When is splenectomy indicated in thalassemia?

When is splenectomy indicated in thalassemia?

Physicians often use splenectomy to decrease transfusion requirements in patients with thalassemia major. (Patients with thalassemia minor only rarely require splenectomy.) Splenectomy is recommended when the calculated annual transfusion requirement is greater than 200-220 mL RBCs/kg/y with a hematocrit value of 70%.

Why splenectomy is indicated in thalassemia?

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).

What are the indications for splenectomy?

Indications for splenectomy include hypersplenism, incidental to other abdominal procedures, trauma, Hodgkin’s staging, and other splenic disease. Hodgkin’s staging has become the most frequent indication for splenectomy during the past five years, whereas splenectomy for hypersplenism has decreased.

How does thalassemia affect 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 are the complications of splenectomy?

Splenectomy Complications

  • Blood clot in the vein that carries blood to the liver.
  • Hernia at the incision site.
  • Infection at the incision site.
  • Inflammation of the pancreas (pancreatitis)
  • Lung collapse.
  • Injury to the pancreas, stomach, and colon.

Why splenectomy is done in anemia?

cases of acquired hemolytic anemia splenectomy will remove the major site of red cell destruction and in turn increase the hemoglobin concentration and abolish the need for an accelerated rate of red cell production.

What is the most common complication of splenectomy?

Infections, particularly pulmonary and abdominal sepsis, constitute the majority of the complications. The mortality rate from postoperative sepsis is substantial. Atelectasis, pancreatitis/fistula, pulmonary embolism and bleeding at the operative site are also relatively common occurrences following splenic removal.

What vaccines should be given before splenectomy?

Ideally, vaccination with both types of pneumococcal (conjugate [PCV] and polysaccharide [PPSV]), both types of meningococcal (MenACWY and MenB), and Hib vaccine should all be completed at least 2 weeks before a scheduled splenectomy, if time permits.

Why is spleen enlarged in thalassemia?

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.

What organs take over after a splenectomy?

What organ takes over after spleen removal? After splenectomy, the functions of the spleen are usually taken up by other organs, such as the liver, bone marrow, and lymph nodes.

For what type of anemia is recommended a splenectomy?

Splenectomy is useful in the treatment of autoimmune hemolytic anemias and in certain hereditary hemolytic disorders (ie, hereditary spherocytosis and elliptocytosis, certain unstable Hb disorders, pyruvic kinase deficiency).

How does splenectomy correct anemia?

What are the signs and symptoms of thalassemia major?

Clinical presentation of thalassemia major occurs between 6 and 24 months. Affected infants fail to thrive and become progressively pale. Feeding problems, diarrhea, irritability, recurrent bouts of fever, and progressive enlargement of the abdomen caused by spleen and liver enlargement may occur.

What happens to the spleen in thalassemia?

An enlarged spleen. The spleen is an organ that helps your body fight infection and remove unwanted material. When a person has thalassemia, the spleen has to work very hard. As a result, the spleen becomes larger than normal.

When is splenectomy indicated in the treatment of thalassemia intermedia?

As hypersplenism may cause worsening anemia, retarded growth and mechanical disturbance from the large spleen, splenectomy is a relevant aspect of the management of thalassemia intermedia.

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.