Why is the spleen removed in beta thalassemia?
The genetic defect in thalassaemia also results in formation of ineffective red blood cells, which are removed by the spleen which in turn becomes enlarged. Removing the spleen can lengthen the lifespan of red blood cells and reduce the need for transfusions.
What are the indications for splenectomy?
Your doctor may recommend splenectomy if you have one of the following:
- Ruptured spleen. If your spleen ruptures due to a severe abdominal injury or because of an enlarged spleen (splenomegaly), the result may be life-threatening, internal bleeding.
- Enlarged spleen.
- Blood disorder.
- Cancer.
- Infection.
- Cyst or tumor.
Why is there no splenomegaly in thalassemia?
From this study we concluded that thalassemia patients do suffer from splenomegaly because of extramedullary hematopoiesis, increased RBC destruction, repeated blood transfusions, and iron overload.
Does beta thalassemia affect the spleen?
An enlarged spleen in individuals with beta thalassemia may occur due to increased destruction of red blood cells, the formation of blood cells outside of the bone marrow (extramedullary hematopoiesis), repeated blood transfusions, or iron overload.
When is splenectomy done in thalassemia?
Splenectomy should be considered when: Annual blood requirements exceed 1.5 times those of splenectomised patients, provided that they are on the same transfusion scheme and have no other reasons for increased consumption.
What is the role of spleen in thalassemia?
In its filtering capacity, the spleen is able to remove large foreign particles from the blood. In addition, when red blood cells come to the end of their lifecycle (after about 30-60 days in thalassemia), the spleen breaks the cells down, removing the globin and iron from these cells so that they can be reused.
Why should patients with thalassemia have splenectomy?
Many patients with thalassaemia require splenectomy. The main therapeutic rationale for splenectomy in transfusion-dependent patients with β-thalassaemia major (TM) is to decrease blood consumption and transfusion requirement with the ultimate goal of reducing iron overload (Rachmilewitz 2011, Cohen 2008).
How does thalassemia reduce spleen size?
The spleen size can be reduced once the patient is transfused at a rate high enough to turn off the need for increased production. Sometimes it may be necessary to treat hypersplenism with a splenectomy (the removal of the spleen.)
How does thalassemia affect the spleen?
Enlarged spleen. Thalassemia is often accompanied by the destruction of a large number of red blood cells. This causes your spleen to enlarge and work harder than normal. An enlarged spleen can make anemia worse, and it can reduce the life of transfused red blood cells.
What is the treatment for β thalassemia minor?
Treatment may include: Regular blood transfusions. Medicines to reduce extra iron from your body (called iron chelation therapy) Surgery to remove the spleen, if needed.
How does thalassemia affect spleen?
Is splenectomy indicated in thalassemia?
Many patients with thalassaemia major require splenectomy. However, optimal clinical management from the time of diagnosis may delay or even prevent hypersplenism, thereby increasing the efficiency of transfusion therapy and reducing the need for splenectomy.
Why are platelets high after splenectomy?
The early, highly significant thrombocytosis, following both splenectomy and general surgery, is caused by increased production of platelets due to the surgical trauma. This is caused by a direct action on bone marrow MK by transforming 2N-8N MK into higher ploidy classes.
How is thalassaemia major (thalassemia major) treated?
Many patients with thalassaemia major require splenectomy. However, optimal clinical management from the time of diagnosis may delay or even prevent hypersplenism, thereby increasing the efficiency of transfusion therapy and reducing the need for splenectomy.
Is splenectomy beneficial for children with thalassemia and hypersplenism?
In conclusion, total splenectomy is beneficial for children with thalassemia and hypersplenism because it reduces their transfusion requirements. Partial splenectomy may be beneficial for those with Hb H disease.
What are the indications for splenectomy in the treatment of hypersplenism?
In general, splenectomy should be avoided unless absolutely indicated. Splenectomy is indicated in the transfusion-dependent patient when hypersplenism increases blood transfusion requirement and prevents adequate control of body iron with chelation therapy.
How is splenectomy performed in the treatment of hemoglobin H–Constant Spring (HCS)?
Full splenectomy can usually be performed by laparoscopic technique. However, open procedure is necessary in cases of marked splenomegaly. The indications for splenectomy in hemoglobin H–Constant Spring patients are different than in beta-thalassemia disorders.