Blood transfusion is the mainstay of care for individuals with thalassemia major and many with intermedia. The purpose of transfusion is twofold: to improve the anemia and to suppress the ineffective erythropoiesis. Chronic transfusions prevent most of the serious growth, skeletal, and neurological complications of thalassemia major.
How is a blood transfusion used to treat thalassemia?
This can be done through a blood transfusion, a safe, common procedure in which you receive blood through a small plastic tube inserted into one of your blood vessels. Some people with thalassemia – usually with thalassemia major – need regular blood transfusions because their body makes such low amounts of hemoglobin.
What is a a transfusion?
A transfusion provides the part or parts of blood you need, with red blood cells being the most commonly transfused. You can also receive whole blood, which contains all the parts, but whole blood transfusions aren't common. Researchers are working on developing artificial blood.
Why do people with TDT need blood transfusions?
Since people with TDT cannot make enough hemoglobin on their own, they require regular red blood cell transfusions in order to treat their anemia and keep them alive. Blood from a donor contains working red blood cells that help treat anemia, facilitate normal growth and activity levels, and improve quality of life.
Why would a child need a blood transfusion?
Blood transfusions could be a regular thing if you or your child has a blood disorder called beta thalassemia. The disease causes a drop in the amount of red blood cells you have. A transfusion gives you healthy red blood cells from a donor to make up for the ones you don't have.
How is thalassemia treated?
Why is thalassemia considered immunocompromised?
What is the best vitamin for thalassemia?
How to tell if you have thalassemia?
Where are blood cells made?
Is there a risk of infection from blood transfusion?
Is it safe to get blood transfusions?
See more
About this website
How do blood transfusions help thalassemia?
Blood transfusions could be a regular thing if you or your child has a blood disorder called beta thalassemia. The disease causes a drop in the amount of red blood cells you have. A transfusion gives you healthy red blood cells from a donor to make up for the ones you don't have.
What is the best treatment for thalassemia?
Bone marrow and stem cell transplant from a compatible related donor is the only treatment that can cure thalassemia. It is the most effective treatment.
How often do people with thalassemia need blood transfusions?
The recommended treatment for thalassaemia major involves lifelong regular blood transfusions, usually administered every two to five weeks, to maintain the pretransfusion haemoglobin level above 9–10.5 g/dl.
What are the benefits of blood transfusion?
Blood transfusion can save a patient's life and limit the complications of severe blood loss. A lot of bleeding can lead to a seriously low hemoglobin level and cause damage to body organs due to a lack of oxygen.
Do people with thalassemia need blood transfusion?
Most people with thalassaemia major or other severe types will need to have regular blood transfusions to treat anaemia. This involves being given blood through a tube inserted into a vein in your arm. It's usually done in hospital and takes a few hours each time.
What is transfusion dependent thalassemia?
Anemia causes people to feel tired, weak or short of breath. In the most severe form of beta-thal, also referred to as transfusion dependent thalassemia (TDT), patients require lifelong regular red blood cell transfusions to survive. If left untreated, the disease can damage organs and potentially lead to death.
What happens with blood transfusion?
During a blood transfusion, a healthcare professional will place a small needle into the vein, usually in the arm or hand. The blood then moves from a bag, through a rubber tube, and into the person's vein through the needle. They will carefully monitor vital signs throughout the procedure.
Why does thalassemia cause iron overload?
Individuals with beta thalassemia major and intermedia may develop iron overload, which occurs because of two reasons. First, blood transfusions cause the accumulation of excess iron in the body. Second, beta thalassemia can cause increased absorption of dietary iron by the gastrointestinal tract.
How does bone marrow transplant help thalassemia?
Bone marrow is usually the best source of red cells as it can provide a larger quantity of stem cells than peripheral or cord blood. If successful, BMT can offer a complete and permanent cure to β-thalassaemia patients, including no need for blood transfusions.
When did blood transfusions become safe?
In 1818, British obstetrician James Blundell successfully transfused human blood to a patient who had hemorrhaged during childbirth. In 1901, Karl Landsteiner, an Austrian physician discovered the first human blood groups, which helped transfusion to become a safer practice.
Do blood transfusions make you feel better?
Ideally, you will start to feel better immediately after receiving the transfusion because your blood is better able to function as it should.
When should you transfuse blood?
The threshold for transfusion of red blood cells should be a hemoglobin level of 7 g per dL (70 g per L) in adults and most children.
If I Have Thalassemia, How Does It Affect My body?
Since your body has fewer red blood cells when you have thalassemia, you may have symptoms of a low blood count, or anemia. When you have anemia, y...
How Is Thalassemia Treated?
The type of treatment a person receives depends on how severe the thalassemia is. The more severe the thalassemia, the less hemoglobin the body has...
How Do Blood Transfusions Affect My body?
People who receive a lot of blood transfusions are at risk for iron overload. Red blood cells contain a lot of iron, and over time, the iron from a...
Standards of Care Guidelines for Thalassemia
PAGE 13 10 Cardiac Dysfunction 10.1 Cardiac evaluation 10.2 Echocardiography standards 10.3 Treatment of established heart failure 10.4 Pulmonary hypertension 10.5 Treatment of pulmonary hypertension
Thalassemia - Diagnosis and treatment - Mayo Clinic
Diagnosis. Most children with moderate to severe thalassemia show signs and symptoms within their first two years of life. If your doctor suspects your child has thalassemia, he or she can confirm a diagnosis with blood tests.
Thalassemia: Symptoms, Causes, Diagnosis, Treatment
Thalassemia is a genetic blood disorder that impacts the ability of the blood to get oxygen to the body’s organs. Learn about the signs, how it’s diagnosed & treated.
Thalassemia - Symptoms and causes - Mayo Clinic
Overview. Thalassemia (thal-uh-SEE-me-uh) is an inherited blood disorder that causes your body to have less hemoglobin than normal. Hemoglobin enables red blood cells to carry oxygen.
Thalassaemia - Treatment - NHS
Thalassaemia usually requires lifelong treatment with blood transfusions and medication. Children and adults with thalassaemia will be supported by a team of different healthcare professionals working together at a specialist thalassaemia centre.
Why do people need blood transfusions?
The purpose of transfusion is twofold: to improve the anemia and to suppress the ineffective erythropoiesis. Chronic transfusions prevent most of the serious growth, skeletal, and neurological complications of thalassemia major. However, once started, the transfusion-related complications become a major source of morbidity. Standards must be developed and maintained to ensure a safe and rational approach to the use of blood transfusions in the management of these rare disorders.
Why do some patients need to be transfused with red blood cells?
Some patients are transfused with irradiated red cells. This process is used to prevent graft-versus-host disease. It is largely unnecessary unless the patient is undergoing a bone marrow transplant or has an underlying immunodeficiency. Cytomegalovirus (CMV) infection is transmitted via transfusion. Leukocyte depletion of a red cell unit prevents its transmission. CMV negative units are usually unnecessary once the unit is leukocyte-depleted.
What is the purpose of chronic transfusions?
Chronic transfusions prevent most of the serious growth, skeletal, and neurological complications of thalassemia major. However, once started, the transfusion-related complications become a major source of morbidity. Standards must be developed and maintained to ensure a safe and rational approach to the use of blood transfusions in ...
What is the normal hemoglobin level for a patient with skeletal changes?
Patients with a hemoglobin level less than 7 g/dL may sometimes require regular transfusions in the presence of growth impairment, marked skeletal changes, or extramedullary hematopoiesis. Baseline laboratory tests prior to regular transfusions.
How long does it take to get blood transfusions stopped?
It may be necessary to initiate a six-month trial of blood transfusions in patients of families whose decision to transfuse is uncertain. After six months , transfusions can be stopped and the patient observed for a brief period of time to give the family and medical team information as to the clinical benefits and psychological impact of the transfusions.
When to start regular blood transfusions?
The decision to start regular transfusions is clear when the initial hemoglobin level is well below 6 g/dL. To assess a child’s need for routine transfusions due to thalassemia, anemia caused by sepsis or viral infection must be ruled out. Assessment may be accomplished by withholding transfusions and monitoring weekly hemoglobin level. If the hemoglobin drops under 7 g/dL on two occasions, two weeks apart, then regular transfusions should be commenced.
Why do we need an extended red cell phenotype?
An extended red cell phenotype must be obtained to reduce the future probability of developing alloantibodies. If a child has already started transfusions, the red cell antigen genotype can be determined by DNA testing, and at the minimum, should include the C, E, and Kell alleles.
How is thalassemia treated?
Hear Robert’s tips on how to successfully transition to adult care for thalassemia.
Why is thalassemia considered immunocompromised?
Because of this, people with thalassemia are said to be “immunocompromised,” which means that some of the body’s defenses against infection aren’t working. When you are immunocompromised, it is easier for you to get infections and you sometimes need extra protection, like flu shots and other vaccines.
What is the best vitamin for thalassemia?
Many times people with thalassemia are prescribed a supplemental B vitamin, known as folic acid, to help treat anemia. Folic acid can help red blood cells develop. Treatment with folic acid is usually done in addition to other therapies.
How to tell if you have thalassemia?
Since your body has fewer red blood cells when you have thalassemia, you may have symptoms of a low blood count, or anemia. When you have anemia, you might feel tired or weak. You might also experience: 1 Dizziness 2 Shortness of breath 3 A fast heart beat 4 Headache 5 Leg cramps 6 Difficulty concentrating 7 Pale skin
Where are blood cells made?
The main place where blood cells are made is the bone marrow, the dark spongy part in the middle of bones. Because your bone marrow may be working harder than normal, it might grow bigger. This causes your bones to expand, and may stretch your bones and make them thinner and more easily broken.
Is there a risk of infection from blood transfusion?
In the United States, the blood supply is screened and monitored for safety, and the risk of getting an infection from a blood transfusion is very low. Nevertheless, there is still a very small risk of getting an infection through a blood transfusion. Learn more about complications of transfusions in thalassemia.
Is it safe to get blood transfusions?
Some infections, like hepatitis, can be carried in blood. In the United States, the blood supply is screened and monitored for safety, and the risk of getting an infection from a blood transfusion is very low. Nevertheless, there is still a very small risk of getting an infection through a blood transfusion. Learn more about complications of transfusions in thalassemia.
When do you need blood transfusions for beta thalassemia?
For a type called beta thalassemia intermedia, you may need transfusions at certain times, like when you're sick or before you have surgery. If you have the most severe form, beta thalassemia major, you'll need regular transfusions. Your doctor will decide whether to start you or your child on blood transfusions based on your symptoms ...
What Happens Before the Transfusion?
The blood you get has to be the right match or you could have a dangerous reaction to it. Blood comes in several types: A, B, AB, or O, which are either positive or negative. Blood types get their names based on which proteins are on the surface of the blood cells.
Why do people get blood transfusions?
Blood transfusions could be a regular thing if you or your child has a blood disorder called beta thalassemia. The disease causes a drop in the amount of red blood cells you have. A transfusion gives you healthy red blood cells from a donor to make up for the ones you don't have.
What are the symptoms of hemoglobin?
Even if your child has higher hemoglobin levels, they may need transfusions if they have symptoms like: 1 An enlarged spleen 2 Tiredness and trouble breathing from anemia 3 Bone fractures 4 Bony growths on the face or other areas 5 Slowed growth
How much risk of getting HIV from blood transfusion?
The risk of getting HIV from a blood transfusion today is less than 1 in 1 million. The risk of hepatitis B is slightly higher, but you'll get a hepatitis B vaccine to protect you before you start transfusions. Allergic reactions. Sometimes the immune system reacts to the transfusion.
What are the complications of beta thalassemia?
Regular transfusions can help prevent beta thalassemia complications like: Weak bones. Enlarged spleen. Slow growth. Heart problems. If you're the parent of a child with beta thalassemia, it's important to find support for the emotions you may feel as you help your child manage the disease.
What happens if you get the wrong blood type?
If you get the wrong type of blood, your immune system -- your body's defense against germs -- could see it as dangerous and attack it. Some immune reactions can be serious. That's why before your transfusion, your medical team will match you with the right blood type.
Why do people need blood transfusions?
The purpose of transfusion is twofold: to improve the anemia and to suppress the ineffective erythropoiesis. Chronic transfusions prevent most of the serious growth, skeletal, and neurological complications of thalassemia major. However, once started, the transfusion-related complications become a major source of morbidity. Standards must be developed and maintained to ensure a safe and rational approach to the use of blood transfusions in the management of these rare disorders.
Why do some patients need to be transfused with red blood cells?
Some patients are transfused with irradiated red cells. This process is used to prevent graft-versus-host disease. It is largely unnecessary unless the patient is undergoing a bone marrow transplant or has an underlying immunodeficiency. Cytomegalovirus (CMV) infection is transmitted via transfusion. Leukocyte depletion of a red cell unit prevents its transmission. CMV negative units are usually unnecessary once the unit is leukocyte-depleted.
Why is splenectomy less common in thalassemia?
The use of splenectomy in thalassemia has declined in recent years. This is partly due to a decreased prevalence of hypersplenism in adequately transfused patients. There is also an increased appreciation of the adverse effects of splenectomy on blood coagulation.
What is the purpose of transfusion therapy?
The aim of transfusion therapy is to permit normal growth and activity level and to prevent skeletal changes associated with marrow hyperplasia. Adequate transfusion therapy will also reduce splenomegaly and hypersplenism and decrease absorption of dietary iron.
What are the risks of thalassemia?
People with thalassemia are at increased risk of thrombosis . Thrombotic events include pulmonary embolism, arterial occlusion, portal thrombosis, and deep vein thrombosis. Approximately 1 to 2 percent of thalassemia major patients and 5 percent of thalassemia intermedia patients experience a serious thrombosis. One of the most common and serious complications is stroke. Recent brain MRI studies suggest that thalassemia patients (particularly those with thalassemia intermedia) are at high risk for subclinical infarction or silent stroke. Splenectomy significantly increases the prevalence of thrombotic events. Inadequate transfusion may increase the risk of thrombosis secondary to increased release of procoagulant red cell particles. Many people recommend that all post-splenectomy patients should receive anti-platelet or anti-thrombosis therapy with aspirin or low dose warfarin.
What is the purpose of chronic transfusions?
Chronic transfusions prevent most of the serious growth, skeletal, and neurological complications of thalassemia major. However, once started, the transfusion-related complications become a major source of morbidity. Standards must be developed and maintained to ensure a safe and rational approach to the use of blood transfusions in ...
How long does it take to get blood transfusions stopped?
It may be necessary to initiate a six-month trial of blood transfusions in patients of families whose decision to transfuse is uncertain. After six months , transfusions can be stopped and the patient observed for a brief period of time to give the family and medical team information as to the clinical benefits and psychological impact of the transfusions.
How Do Blood Transfusions Help?
Since people with TDT cannot make enough hemoglobin on their own, they require regular red blood cell transfusions in order to treat their anemia and keep them alive. Blood from a donor contains working red blood cells that help treat anemia, facilitate normal growth and activity levels, and improve quality of life. The new blood is able to deliver the oxygen (fuel) that their body needs.
How does chelation therapy work?
Chelation therapy involves administering a medicine called an iron chelator that binds to the iron in the blood and then works to remove it through urine and/or bowel movements. Chelation therapy can be administered a number of different ways, including intravenous (IV), subcutaneous pump, or in oral form.
How to determine a transfusion schedule?
Listening to your body and reporting any patterns that you notice (like feeling more tired or less energetic) to your doctor will help you work together to determine a transfusion schedule that works for you. Figuring out the right timing, transfusion amount, and target hemoglobin level for your body may not happen at first, but through a strong partnership with your doctor it can come together over time.
What is TDT treatment?
There are currently two standard treatment options for#N#transfusion-dependent β‑thalassemia (TDT)#N#×#N#×#N#Transfusion-dependent β‑thalassemia (TDT) a form of β-thalassemia that requires regular transfusions of red blood cells#N#: regular blood transfusions and stem cell transplants.
What happens to hemoglobin after TDT?
After people with TDT receive a red blood cell transfusion, their hemoglobin levels may go up, and they usually feel good and energized for a period of time. But once their hemoglobin levels begin to decrease and levels get low, they can start to feel tired and weak, which often means it’s time for another transfusion.
What is the treatment for iron overload?
Your doctor may treat iron overload with special medications. This kind of treatment is called chelation kee-LAY-shun therapy .
Why is it important to take chelation therapy?
It is important that you take your medication for chelation therapy as prescribed by your doctor so that your iron levels are managed appropriately. “I love playing soccer with my friends after school, but some days, I just don’t have the energy. Maybe I can talk to my doctor about how I can be more active.”.
Why do people need blood transfusions?
A blood transfusion also can help if an illness prevents your body from making blood or some of your blood's components correctly. Blood transfusions usually occur without complications. When complications do occur, they're typically mild.
What is blood transfusion?
Overview. A blood transfusion is a routine medical procedure in which donated blood is provided to you through a narrow tube placed within a vein in your arm. This potentially life-saving procedure can help replace blood lost due to surgery or injury. A blood transfusion also can help if an illness prevents your body from making blood or some ...
Why does the immune system attack the transfused red blood cells?
Your immune system attacks the transfused red blood cells because the donor blood type is not a good match. The attacked cells release a substance into your blood that harms your kidneys. Delayed hemolytic reaction. Similar to an acute immune hemolytic reaction, this reaction occurs more slowly.
How long does it take for blood to decrease?
It can take one to four weeks to notice a decrease in red blood cell levels. Graft-versus-host disease. In this condition, transfused white blood cells attack your bone marrow. Usually fatal, it's more likely to affect people with severely weakened immune systems, such as those being treated for leukemia or lymphoma.
Why do blood banks screen donors?
Blood banks screen donors and test donated blood to reduce the risk of transfusion-related infections, so infections, such as HIV or hepatitis B or C, are extremely rare.
How long does it take to get IV blood?
You'll be seated or lying down for the procedure, which usually takes one to four hours.
Is artificial blood available?
Researchers are working on developing artificial blood. So far, no good replacement for human blood is available.
Why do people need blood transfusions?
Beta thalassemia intermedia causes more serious anemia. Some people will need blood transfusions to boost their red blood cell count. Beta thalassemia major is the most severe type.
How often do you need blood transfusions for beta thalassemia?
If you have beta thalassemia major, you'll need blood transfusions every 2 to 4 weeks to raise the number of your red blood cells. Some people with this type of beta thalassemia need transfusions only at certain times, such as when they have an illness or infection.
What age does beta thalassemia start?
Beta thalassemia major is the most severe type. It often starts by age 2 and causes severe anemia and other serious symptoms. Treatment involves regular blood transfusions. If your child has beta thalassemia major or intermedia, make sure to reach out to family and friends for the emotional backing you need.
What is a chelation pill?
Chelation therapy is a treatment that removes the additional iron from your body. You'll get a pill or shot of a medicine like deferasirox (Exjade, Jadenu), deferiprone (Ferriprox), or deferoxamine (Desferal). These drugs bind to iron in your body, which then leaves your body when you pee or have a bowel movement.
How many types of beta thalassemia are there?
There are three types of beta thalassemia, and each has a different treatment.
What organs are affected by thalassemia?
Your spleen filters blood cells. In beta thalassemia, too much iron and too many abnormal red blood cells can collect in that organ and make it swell. An enlarged spleen can cause your hemoglobin levels to drop even lower, plus lower the number of blood cells called platelets that help your blood clot.
What is it called when you have too few red blood cells?
Too few red blood cells is called anemia. Without enough oxygen, you'll feel tired and weak.
How is thalassemia treated?
Hear Robert’s tips on how to successfully transition to adult care for thalassemia.
Why is thalassemia considered immunocompromised?
Because of this, people with thalassemia are said to be “immunocompromised,” which means that some of the body’s defenses against infection aren’t working. When you are immunocompromised, it is easier for you to get infections and you sometimes need extra protection, like flu shots and other vaccines.
What is the best vitamin for thalassemia?
Many times people with thalassemia are prescribed a supplemental B vitamin, known as folic acid, to help treat anemia. Folic acid can help red blood cells develop. Treatment with folic acid is usually done in addition to other therapies.
How to tell if you have thalassemia?
Since your body has fewer red blood cells when you have thalassemia, you may have symptoms of a low blood count, or anemia. When you have anemia, you might feel tired or weak. You might also experience: 1 Dizziness 2 Shortness of breath 3 A fast heart beat 4 Headache 5 Leg cramps 6 Difficulty concentrating 7 Pale skin
Where are blood cells made?
The main place where blood cells are made is the bone marrow, the dark spongy part in the middle of bones. Because your bone marrow may be working harder than normal, it might grow bigger. This causes your bones to expand, and may stretch your bones and make them thinner and more easily broken.
Is there a risk of infection from blood transfusion?
In the United States, the blood supply is screened and monitored for safety, and the risk of getting an infection from a blood transfusion is very low. Nevertheless, there is still a very small risk of getting an infection through a blood transfusion. Learn more about complications of transfusions in thalassemia.
Is it safe to get blood transfusions?
Some infections, like hepatitis, can be carried in blood. In the United States, the blood supply is screened and monitored for safety, and the risk of getting an infection from a blood transfusion is very low. Nevertheless, there is still a very small risk of getting an infection through a blood transfusion. Learn more about complications of transfusions in thalassemia.
