Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2022 Jul;62(7):1446-1451.
doi: 10.1111/trf.16932. Epub 2022 May 30.

Hyperhemolysis in a patient with sickle cell disease and recent SARS-CoV-2 infection, with complex auto- and alloantibody work-up, successfully treated with tocilizumab

Christine Fuja  1 , Vishesh Kothary  2 , Timothy Clifford Carll  1 , Savita Singh  3 , Paul Mansfield  3 , Geoffrey D Wool  1
Affiliations
Case Reports

Hyperhemolysis in a patient with sickle cell disease and recent SARS-CoV-2 infection, with complex auto- and alloantibody work-up, successfully treated with tocilizumab

Christine Fuja et al. Transfusion. 2022 Jul.

Abstract

Background: Hyperhemolysis syndrome (HHS) is a severe delayed hemolytic transfusion reaction seen in sickle cell disease (SCD) patients, characterized by destruction of donor and recipient RBCs. It results in a drop in hemoglobin to below pretransfusion levels and frequently reticulocytopenia.

Case report: We report a case of a man in his thirties with SCD with a recent hospitalization 2 weeks prior for COVID-19. His red cell antibody history included anti-Fy(a) and warm autoantibody. At that time, he was given 2 units of RBC and discharged with a hemoglobin of 10.2 g/dl. He returned to the hospital approximately 1.5 weeks later with hemoglobin 6.0 g/dl and symptoms concerning for acute chest syndrome. Pretransfusion testing now showed 4+ pan-agglutinin in both gel-based and tube-based testing. Alloadsorption identified an anti-N and a strong cold agglutinin. Three least incompatible units were transfused to this patient over several days, with evidence of hemolysis. Further reference lab work revealed anti-Fya , anti-Fyb , anti-Lea , anti-Leb , and an anti-KN system antibody. The patient's hemoglobin nadired at 4.4 g/dl. The patient was treated with a single dose of tocilizumab, his hemoglobin stabilized, and he was discharged.

Discussion: We present a case of HHS proximate to recent SARS-CoV-2 infection with multiple allo and autoantibodies identified. Information on the relationship between SARS-CoV-2 infection and HHS is limited; however, it is possible that inflammation related to COVID-19 could predispose to HHS. Tocilizumab is an approved treatment for COVID-19. Additionally, tocilizumab appears to be a promising treatment option for patients with HHS.

Keywords: AIHA/drug-induced IHA; immunohematology (RBC serology, blood groups); transfusion complications-non-infectious.

PubMed Disclaimer

Conflict of interest statement

CF, VK, TCC, SS, and PM have disclosed no conflicts of interest. GDW has disclosed financial relationships with Diagnostica Stago.

Figures

FIGURE 1
FIGURE 1
Icteric plasma from (left) pre‐ and (right) post‐RBC #3 (Day +17) transfusion samples. Degree of icterus was unchanged. [Color figure can be viewed at wileyonlinelibrary.com]
FIGURE 2
FIGURE 2
Trends of hemoglobin, lactate dehydrogenase, and absolute reticulocyte count during admissions
See this image and copyright information in PMC

References

    1. Panepinto JA, Brandow A, Mucalo L, Yusuf F, Singh A, Taylor B, et al. Coronavirus disease among persons with sickle cell disease, United States, March 20‐May 21, 2020. Emerg Infect Dis. 2020;26(10):2473–6. - PMC - PubMed
    1. Hoogenboom WS, Alamuri TT, McMahon DM, Balanchivadze N, Dabak V, Mitchell WB, et al. Clinical outcomes of COVID‐19 in patients with sickle cell disease and sickle cell trait: a critical appraisal of the literature. Blood Rev. 2021;53:100911. - PMC - PubMed
    1. Allison D, Campbell‐Lee S, Crane J, Vidanovic V, Webb S, Fraidenburg D, et al. Red blood cell exchange to avoid intubating a COVID‐19 positive patient with sickle cell disease? J Clin Apher. 2020;35(4):378–81. - PMC - PubMed
    1. Merrill SA, Brodsky RA, Lanzkron SM, Naik R. A case‐control analysis of hyperhemolysis syndrome in adults and laboratory correlates of complement involvement. Transfusion. 2019;59(10):3129–39. - PubMed
    1. King KE, Shirey RS, Lankiewicz MW, Young‐Ramsaran J, Ness PM. Delayed hemolytic transfusion reactions in sickle cell disease: simultaneous destruction of recipients' red cells. Transfusion. 1997;37(4):376–81. - PubMed

Publication types