congenital leukemia
Introduction
Introduction to congenital leukemia Congenital leukemia (CL) is a rare type of leukemia that refers to leukemia diagnosed from birth to 8 weeks after birth. The etiology and pathogenesis are still unclear and may be related to genetic and physical abnormalities. Often accompanied by congenital malformations, such as 21-trisomy syndrome, Turner syndrome. basic knowledge The proportion of illness: 0.001% Susceptible people: infants and young children Mode of infection: non-infectious Complications: purpura
Cause
Cause of congenital leukemia
(1) Causes of the disease
The etiology of congenital leukemia is still unclear. It is generally believed to be related to the physical and genetic factors of the newborn and the intrauterine environmental factors. There is a clear genetic predisposition in the same twins, such as leukemia before the age of 6 years. Another risk of leukemia is 20%, usually occurring within a few months after the first onset. The incidence of leukemia in twins and siblings is 2 to 4 times higher than in children. Chromosomal abnormalities in congenital leukemia are more common in 9 - Trisomy, 13-trisomy, Turner syndrome and Down syndrome, the literature believes that congenital leukemia may be one of the causes of stillbirth, mostly occurred in the 34 to 38 weeks of pregnancy, accompanied by fetal edema, suggesting that the lesion is in the palace It already exists, the histological pathology of the fetus and placenta (intrauterine environmental factors) including immunohistochemistry and electron microscopy analysis may help to understand the prenatal behavior of congenital leukemia.
(two) pathogenesis
still not clear.
Prevention
Congenital leukemia prevention
Through daily exercise and activities, the physical and physical health is strong, and the ability to resist disease is enhanced. Secondly, optimistic emotions, positive attitudes to life, and all kinds of illnesses mean that bad emotions are the root cause of illness. Learn to calm down in daily life, eliminate troubles, and avoid excessive emotional changes to the human body.
Complication
Congenital leukemia complications Complications
The same complications as childhood leukemia. The incidence of leukemia in children is 3/100,000-4/100,000. In children with acute leukemia, acute lymphoblastic leukemia (ALL) is about 65%, and acute myeloid leukemia (AML) is about 25-30%. In the past 30 years, with the comprehensive and in-depth study of the disease, human understanding of its essence and clinical treatment effects have been greatly developed. Children ALL has become a malignant tumor that can be cured. There are many manifestations in the early stage, such as pale, general malaise, exertion, loss of appetite, some fever for unknown reasons, some with osteoarthritis, some manifested as local bleeding, nosebleed, skin purpura or persistent bleeding after minor surgery. More than, from the above symptoms, pale and fever are the most common.
Symptom
Congenital leukemia symptoms common symptoms nosebleeds bleeding tendency black stool lymph node enlargement gene fusion intracranial hemorrhage shortness of sleepiness skin infiltration herpes
1. General symptoms of fever, lethargy, loss of appetite, weight loss, shortness of breath and so on.
2. Skin damage 50% of the pathology has a variety of skin infiltration, from birth to several weeks after birth, skin nodules diameter 0.2 ~ 0.3cm, blue gray or purple, no adhesion to the subcutaneous tissue, distribution In the head, cheeks, trunk and limbs, from soft to hard, can also be papules, eczema or herpes-like damage, neonatal acute monocytic leukemia (Amol) skin nodules can be self-relieving, but weeks or months After recurrence, eye involvement and green tumors.
3. Most of the bleeding tendency can be seen in the skin's stasis, ecchymosis, sometimes visible hematemesis, melena, severe intracranial hemorrhage is life-threatening.
4. Liver is large, splenomegaly is common, liver and spleen can be severely swollen and enter the pelvic cavity, but there is very little lymphadenopathy.
5. Central nervous system leukemia occurs early, once diagnosed after birth, leukemia cells can be found in the cerebrospinal fluid.
6. In addition to the above symptoms, there may be symptoms associated with lung, heart, kidney, peritoneum and other organ involvement.
The diagnostic criteria for congenital leukemia are:
1 A large number of myeloid cell lines or lymphocyte naive cells appear in blood or bone marrow.
2 has the performance of extramedullary infiltration.
3 exclude leukemia-like reactions.
Examine
Examination of congenital leukemia
1. Peripheral blood at birth of hemoglobin at 70 ~ 200g / L, white blood cells increased significantly, up to 15 × 109 / L; dominant cells predominance, platelets <70 × 109 / L, peripheral blood smear visible teardrop and nucleated Red blood cells.
2. Bone marrow hyperplasia is extremely active, clear undifferentiated cells, more common in acute myeloid leukemia (AML), accounting for 70% to 80%, acute lymphoblastic leukemia (ALL) is less, the ratio of the two is about 2:1 ~4:1, AMOL is more common in acute myeloid leukemia, accounting for 20%. Some rare types of leukemia, such as erythroleukemia, basophilic leukemia, and double or double phenotype leukemia, can also be seen. Myeloid surface antigens, immunofluorescence and electron microscopy of monoclonal antibodies contribute to immunophenotyping, chemical staining of lipidated luciferin hydrolase, spermine cells, etc.
3. Genetic changes change about 50% of children with chromosomal abnormalities, the most common is t (4; 11), is considered to be a characteristic change in ALL accounted for about 78%, only 10% of children with AML have this abnormality, in There are many diseases on chromosome 11 involving 11q in the same zone, and t(4,11)(q21,q23) is one of the consistent cellular genetic changes in human ALL, in which ALL breakpoint is limited to 4q13-21, while 11q23 The external rupture can be seen in the lymphoid and myeloid cell lines. It can be seen that the expression gene carried by 11q23 is non-specific, and the obvious high rate of 11q23 rearrangement in congenital or childhood leukemia indicates that this gene region is in the fetus and adult. Inv(16)(p13q22) has high specificity in MLL-M4 of ANLL, and it is considered that 16q23 breaks the key to inv(16)(p13q22) abnormality in AML11q23 and 19q and 19p. Lateral translocation is also more common, chromosomal abnormalities of congenital leukemia can return to normal after treatment, reappear when recurrence, and its reproduction is earlier than bone marrow and clinical symptoms, so it can accurately predict the recurrence of leukemia.
4. Skin rash biopsy has certain value for diagnosis, mostly myeloid cell line, and a few are lymphocyte and monocyte cell lines.
5. According to the condition, clinical manifestations and symptoms, physical signs are selected for ECG, X-ray, B-ultrasound, biochemical examination.
Diagnosis
Diagnosis and diagnosis of congenital leukemia
Mainly should be differentiated from neonatal leukemia, more common in severe infection, neonatal sepsis, etc., the total number of peripheral white blood cells is greater than 50.0 × 109 / L, and visible immature cells, hepatosplenomegaly, blood culture should be done at this time, bone marrow , leukemia alkaline phosphatase assay to assist in diagnosis.
In vitro bone marrow culture facilitates early differential diagnosis, semi-solid bone marrow culture detects untreated AML, showing lack of colony formation, increased cluster formation, bone marrow from no growth to excessive cluster formation, untreated ALL showing no growth or colonies The rate of formation decreased. In summary, untreated acute leukemia showed a decrease in the number of colonies and colony/cluster ratio. Conversely, children with Down syndrome who responded to leukemia had normal maturation into granulocytes, monocytes, in bone marrow culture. Normal colonies of macrophages formed and the ratio of colonies to colonies/clusters was normal.
