Delayed hemolytic transfusion reactions (DHTR) are potentially life-threatening complications observed in patients with sickle cell disease. We review the clinical features, pathophysiology, laboratory evaluation, and management of this complication.
Why are sickle cell anemia patients at increased risk of death?
Sickle cell patients, because of their intolerance of hypovolemia and proclivity to endothelial cell activation, are at increased risk to die from this viral infection (101, 102). Parasites
How does sickle cell anemia affect the eyes?
Adults with sickle cell anemia often tend to be smaller than average. Sickle cells can plug up the tiny blood vessels that supply your eyes, damaging the retina, the light-sensitive layer of tissue at the back of the inner eye, and potentially causing blindness.
What are the possible complications of transfusion in sickle cell disease (SCD)?
Although transfusion is regarded as an important therapy to avert serious SCD complications, it is not benign, and repeated transfusions can result in complications such as transfusion-related iron overload, alloimmunization, and delayed hemolytic transfusion reactions, which can result in hyperhemolysis syndrome and death.
What happens to B-cell lymphocytes in sickle cell disease?
B- and T-cell lymphocyte function are impaired in SCD, resulting in inadequate memory B-cell function and T-cell–independent production of natural anti-polysaccharide antibodies (25, 40, 41). The IgM antibody response to the influenza vaccine is also diminished in SCD patients (41).
What complication is associated with sickle cell anemia?
Sickle cell anemia can lead to a host of complications, including: Stroke. Sickle cells can block blood flow to an area of the brain. Signs of stroke include seizures, weakness or numbness of the arms and legs, sudden speech difficulties, and loss of consciousness.
Is sickle cell anemia life-threatening?
Crises are a result of sickle cells pooling in the spleen. This can cause a sudden drop in hemoglobin and can be life-threatening if not treated promptly.
Which medication is contraindicated in patients with sickle cell disease SCD )?
Morphine is considered the drug of choice for the treatment of acute sickle cell pain (Table 2), whereas meperidine (Demerol, Sanofi-Synthelabo) should be avoided because of the increased risk of seizures in patients with renal dysfunction, which can occur in patients with SCD.
What are the major treatments for sickle cell anemia?
TreatmentHydroxyurea (Droxia, Hydrea, Siklos). Daily hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations. ... L-glutamine oral powder (Endari). ... Crizanlizumab (Adakveo). ... Voxelotor (Oxbryta). ... Pain-relieving medications.
What is the most likely cause of death associated with sickle cell disease?
Main Findings From This Study From 2015–2017, SCD-related deaths were most often related to chronic heart conditions such as high blood pressure (27%). Acute cardiac events, such as heart attacks, and infections were also common causes of death (24% and 22%, respectively).
How does sickle cell disease cause sepsis?
Answer. Patients with sickle cell disease (SCD) are considered functionally asplenic. Asplenic patients are at exceptional high risk of sepsis due to encapsulated organisms, such as Streptococcus pneumonia. Because of this, patients with SCD are often hospitalized for observation and empiric treatment.
What is a known and serious adverse reaction for a sickle cell patient taking hydroxyurea?
Pain crisis — pain in your chest, stomach, or bones. This happens when sickle cells block your blood vessels. Acute chest syndrome — a lung problem that happens when sickle cells block the blood vessels in your lungs. This can be life threatening and you will need to go to the hospital.
What is contraindicated in sickle cell anemia?
Patients should avoid the following: Alcohol. Nonprescribed prescription drugs. Cigarettes, marijuana, and cocaine.
Are NSAIDs contraindicated in sickle cell?
Due to various side effects associated with non-aspirin NSAIDs and aspirin, there are no risk-free anti-inflammatory drugs. Both non-aspirin NSAIDs and aspirin should be used cautiously in sickle cell patients, especially those with renal, GI, and cardiovascular risk factors.
What are the side effects of hydroxyurea?
Hydroxyurea may cause side effects. Tell your doctor if any of these symptoms are severe or do not go away:nausea.vomiting.diarrhea.loss of appetite.weight gain.sores in the mouth and throat.constipation.rash.More items...•
Why is aspirin used as a treatment for sickle cell disease?
We hypothesize that daily, low-dose aspirin therapy will safely diminish the incidence and progression of cognitive deficits as well as the predisposition to overt and silent stroke in children with homozygous sickle cell disease (Hgb SS) or hemoglobin S Beta Zero Thalassemia (Hgb SB-0 Thal).
Why do sickle cells cause anemia?
People with sickle cell anemia have a higher-than-normal risk for infection for several reasons, including: 1 The sickle gene itself makes people more susceptible to infection. 2 The spleen, which normally helps to fight infection, gets damaged by sickle red blood cells and does not work normally.
How long does it take for sickle cell anemia to break down?
People with sickle cell anemia often have a high bilirubin level because sickled red blood cells are broken down after 10 to 20 days — versus 120 days for normal red blood cells.
What happens if you have sickle cells in your penis?
Priapism. If sickle cells block the blood vessels in the penis, the result can be priapism — a painful, long-lasting erection. Priapism can cause serious damage to the penis and result in impotence. Men experiencing priapism should seek medical attention immediately.
What makes a person more susceptible to infection?
The sickle gene itself makes people more susceptible to infection. The spleen, which normally helps to fight infection, gets damaged by sickle red blood cells and does not work normally. Any infection in a child with sickle cell anemia is considered an emergency.
What is the percentage of strokes in children with sickle cell anemia?
Changes in the ability to think or remember. Personality changes. According to the Internet Stroke Center, stroke occurs in 17 to 24 percent of children with sickle cell anemia, most often between the ages of 3 and 10.
What is splenic sequestration?
Splenic Sequestration. Splenic sequestration is an acute condition in which large amounts of blood build up in the spleen, causing the spleen to enlarge, sometimes enormously. It is seen most commonly in children and is potentially life threatening.
Can sickle cell anemia cause delayed puberty?
Delayed Growth and Puberty. Delayed growth and delayed puberty are common among children with sick le cell anemia. Adults with sickle cell anemia often tend to be smaller than average.