How long can a person with congenital anemia live

How long can a person with congenital anemia live

If the symptoms of anemia are not treated promptly, it can have serious consequences for life. Anemia can be caused by acquired factors or congenital factors. So how long can people with congenital anemia live? Congenital anemia refers to a disorder in the body's hematopoietic function, which is a problem with the body's immune system. If a child has this disease, symptoms will appear when the child is about one year old. If the treatment is appropriate and timely, the survival rate is still very high.

1. Can congenital anemia be cured?

Simple red cell aplasia. Clinically it is referred to as pure red blood cell aplastic anemia! Similar to selective aplastic anemia, the onset of the disease is mainly related to the patient's own immune system and is generally divided into acquired and congenital.

The chance of curing pure red cell aplastic anemia is related to several factors. One is the immune status of the individual. Another and most important one is whether there is a suitable bone marrow match. The third point is the family's economic conditions. We all know that the cost of treating ordinary aplastic anemia is not low, not to mention the rare pure red cell aplastic anemia!

To treat pure red aplastic anemia, we must first understand whether it is primary or secondary. After finding out the cause, we can treat it according to the situation. If it is primary acquired pure red aplastic anemia, the combined use of adrenal cortex hormones, androgens and tripterygium wilfordii polyglycosides can improve the efficacy. The treatment of this disease must be carried out under the advice and guidance of a doctor, and drugs must not be used privately!

Parents should pay attention to whether their children have symptoms of pure red aplastic anemia if someone in the family has had pure red aplastic anemia. Generally, 90% of congenital pure red aplastic anemia occurs at around one year old. Careful parents will detect and treat it in time. Early treatment of pure red aplastic anemia is very helpful to the cure rate of the disease!

2. Causes of congenital anemia

Congenital aplastic anemia is occasionally seen in siblings, suggesting that congenital aplastic anemia is a hereditary disease. Less than 10% of patients have a family history, and the majority of the rest are sporadic. One third of patients have autosomal dominant inheritance, and the rest have recessive inheritance. Linkage analysis revealed that there are at least three genetic loci for DBA, two of which have been identified, namely 19q13.2 and 8p23.3-p22. The related pathogenic gene has been cloned in the 19q13.2 region, which is the ribosomal protein S19 (RPS19) gene. Sequence analysis found that approximately 25% of DBA patients had RPS19 mutations.

3. Complications of congenital anemia

Patients with congenital pure red cell aplasia are prone to multiple malignant tumors. There are more than 480 cases of congenital pure red cell aplasia reported in the literature, of which 12 developed malignant tumors 2 to 43 years after diagnosis, including 6 cases of acute myeloid leukemia (AML), 1 case of acute lymphoblastic leukemia (ALL), 2 cases of Hodgkin's disease (HD), 2 cases of myelodysplastic syndrome (MDS) and 1 case of hepatocellular carcinoma.

Long-term use of hormone therapy may be accompanied by developmental disorders and secondary infections.

Progression of the disease may lead to heart failure, and in the late stage, excessive blood transfusion may lead to secondary hemochromatosis or cardiac cirrhosis.

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