Transfusion therapy (Sickle-cell disease)

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Red blood cells (erythrocytes) from donors contain normal hemoglobin (HbA), and transfusion of normal red blood cells into people with sickle cell disease reduces the percentage of red cells in the circulation containing the abnormal hemoglobin (HbS). [1] Although transfusion of donor red blood cells can ameliorate and even prevent complications of sickle cell disease in certain circumstances, transfusion therapy is not universally beneficial in sickle cell disease. [2]

Contents

Types of transfusion therapy

There are two main types of transfusion, simple red cell transfusion and exchange transfusion.

Simple transfusion

Involves transfusing red blood cells without removing any of the patient’s blood. [3] It is used when the patient's hemoglobin is much lower than normal, for example an aplastic crisis. [4]

Exchange transfusion

Exchange transfusion involves removal of the patient’s blood and replacement with donor red blood cells. [3] It is used to treat life-threatening complications of sickle cell disease such as stroke or acute chest crisis. [4]

There are three main benefits of an exchange transfusion compared to a simple transfusion, these relate primarily to the ability to remove hemoglobin S containing red blood cells: [5]

  1. Higher percentage of normal (donor) hemoglobin (HbA) containing red cells after the transfusion
  2. Larger volumes of donor blood can be given without increasing the hematocrit to levels that excessively increase blood viscosity
  3. Reduced net transfused volume of red blood cells, which reduces iron overload. [3]

However, there are also potential risks associated with an exchange transfusion:

  1. Red cell alloimmunization due to increased donor exposure
  2. Higher costs
  3. Need for specialized equipment
  4. Need for good venous access. [3]

Automated red cell exchange

The exchange is performed using a machine (pheresis). This method rapidly and substantially reduces the concentration of sickle cells within the blood without increasing the overall hematocrit or blood viscosity.[ citation needed ]

Manual red cell exchange

The exchange is performed manually. It refers to manually phlebotomizing a percentage of the patient’s blood prior to or concomitantly with giving a red cell transfusion.[ citation needed ]

Frequency of red cell transfusions

Red cell transfusions can be further classified as episodic or chronic. [3]

Episodic transfusion

Episodic transfusion is used either acutely in response to a complication of sickle cell disease such as acute chest syndrome or to prevent complications prior to surgery. [3]

Chronic transfusion

Chronic transfusion is used when sustained, low levels of HbS are needed to prevent sickle cell-related complications, most commonly stroke in children. [3]

Indications for red blood cell transfusion

Transfusion therapy for sickle-cell disease entails the use of red blood cell transfusions in the management of acute cases of sickle cell disease and as a prophylaxis to prevent complications by decreasing the number of red blood cells (RBC) that can sickle by adding normal red blood cells.[ citation needed ]

Prevention of complications

Stroke

In children prophylactic chronic red blood cell (RBC) transfusion therapy has been shown to be efficacious to a certain extent in reducing the risk of first stroke or silent stroke when transcranial Doppler (TCD) ultrasonography shows abnormal increased cerebral blood flow velocities. In those who have sustained a prior stoke event it also reduces the risk of recurrent stroke and additional silent strokes. [6] [7] [8] There is no evidence for the use of red blood cell transfusion in adults to prevent primary stroke, although it is recommended to prevent secondary stroke. [8] [9] [10] [11]

Surgery

In children and adults red blood cell transfusion to increase the hemoglobin level to 100 g/L has been shown to decrease the risk of sickle cell-related complications. [2] However, this has not been seen in all studies, and has only been demonstrated for African haplotypes of Hemoglobin SS. [12] [13]

Respiratory problems

In children who have been given transfusions to prevent stroke there was also a reduction in the number of children who developed acute chest crises. [13] There is no evidence about whether or not red cell transfusions prevent chronic lung complications. [14]

Pregnancy

There is a paucity of high-quality evidence which has led to conditional recommendations from American Society of Hematology for transfusional support in pregnant patients. Recent evidence suggests that patients with prior pregnancy complications, high hospitalization rates, or preterm deliveries may benefit from scheduled partial exchange transfusions. [15]

Treatment of complications

Aplastic crisis

This should be suspected if there is a significant drop in the hemoglobin level compared to the patient's usual hemoglobin level which is associated with a low level of reticulocytes. This is usually due to infection with erythrovirus B19 (previously known as parvovirus B19). [11] The anemia is usually severe with an average drop in hemoglobin of 40 g/L, and is usually treated with a simple transfusion. [11]

Splenic and hepatic sequestration

Acute splenic and hepatic sequestration associated with severe anemia requires a simple transfusion to raise the hemoglobin. [3] [11]

Acute chest crisis

Red cell transfusions are used to treat patients with acute chest crisis and respiratory compromise. [9] [2] Exchange transfusion is recommended for those patients who have a higher hemoglobin (> 90g/L), those who have not improved after a simple transfusion, or those who have severe respiratory compromise. [11]

Alloimmunisation

Red cell alloimmunisation is common in people with sickle cell disease who receive transfusions in Europe and North America. [4] This is because there are ethnic differences in the frequencies of blood group antigens. [4] Blood donors are usually Caucasian whereas the blood transfusion recipients usually have an African or Afro-Caribbean ancestry. [4] [16] Extended phenotype matching of red blood cells (matching Rh and Kell blood groups as well as ABO) decreases the risk of alloimmunisation, but it still occurs. [4]

Iron overload

Each unit of transfused blood has approximately 250 mg of iron, with each successive transfusion, patients receiving chronic transfusion therapy accumulate iron in various tissues in the body as the body has no way to excrete the excess, this is a cause of increased morbidity and mortality. [17] The effects of iron overload are countered by chelation therapy [18] Guidelines recommend if patients are receiving regular or intermittent transfusions they should be monitored for iron overload. [4]

Related Research Articles

<span class="mw-page-title-main">Anemia</span> Reduced ability of blood to carry oxygen

Anemia is a blood disorder in which the blood has a reduced ability to carry oxygen. This can be due to a lower than normal number of red blood cells, a reduction in the amount of hemoglobin available for oxygen transport, or abnormalities in hemoglobin that impair its function. The name is derived from Ancient Greek ἀν- (an-) 'not' and αἷμα (haima) 'blood'.

<span class="mw-page-title-main">Thalassemia</span> Family of inherited blood disorders

Thalassemias are a group of inherited blood disorders that manifest as the production of reduced or zero quantities of hemoglobin. Symptoms depend on the type of thalassemia and can vary from none to severe, including death. Often there is mild to severe anemia as thalassemia can affect the production of red blood cells and also affect how long the red blood cells live. Symptoms include tiredness, pallor, bone problems, an enlarged spleen, jaundice, pulmonary hypertension, and dark urine. Children's' growth and development may be slower than normal.

<span class="mw-page-title-main">Graft-versus-host disease</span> Medical condition

Graft-versus-host disease (GvHD) is a syndrome, characterized by inflammation in different organs. GvHD is commonly associated with bone marrow transplants and stem cell transplants.

<span class="mw-page-title-main">Gastrointestinal bleeding</span> Bleeding in the gastrointestinal tract

Gastrointestinal bleeding, also called gastrointestinal hemorrhage (GIB), is all forms of bleeding in the gastrointestinal tract, from the mouth to the rectum. When there is significant blood loss over a short time, symptoms may include vomiting red blood, vomiting black blood, bloody stool, or black stool. Small amounts of bleeding over a long time may cause iron-deficiency anemia resulting in feeling tired or heart-related chest pain. Other symptoms may include abdominal pain, shortness of breath, pale skin, or passing out. Sometimes in those with small amounts of bleeding no symptoms may be present.

Autoimmune hemolytic anemia (AIHA) is an autoimmune disorder which occurs when antibodies directed against the person's own red blood cells (RBCs) cause them to burst (lyse), leading to an insufficient number of oxygen-carrying red blood cells in circulation (anemia). The lifetime of the RBCs is reduced from the normal 100–120 days to just a few days in serious cases. The intracellular components of the RBCs are released into the circulating blood and into tissues, leading to some of the characteristic symptoms of this condition. The antibodies are usually directed against high-incidence antigens, therefore they also commonly act on allogenic RBCs. AIHA is a relatively rare condition, with an incidence of 5–10 cases per 1 million persons per year in the warm-antibody type and 0.45 to 1.9 cases per 1 million persons per year in the cold-antibody type. Autoimmune hemolysis might be a precursor of later onset systemic lupus erythematosus.

Alpha-thalassemia is an inherited blood disorder and a form of thalassemia. Thalassemias are a group of inherited blood conditions which result in the impaired production of hemoglobin, the molecule that carries oxygen in the blood. Symptoms depend on the extent to which hemoglobin is deficient, and include anemia, pallor, tiredness, enlargement of the spleen, iron overload, abnormal bone structure, jaundice, and gallstones. In severe cases death ensues, often in infancy, or death of the unborn fetus.

<span class="mw-page-title-main">Platelet transfusion</span> Treatment for bleeding irregularities

Platelet transfusion, is the process of infusing platelet concentrate into the body via vein, to prevent or treat the bleeding in people with either a low platelet count or poor platelet function. Often this occurs in people receiving cancer chemotherapy. Preventive transfusion is often done in those with platelet levels of less than 10 x 109/L. In those who are bleeding transfusion is usually carried out at less than 50 x 109/L. Blood group matching (ABO, RhD) is typically recommended before platelets are given. Unmatched platelets, however, are often used due to the unavailability of matched platelets. They are given by injection into a vein.

<span class="mw-page-title-main">Beta thalassemia</span> Blood disorder

Beta thalassemias are a group of inherited blood disorders. They are forms of thalassemia caused by reduced or absent synthesis of the beta chains of hemoglobin that result in variable outcomes ranging from severe anemia to clinically asymptomatic individuals. Global annual incidence is estimated at one in 100,000. Beta thalassemias occur due to malfunctions in the hemoglobin subunit beta or HBB. The severity of the disease depends on the nature of the mutation.

An exchange transfusion is a blood transfusion in which the patient's blood or components of it are exchanged with other blood or blood products. The patient's blood is removed and replaced by donated blood or blood components. This exchange transfusion can be performed manually or using a machine (apheresis).

<span class="mw-page-title-main">Packed red blood cells</span> Red blood cells separated for blood transfusion

Red blood cell concentrates, also known as red cell concentrates or packed red blood cells, are red blood cells that have been separated for blood transfusion. A red blood cell concentrate typically has a haematocrit of 0.50 – 0.70 L/L and a volume between 250 and 320 mL. Transfusion of red blood cell concentrates is indicated to compensate for a deficit caused by critical bleeding or to correct anaemic conditions, in order to increase the oxygen-carrying capacity and avoid detrimental effects caused by oxygen debt.

The acute chest syndrome is a vaso-occlusive crisis of the pulmonary vasculature commonly seen in people with sickle cell anemia. This condition commonly manifests with a new opacification of the lung(s) on a chest x-ray.

<span class="mw-page-title-main">Sickle cell disease</span> Medical condition

Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders that are typically inherited. The most common type is known as sickle cell anemia. Sickle cell anemia results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to the red blood cells adopting an abnormal sickle-like shape under certain circumstances; with this shape, they are unable to deform as they pass through capillaries, causing blockages. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain in joints, anemia, swelling in the hands and feet, bacterial infections, dizziness and stroke. The probability of severe symptoms, including long-term pain, increases with age. Without treatment, people with SCD rarely reach adulthood but with good healthcare, median life expectancy is between 58 and 66 years. All of the major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can be damaged from the abnormal functions of the sickle cells and their inability to effectively flow through the small blood vessels.

<span class="mw-page-title-main">Patient blood management</span> Set of medical practices

Patient Blood Management (PBM) is a set of medical practices designed to optimise the care of patients who might need a blood transfusion. Patient blood management programs use an organized framework to improve blood health, thus increasing patient safety and quality of life, reducing costs, and improving clinical outcomes. Some strategies to accomplish this include ensuring that anemia is treated prior to a surgical operation, using surgical techniques that limit blood loss, and returning blood lost during surgery to the patient via intraoperative blood salvage.

Congenital hemolytic anemia (CHA) is a diverse group of rare hereditary conditions marked by decreased life expectancy and premature removal of erythrocytes from blood flow. Defects in erythrocyte membrane proteins and red cell enzyme metabolism, as well as changes at the level of erythrocyte precursors, lead to impaired bone marrow erythropoiesis. CHA is distinguished by variable anemia, chronic extravascular hemolysis, decreased erythrocyte life span, splenomegaly, jaundice, biliary lithiasis, and iron overload. Immune-mediated mechanisms may play a role in the pathogenesis of these uncommon diseases, despite the paucity of data regarding the immune system's involvement in CHAs.

<span class="mw-page-title-main">Sickle cell nephropathy</span> Medical condition

Sickle cell nephropathy is a type of kidney disease associated with sickle cell disease which causes kidney complications as a result of sickling of red blood cells in the small blood vessels. The hypertonic and relatively hypoxic environment of the renal medulla, coupled with the slower blood flow in the vasa recta, favors sickling of red blood cells, with resultant local infarction. Functional tubule defects in patients with sickle cell disease are likely the result of partial ischemic injury to the renal tubules.

Anemia is a condition in which blood has a lower-than-normal amount of red blood cells or hemoglobin. Anemia in pregnancy is a decrease in the total red blood cells (RBCs) or hemoglobin in the blood during pregnancy. Anemia is an extremely common condition in pregnancy world-wide, conferring a number of health risks to mother and child. While anemia in pregnancy may be pathologic, in normal pregnancies, the increase in RBC mass is smaller than the increase in plasma volume, leading to a mild decrease in hemoglobin concentration referred to as physiologic anemia. Maternal signs and symptoms are usually non-specific, but can include: fatigue, pallor, dyspnea, palpitations, and dizziness. There are numerous well-known maternal consequences of anemia including: maternal cardiovascular strain, reduced physical and mental performance, reduced peripartum blood reserves, increased risk for peripartum blood product transfusion, and increased risk for maternal mortality.

<span class="mw-page-title-main">Transfusion-dependent anemia</span> Anemia which requires continuous blood transfusion

Transfusion-dependent anemia is a form of anemia characterized by the need for continuous blood transfusion. It is a condition that results from various diseases, and is associated with decreased survival rates. Regular transfusion is required to reduce the symptoms of anemia by increasing functional red blood cells and hemoglobin count. Symptoms may vary based on the severity of the condition and the most common symptom is fatigue.

A granulocyte transfusion is a medical procedure in which granulocytes are infused into a person's blood. Granulocytes are a category of white blood cell that includes neutrophils, eosinophils, and basophils. Granulocyte transfusions were historically used to prevent and treat infections in people with neutropenia, but the practice declined in popularity in the 1980s. Interest in the procedure increased in the 1990s due to the development of more effective methods for harvesting granulocytes and a growing population of people with severe neutropenia from chemotherapy. However, the treatment's efficacy remains poorly understood and its use is controversial.

Sickle cell retinopathy can be defined as retinal changes due to blood vessel damage in the eye of a person with a background of sickle cell disease. It can likely progress to loss of vision in late stages due to vitreous hemorrhage or retinal detachment. Sickle cell disease is a structural red blood cell disorder leading to consequences in multiple systems. It is characterized by chronic red blood cell destruction, vascular injury, and tissue ischemia causing damage to the brain, eyes, heart, lungs, kidneys, spleen, and musculoskeletal system.

Hemolytic jaundice, also known as prehepatic jaundice, is a type of jaundice arising from hemolysis or excessive destruction of red blood cells, when the byproduct bilirubin is not excreted by the hepatic cells quickly enough. Unless the patient is concurrently affected by hepatic dysfunctions or is experiencing hepatocellular damage, the liver does not contribute to this type of jaundice.

References

  1. ↑ Drasar E, Igbineweka N, Vasavda N, Free M, Awogbade M, Allman M, Mijovic A, Thein SL (March 2011). "Blood transfusion usage among adults with sickle cell disease - a single institution experience over ten years". British Journal of Haematology. 152 (6): 766–70. doi: 10.1111/j.1365-2141.2010.08451.x . PMID   21275951. S2CID   44562296.
  2. 1 2 3 Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, et al. (September 2014). "Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members". JAMA. 312 (10): 1033–48. doi:10.1001/jama.2014.10517. PMID   25203083.
  3. 1 2 3 4 5 6 7 8 "Evidence-Based Management of Sickle Cell Disease". 2014. Retrieved 9 February 2016.
  4. 1 2 3 4 5 6 7 Davis BA, Allard S, Qureshi A, Porter JB, Pancham S, Win N, Cho G, Ryan K (January 2017). "Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects". British Journal of Haematology. 176 (2): 179–191. doi: 10.1111/bjh.14346 . PMID   28092109. S2CID   3462324.
  5. ↑ Howard, Jo (2016-12-02). "Sickle cell disease: when and how to transfuse". Hematology. American Society of Hematology. Education Program. 2016 (1): 625–631. doi:10.1182/asheducation-2016.1.625. ISSN   1520-4391. PMC   6142434 . PMID   27913538.
  6. ↑ Gyang E, Yeom K, Hoppe C, Partap S, Jeng M (January 2011). "Effect of chronic red cell transfusion therapy on vasculopathies and silent infarcts in patients with sickle cell disease". American Journal of Hematology. 86 (1): 104–6. doi:10.1002/ajh.21901. PMID   21117059. S2CID   38286439.
  7. ↑ Mirre E, Brousse V, Berteloot L, Lambot-Juhan K, Verlhac S, Boulat C, Dumont MD, Lenoir G, de Montalembert M (March 2010). "Feasibility and efficacy of chronic transfusion for stroke prevention in children with sickle cell disease". European Journal of Haematology. 84 (3): 259–65. doi:10.1111/j.1600-0609.2009.01379.x. PMID   19912310. S2CID   24316310.
  8. 1 2 Estcourt, Lise J.; Kohli, Ruchika; Hopewell, Sally; Trivella, Marialena; Wang, Winfred C. (27 July 2020). "Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease". The Cochrane Database of Systematic Reviews. 2020 (7): CD003146. doi:10.1002/14651858.CD003146.pub4. ISSN   1469-493X. PMC   7388696 . PMID   32716555.
  9. 1 2 "ISBT: 14. Transfusion in Hemoglobinopathies". www.isbtweb.org. Retrieved 2019-01-09.
  10. ↑ Estcourt, Lise J.; Kimber, Catherine; Hopewell, Sally; Trivella, Marialena; Doree, Carolyn; Abboud, Miguel R. (6 April 2020). "Interventions for preventing silent cerebral infarcts in people with sickle cell disease". The Cochrane Database of Systematic Reviews. 2020 (4): CD012389. doi:10.1002/14651858.CD012389.pub3. ISSN   1469-493X. PMC   7134371 . PMID   32250453.
  11. 1 2 3 4 5 Davis BA, Allard S, Qureshi A, Porter JB, Pancham S, Win N, Cho G, Ryan K (January 2017). "Guidelines on red cell transfusion in sickle cell disease Part II: indications for transfusion" (PDF). British Journal of Haematology. 176 (2): 192–209. doi:10.1111/bjh.14383. PMID   27858994. S2CID   3534824.
  12. ↑ Estcourt, Lise J.; Kimber, Catherine; Trivella, Marialena; Doree, Carolyn; Hopewell, Sally (2 July 2020). "Preoperative blood transfusions for sickle cell disease". The Cochrane Database of Systematic Reviews. 2020 (7): CD003149. doi:10.1002/14651858.CD003149.pub4. ISSN   1469-493X. PMC   7389247 . PMID   32614473.
  13. 1 2 Fortin PM, Hopewell S, Estcourt LJ (August 2018). "Red blood cell transfusion to treat or prevent complications in sickle cell disease: an overview of Cochrane reviews". The Cochrane Database of Systematic Reviews. 2018 (8): CD012082. doi:10.1002/14651858.cd012082.pub2. PMC   4826604 . PMID   30067867.
  14. ↑ Estcourt, Lise J.; Hopewell, Sally; Trivella, Marialena; Hambleton, Ian R.; Cho, Gavin (25 October 2019). "Regular long-term red blood cell transfusions for managing chronic chest complications in sickle cell disease". The Cochrane Database of Systematic Reviews. 2019 (10). doi:10.1002/14651858.CD008360.pub5. ISSN   1469-493X. PMC   6814284 . PMID   31684693.
  15. ↑ Ananthaneni, Anil; Jones, Sarah; Ghoweba, Mohamed; Grant, Vishwa; Leethy, Kenna; Benzar, Taras; Master, Samip; Mansour, Richard; Ramadas, Poornima (November 2024). "Impact of scheduled partial exchange transfusions on outcomes in pregnant patients with severe sickle cell disease: a retrospective study". Hematology, Transfusion and Cell Therapy. 46 Suppl 5 (Suppl 5): S109 –S114. doi:10.1016/j.htct.2024.07.001. ISSN   2531-1387. PMC   11670625 . PMID   39322530.
  16. ↑ "Why black, Asian and minority ethnic donors are needed". NHS Blood and Transplant. Retrieved 2019-01-09.
  17. ↑ Harmatz P, Butensky E, Quirolo K, Williams R, Ferrell L, Moyer T, Golden D, Neumayr L, Vichinsky E (July 2000). "Severity of iron overload in patients with sickle cell disease receiving chronic red blood cell transfusion therapy". Blood. 96 (1): 76–9. doi:10.1182/blood.V96.1.76. PMID   10891433.
  18. ↑ Walter PB, Harmatz P, Vichinsky E (2009). "Iron metabolism and iron chelation in sickle cell disease". Acta Haematologica. 122 (2–3): 174–83. doi:10.1159/000243802. PMID   19907155. S2CID   33596378.
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