosplenomegaly. Due to a decrease in platelet secondary bleeding is not uncommon.
JCML originated in multipotent hemopoietic stem cells, it can cause erythroid hyperplasia obstacles, the number and quantity of platelet abnormalities, as well as lymphocyte dysfunction. Adult and different, mainly in its dysplasia tablets single system, stem cells cultured in vitro mainly formed CFU-GM. Normal chromosome too many inspections, individual Visibility -7, +8 (8 three-body) or +21 (21 three-body).
Peripheral blood leukocytes increased platelet reduce and moderate anemia. Interleukin moderately higher, and more than 100 × 109 / L. Tablets of immature cells and nucleated red blood cells in peripheral blood may arise, and mononucleosis. Interleukin alkaline phosphatase decreased, even normal. Serum and urinary lysozyme increased. HbF increased. Bone marrow tablets: red for 3 ~ 5:1. Tablets of the proliferation of monocytes and vigorous, visible erythroid hyperplasia anomaly. The original tablets of cells in less than 20%. Megakaryocyte reduction. Bone marrow cells cultivated in vitro monocyte-based.
As JCML often fever, hepatosplenomegaly, moderate anemia, leukocytosis, due to infection with the type of leukemia reaction differential. Also with infectious mononucleosis of identification.
2. Adult chronic myeloid leukemia (adultchronic myelogenous leukemia, ACML) incidence over the age of 5 years old to 10 ~ 14-year-old see more rarely found in children under 3 years old. Little difference between men and women. Because it is more to stem the proliferation of malignant cells, it granulocyte, erythroid, megakaryocytic, and so more of involvement, the period of radical change can be changed lymphoblastic leukemia. About 85 percent of children with Ph1 chromosome (ie, t (9:22)). The Ph1 chromosome-negative, using molecular biology techniques can be divided into a bcr Reorganization (phbcr + CML) and non-bcr Reorganization (PH - bcr - cml) two subtypes. The former clinical symptoms and PH1 chromosome positive similar, the latter is not typical clinical symptoms.
Incidence slow at the beginning of lesser symptoms showed weakness, weight loss, bone and joint pain. Giant signs visible spleen, liver enlargement, mildly swollen lymph nodes, papilledema, and so on. Few bleeding.
As for the main peripheral blood leukocytes increased 80% in 100 × 109 / L and above. Hemoglobin in the 80 g / L. Thrombocythemia. Increased Visibility tablets of the classification, including eosinophils, cells of Alkaliphilic tablets increased. The original tablets cells increased not obvious to, the late David and mature cell-based tablets. Leukocyte reduction of alkaline phosphatase. HbF not increased. Serum immunoglobulin not increased. Myelodysplastic active, mainly in tablets of hyperplasia, the original tablets of <10%, mostly in the late David tablets of rod-shaped cells and nucleated cells. Tablets: red for 10 ~ 50:1. Some patients Visibility myelofibrosis. Bone marrow megakaryocyte cells markedly increased, mainly mature megakaryocyte cells. Serum and urine lysozyme not increased, but VitB12 and VitB12 carrying protein increased. Bone marrow cultured colony Cong charged with both increased.