Keywords: clinical manifestations of leukemia
Chronic leukemia in children during the less, accounting for childhood leukemia from 3% to 5%, which is mainly for chronic myeloid leukemia (chronic myelogenors leukemia, CML).
The CML in clinical manifestations of infancy and adult CML significant difference, it is generally divided into juvenile and adult children with CML. The literature also divided into infantile, family type, child care, adult type IV, which familial infantile performance is similar, but often close relatives in the incidence.
1. This type of juvenile chronic myelogenous leukemia (juvenilechronic mydogenous leukemia, JCML) almost all occurred in children under 5 years of age, more common in infants and young children, especially in less than 2 years old. The incidence of men than women. Can occur in familial neurofibroma, genitourinary abnormalities and mental retardation in children.
The onset can be acute or slow, often the chief complaint of respiratory symptoms. Common facial rash or eczema-like rash, even purulent rash, visible skin coffee spots, skin symptoms, there may be several months before the infiltration of leukemic cells. Swollen lymph nodes, and even purulent. Progressive hepatosplenomegaly. Thrombocytopenia and hemorrhage are not uncommon.
JCML originated in pluripotent hematopoietic stem cells, it can be the cause erythroid hyperplasia obstacles, abnormal platelet count, and lymphocyte dysfunction. Unlike adult type, the abnormal proliferation of granulocyte system, in vitro stem cell culture formed mainly CFU-GM. Chromosome is normal, individual visible -7, +8 (trisomy 8) or +21 (trisomy 21).
Peripheral blood white blood cells, platelets reduce and moderate anemia. The increased leukocyte moderate, more in 100 × 109 / L or less. Immature granulocytes and nucleated red blood cells in the peripheral blood, and mononucleosis. Leukocyte alkaline phosphatase decreased even normal. Increased serum and urine lysozyme. The HbF higher. Bone marrow granulocyte: red for 3 to 5:1. Granulocytic and monocytic hyperplasia strong, visible abnormal erythroid hyperplasia. Myeloblast at below 20%. Megakaryocytes reduced. Cultured bone marrow cells in vitro mainly monocytes.
Due JCML often fever, hepatosplenomegaly, moderate anemia, leukocytosis, and the infection caused by the leukemia-like reaction to identify. Histiocytosis with infectious mononucleosis should also identify.
2. Adult chronic myeloid leukemia (adultchronic myelogenous leukemia, ACML) age of onset in more than five years of age, 10 to 14 years old were more common, rarely seen in children under 3 years old. Little difference between men and women. The pluripotent hematopoietic stem the proliferation of malignant cells, myeloid, erythroid, megakaryocytic multi-system involvement, The blastic phase can be transformed into lymphoblastic leukemia. About 85% of children with Ph1 chromosome (t (9:22)). Ph1 chromosome negative, can be divided into the bcr restructuring (phbcr + CML) and bcr-free reorganization (PH-bcr-cml) subtypes using molecular biology techniques. Similar the former clinical symptoms and PH1 chromosome-positive, the latter are not typical clinical symptoms.
Slow onset, mild symptoms in the beginning, as fatigue, weight loss, bone and joint pain. The signs visible splenomegaly, enlargement of the liver, lymph nodes, mild swelling, papilledema. There is little bleeding symptoms.
Peripheral blood leukocytosis, 80% in more than 100 × 109 / L. Hemoglobin in about 80g / L. Thrombocytosis. Classification visible increase in granulocytic, including eosinophils, basophils increased. The myeloblast increased is not obvious, mainly in late immature and mature granulocytes. Leukocyte alkaline phosphatase to reduce. Increased HbF not. The serum immunoglobulin does not increase. Bone marrow hyperplasia, myeloid hyperplasia myeloblast <10%, mostly in late promyelocytic and rod-shaped cells. Tablets: red for 10 to 50:1. Seen in some patients myelofibrosis. Bone marrow megakaryocytes increased significantly, mainly mature megakaryocytes. Serum and urinary lysozyme is not increased, but increased VitB12 and VitB12 carrying protein. Bone marrow culture colony Cong drop all increased.