COVID-19 and Thalassemia: Frequently Asked Questions
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(Version 2.1; last updated January 19, 2022)
Input from Maria Cappellini, MD; Antonio Piga, MD; Janet Kwiatkowski, MD; and Alexis Thompson, MD.
Note: Please review ASH's disclaimer regarding the use of the following information.
Are patients with thalassemia more susceptible to serious COVID-19 disease? Does splenectomy confer a higher risk?
Thalassemia patients, especially young adults/adults, have a chronic condition which may be associated with several co-morbidities linked to the underlying disease as well as complications of chronic transfusions, including heart failure, pulmonary hypertension, and diabetes. Thus, it seems possible that there could be an increased risk of more severe COVID-19 disease in some patients. Nonetheless, outcomes were recently reported from a small cohort of Italian patients followed in the northern part of Italy, where the pandemic has been the most widespread, showing most experienced relatively mild to moderate COVID-19 disease.1 The number of infected thalassemia patients was lower than expected, likely due to earlier and more vigilant self-isolation compared to the general population. An update of Italian data has been recently published (Am J Hematol. 2022;97:E75-E78) showing a fivefold increase in age-standardized lethality due to SARS-CoV-2 infection.
Splenectomy is not known to increase the general risk of viral infection or severe viral disease, but no specific data exists for SARS-CoV-2. Splenectomized patients who develop fever should be evaluated for possible bacterial infection and should receive antibiotics to cover secondary bacterial infections.
Should any changes in transfusion schedules or thresholds be made in the context of the COVID-19 pandemic?
At present there is no evidence that the SARS-CoV-2 virus may be transmitted through donated blood. It is advisable to maintain the individual’s chronic transfusion regimen. Clinics and infusion centers should offer patients the safest possible environment for receiving transfusions, in areas free of COVID-19 patients or those being screened for respiratory symptoms and providing health care personnel protective equipment.
Hematologists and other thalassemia care providers should continue to follow local and national developments related to possible blood shortages related to COVID-19. Clinical sites and blood banks should develop contingency plans for adjusting transfusion regimens and obtaining appropriate donor units for individuals with alloimmunization in the event a significant shortage develops.
Should thalassemia patients continue iron chelation if they are exposed to or have confirmed COVID-19 disease?
No data are available regarding iron chelation and susceptibility to COVID-19 or severity of infection. If a patient is exposed but asymptomatic there is no reason to interrupt iron chelation. If a patient becomes symptomatic, particularly with moderate to severe disease, then interruption of iron chelation is advisable, with ongoing communication between the treating physicians and the hematologist.
Many comorbidities in thalassemia are related to iron overload. Patients should be reminded that adherence to the iron chelation dose and schedule recommended by their thalassemia care provider will reduce organ injury and thalassemia complications.
What about treatment with the recently approved disease-modifying drug luspatercept during the COVID-19 pandemic?
Luspatercept has been approved for adults with transfusion dependent beta thalassemia and has been shown to significantly reduce transfusion burden in this population. No data are available to date on luspatercept and COVID-19. If patients are currently taking luspatercept, there are no theoretical reasons to stop treatment. If the drug has decreased transfusion frequency, then continuing treatment would be beneficial in terms of avoiding prolonged clinic visits for transfusions and decreasing utilization of a limited blood supply.
What are the recommendations for stem cell transplantation or gene therapy for thalassemia during the COVID pandemic?
Due to high risk of infection in hospital settings and the risk of myeloablation, most allogeneic stem cell transplantation and gene therapies were postponed during the initial phases of the pandemic. In localities with a fall in COVID-19 hospitalizations, patients and their physicians can begin to discuss moving forward with planned procedures, particularly in patients with gene therapy cell products already prepared or with limited allogeneic donor availability. There is no evidence that SARS-CoV-2 can be transmitted via hematopoietic stem cell grafts.
- Motta et al, Am J Hematol, 2020 SARS‐CoV‐2 infection in beta thalassemia: preliminary data from the Italian experience. https://doi.org/10.1002/ajh.25840
- Karimi M, et al. Prevalence and Mortality due to Outbreak of Novel Coronavirus Disease (COVID-19) in β-Thalassemias: The Nationwide Iranian Experience. Br J Haematol. 2020 Jun 2.
- Roy NBA, et al. Protecting vulnerable patients with inherited anaemias from unnecessary death during the COVID-19 pandemic. Br J Haematol. 2020 May;189(4):635-639.
For additional information, see:
- Resources for providers to share with their patients