Senior Consultant, Bone Marrow Transplant & Haemato-Oncology, BLK-Max Super Speciality Hospital, New Delhi
Part 9 of 11 in Diagnosis and Management of Sickle Cell Disease
Blood Transfusion and Iron Overload Management in Sickle Cell Disease
November 24, 2024
Red cell transfusion in sickle cell disease can be simple or exchange, and a tertiary centre managing sickle cell disease will do a substantial number of red cell exchanges, particularly before surgery, to bring the haemoglobin S percentage down for a safer operation.
Why Haemoglobin Targets Matter
A key rule with transfusion in sickle cell disease is to keep the post-transfusion haemoglobin at or below 10 g, because raising it higher increases blood viscosity, which in turn worsens sickling and increases painful episodes. The complications of transfusion itself include iron overload, alloimmunisation, which is a bigger problem in sickle cell disease than in thalassaemia, and delayed haemolytic transfusion reactions. Pre-operative transfusion is used specifically for complicated, high-risk surgery, to bring haemoglobin S down below 30% so the operation can proceed safely.
Monitoring and Treating Iron Overload
Patients who need chronic transfusions build up significant iron overload over time, so ferritin levels should be checked every three months. If ferritin rises above 1,000, a liver MRI is recommended to measure liver iron content directly; if that liver iron content is more than 7 mg of iron per gram of dry liver weight, iron chelation should be started, and if it rises above 15, a T2-star cardiac MRI should be done to check for cardiac iron overload, along with an assessment of cardiac function.
This article is based on a Jivo Masterclass session conducted by Dr. Divya Doval, Senior Consultant, Bone Marrow Transplant & Haemato-Oncology, BLK-Max Super Speciality Hospital, New Delhi. The article has been summarised with the assistance of an AI tool from the original masterclass recording. Watch the full Masterclass recording
This guide is based on a live Jivo Masterclass: Dr. Divya Doval taught doctors across Africa on November 24, 2024.
FROM THE LIVE Q&A
Jivo Doctor Partner (name unclear from transcript)
What is the management of vaso-occlusive crisis in sickle cell disease? Is there a curative treatment for it?
Dr. Divya Doval
There is no definitive curative treatment for vaso-occlusive crisis. We can only support with oxygen, fluids and painkillers, and if the pain is excessive and not controlled, a blood transfusion.
Frequently Asked Questions
Under what age is stem cell transplant effective?▼
There is no age limit. We have transplanted adults as well. The risk simply increases with age and the outcomes are not as good, but otherwise transplant can be done at any age if the organ systems are fine. With age, the risk of stroke and of the liver and kidneys being affected increases, which then reduces eligibility for transplant.
What is the overall cost of bone marrow transplant at your facility?▼
If it's a matched sibling donor, an HLA-matched sibling, it is somewhere between 20,000 and 24,000 US dollars. If a matched sibling is not present and it is a half-match transplant, the price goes up to around 35,000.
What is the role of urine alkalisation in the management of vaso-occlusive crisis?▼
Alkalinisation of the urine prevents acidosis and infections, and once the urine is alkaline, crystallisation is prevented as well. Overall, it helps with the pain.
What is the management of sickle cell disease in pregnancy?▼
Once a patient is pregnant, hydroxyurea cannot be given for the first three months, which can be challenging, so we make sure the patient is well hydrated and stays warm, and we try to prevent all sorts of crisis through the pregnancy. In the second and third trimester, one can start hydroxyurea if required, and transfusions can be done if needed. Morbidity and mortality are high, and every patient should be counselled before starting a family that the risks for both mother and baby are high, since a crisis during labour or childbirth can be quite difficult to manage.
What is the symptomatic treatment for chronic jaundice in sickle cell disease, especially in children who have never had a blood transfusion?▼
This jaundice happens because the red blood cells are breaking down. It is chronic, and these red cells will keep breaking, so a lot of patients will have a high bilirubin, but there isn't a specific treatment for the jaundice itself. We can reduce the underlying haemolysis by starting hydroxyurea, but the jaundice itself does not otherwise affect most patients. If there is chronic jaundice, we do need to find the cause by checking for hepatitis B and C and other infective causes, to make sure it isn't due to something else.
In This Series: Diagnosis and Management of Sickle Cell Disease
- 1.Diagnosis and Management of Sickle Cell Disease
- 2.Sickle Cell Disease Explained: Inheritance, Haemoglobin S and Why Cells Sickle
- 3.Diagnosing Sickle Cell Disease: From Newborn Screening to Confirmatory Testing
- 4.Pain Crisis and Acute Chest Syndrome: The Two Most Common Sickle Cell Emergencies
- 5.Stroke Risk in Sickle Cell Disease: Screening and Preventing a Devastating Complication
- 6.How Sickle Cell Disease Damages the Spleen, Liver, Kidneys and Eyes
- 7.Priapism, Infections and Aplastic Crisis in Sickle Cell Disease
- 8.Hydroxyurea and Newer Drug Therapies for Sickle Cell Disease
- 9.Blood Transfusion and Iron Overload Management in Sickle Cell Disease
- 10.Bone Marrow Transplant and Gene Therapy: Curing Sickle Cell Disease
- 11.Sickle Cell Disease and Pregnancy: Managing a High-Risk Combination