1.juvenilechronic myeloid leukemia (juvenile leukemia
mydogenous
Leukemia (JCML) almost always occurs in children under 5 years old, especially in infants under 2 years old. Men have more diseases than women. Can occur in children with familial neurofibroma, genitourinary malformation or mental retardation.
The onset can be urgent or slow, and respiratory symptoms are often the main complaint. More maculopapules or eczema-like rashes, even purulent rashes, can also be seen on the skin, and skin symptoms can appear several months before leukemia cells infiltrate. Lymph nodes are swollen and even purulent. Progressive hepatosplenomegaly. Secondary bleeding due to thrombocytopenia is not uncommon.
JCML originated from pluripotent hematopoietic stem cells, so it can cause erythroid hyperplasia disorder, abnormal platelet number and quantity, and abnormal lymphocyte function. Different from adult type, its abnormal proliferation is mainly in the granulocyte system, and CFU-GM is mainly formed in stem cell culture in vitro. Chromosome examination was mostly normal, with -7, octadeca (8 trisomy) or +2 1(2 1 trisomy) in some cases.
Peripheral blood picture showed leukocytosis, thrombocytopenia and moderate anemia. White blood cells are moderately increased, mostly below100×109/L. Immature granulocytes and nucleated red blood cells can appear in peripheral blood, and mononuclear cells increase. Leukocyte alkaline phosphatase decreased, even normal. Lysozyme in serum and urine increased. HbF increased. Bone marrow granules: red is 3 ~ 5 ∶1. Granulocyte and mononuclear cells proliferate vigorously, and erythroid cells proliferate abnormally. The number of granulocytes is below 20%. Megakaryocytopenia. In vitro bone marrow cell culture is mainly mononuclear cells.
Because JCML often has fever, hepatosplenomegaly, moderate anemia and leukocytosis, it should be differentiated from leukemia-like reaction caused by infection. It should also be differentiated from infectious mononucleosis.
2. adultchronic myeloid leukemia (adult chronic)
myelogenous
Leukemia (ACML) is more than 5 years old, especially10 ~14 years old, and rarely seen in children under 3 years old. There is not much difference between men and women. Because of the malignant proliferation of pluripotent hematopoietic stem cells, many lines such as granulocyte, erythroid and megakaryocyte are involved, and they can turn into lymphoblastic leukemia in the acute phase. About 85% of children have Ph 1 chromosome (that is, t(9∶22)). Those with negative Ph 1 chromosome can be divided into two subtypes by molecular biology technology: bcr recombination (phbcr+CML) and bcr-free recombination (Ph-bcr-CML). The former has similar clinical symptoms to those with positive Ph 1 chromosome, while the latter has atypical clinical symptoms.
The onset is slow, and the symptoms are mild at first, showing fatigue, weight loss and joint pain. Signs can be seen as splenomegaly, hepatomegaly, slight enlargement of lymph nodes and edema of optic papilla. There are few bleeding symptoms.
The peripheral hemogram is mainly leukocytosis, with 80% above100×109/L. Hemoglobin is about 80g/l. Thrombocytosis Classification shows that the number of granulocytes increases, including eosinophilia and basophilia. The increase of primordial granulocytes is not obvious, and it is mainly middle, late and mature granulocytes. Leukocyte alkaline phosphatase decreased. HbF does not increase. Serum immunoglobulin is not increased. Bone marrow hyperplasia is active, mainly granular hyperplasia, with primordial granulocytes <10%, mostly middle and late granulocytes and rod-shaped nuclear cells. Grain: red is10 ~ 50:1. Bone marrow fibrosis can be seen in some patients. The number of megakaryocytes in bone marrow increased significantly, mainly mature megakaryocytes. Serum and urine lysozyme did not increase, but VitB 12 and VitB 12 carrier protein increased. Colonies and clumps in bone marrow culture increased.