[go: up one dir, main page]
Skip to main content
Springer Nature Link
Log in

Severe Hyperhaemolysis Syndrome in a Post-partum Woman with Haemoglobin E Disease: A Case Report

  • Case Report
  • Published:
SN Comprehensive Clinical Medicine Aims and scope Submit manuscript

Abstract

Introduction

Hyperhaemolysis syndrome (HHS) is a rare, delayed type of haemolytic transfusion reaction. Early diagnosis and appropriate treatment are required to avoid adverse consequences and increased mortality. HHS has not been reported before to be associated with haemoglobin E (HbE) disease.

Case Presentation

A 37-year-old lady with a pre-existing diagnosis of HbE disease in her second pregnancy was admitted with established pre-term labour. Following a vaginal delivery, her haemoglobin levels (Hb) continued to decline from 9.4 to 5.4 g/dL despite repeated transfusions. Her investigations showed evidence of haemolysis, positive antibody screening, and DAT with weak C3d. Phenotyping was conducted on a post-transfusion sample, revealing mixed reactions with Jkb antisera. Consequently, a delayed haemolytic transfusion reaction due to Jkb antibodies was suspected, for which Jkb antigen-negative cross-match-compatible blood was transfused. However, her haemoglobin further dropped beyond the pre-transfusion value. Repeated lab investigations revealed evidence of intravascular haemolysis and poor reticulocyte response, leading to the diagnosis of hyperhaemolysis. Consequently, red-cell transfusions were withheld, with a decision to transfuse only in life-threatening situations. She was initiated on intravenous immunoglobulin and high-dose methylprednisolone, followed by oral prednisolone. Haematinics and erythropoietin were added to support erythropoiesis. The Hb began to rise to 9.8 g/dL after 3 weeks of immunoglobulin and steroids.

Conclusion

This case highlights the challenges in managing HHS in a pregnant woman with HbE disease. The treatment of HHS needs a multidisciplinary team approach with continuous monitoring and commencing treatment urgently to achieve a favourable outcome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+
from €37.37 /Month
  • Starting from 10 chapters or articles per month
  • Access and download chapters and articles from more than 300k books and 2,500 journals
  • Cancel anytime
View plans

Buy Now

Price includes VAT (France)

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

Explore related subjects

Discover the latest articles, books and news in related subjects, suggested using machine learning.

Data Availability

No datasets were generated or analysed during the current study.

Materials Availability

Data can be acquired from the corresponding author on request.

Code Availability

Not applicable.

Abbreviations

DAT:

direct antiglobulin test

DTHR:

delayed type haemolytic reaction

EF:

ejection fraction

Hb:

haemoglobin

HbE:

haemoglobin E

HHS:

hyperhaemolysis syndrome

IVH:

intravascular haemolysis

IVIG:

intravenous immunoglobulin

LDH:

lactate dehydrogenase

RCC:

red cell concentrate

RPI:

reticulocyte-production-index

References

  1. Win N. Hyperhemolysis syndrome in sickle cell disease. Expert Rev Hematol. 2009;2(2):111–5.

    Article  PubMed  Google Scholar 

  2. Danaee A, Inusa B, Howard J, Robinson S. Hyperhemolysis in patients with hemoglobinopathies: a single-center experience and review of the literature. Transfus Med Rev. 2015;29(4):220–30.

    Article  PubMed  Google Scholar 

  3. Eberly LA, Osman D, Collins NP. Hyperhemolysis syndrome without underlying hematologic disease. Case Rep Hematol. 2015;2015:1–3.

    Google Scholar 

  4. Darabi K, Dzik S. Hyperhemolysis syndrome in anemia of chronic disease. Transfusion (Paris) 2005;45(12):1930–3.

    Article  Google Scholar 

  5. Petz LD, Calhoun L, Shulman IA, Johnson C, Herron RM. The sickle cell hemolytic transfusion reaction syndrome. Transfusion (Paris) 1997;37(4):382–92.

    Article  CAS  Google Scholar 

  6. Garratty G. What do we mean by “hyperhaemolysis” and what is the cause? Transfus Med 2012;22(2):77–9.

    Article  CAS  PubMed  Google Scholar 

  7. Lee LE, Beeler BW, Henderson AT, Graham BC, Osswald MB, Win N, et al. Targeting macrophage activation in hyperhemolysis syndrome with novel use of tocilizumab. Blood 2018;132(Supplement 1):4933–4933.

    Article  Google Scholar 

  8. Meenan J, Hall R, Badle S, Chatterjee B, Win N, Tsitsikas DA. Tocilizumab in the management of posttransfusion hyperhemolysis syndrome in sickle cell disease: the experience so far. Transfusion (Paris) 2022;62(3):546–50.

    Article  CAS  Google Scholar 

  9. Gouveia ME, Soares NB, Santoro MS, de Azevedo FCM. Hyperhemolysis syndrome in a patient with sickle cell anemia: case report. Rev Bras Hematol Hemoter. 2015;37(4):266–8.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Win N, Yeghen T, Needs M, Chen FE, Okpala I. Use of intravenous immunoglobulin and intravenous methylprednisolone in hyperhaemolysis syndrome in sickle cell disease. Hematology. 2004;9(5–6):433–6

    Article  CAS  PubMed  Google Scholar 

  11. Menakuru SR, Priscu A, Dhillon V, Salih A. Acute hyperhemolysis syndrome in a patient with known sickle cell anemia refractory to steroids and IVIG treated with tocilizumab and erythropoietin: a case report and review of literature. Hematol Rep 2022;14(3):235–9

    Article  PubMed  PubMed Central  Google Scholar 

  12. Shaulov A, Rund D, Filon D, Nachmias B, Khalili A, Manny N, et al. Successful treatment with plasma exchange in life-threatening hyperhemolytic syndrome unrelated to sickle cell disease. Transfusion (Paris) 2023;63(5):1100–6

    Article  Google Scholar 

  13. El Afifi AM, Saeed AM, Fekry GH, Mostafa MA, Elmetwally RA, Hamed IM, et al. Successful management of severe hyperhaemolysis with combined tocilizumab and rituximab in non-transfusion-dependent thalassaemia: a case report. Transfus Med Hemother. 2023;50(1):66–70.

    Article  PubMed  Google Scholar 

  14. Kirui LC, Scully M, Mcqueen N, Porter J, Eleftheriou P. Use of eculizumab for the treatment of hyperhaemolysis in pregnancy in sickle cell disease: a case report. Blood 2018;132(Supplement 1):4922–4922.

    Article  Google Scholar 

  15. Noizat-Pirenne F, Habibi A, Mekontso-Dessap A, Razazi K, Chadebech P, Mahevas M, et al. The use of rituximab to prevent severe delayed haemolytic transfusion reaction in immunized patients with sickle cell disease. Vox Sang 2015;108(3):262–7.

    Article  CAS  PubMed  Google Scholar 

Download references

Funding

None.

Author information

Authors and Affiliations

  1. Department of Pathology, Faculty of Medical Sciences, University of Sri Jayewardenepura, Colombo, Sri Lanka

    N. R. Muthumala, C. Kulathilake, K. H. B. P. Fernandopulle & C. S. Moonesinghe

  2. Transfusion Medicine Unit, Colombo South Teaching Hospital, Colombo, Sri Lanka

    N. N. Manawasinghe & K. Gonsalkorale

  3. Department of Medicine, Faculty of Medical Sciences, University of Sri Jayewardenepura, Colombo, Sri Lanka

    F. H. D. S. Silva

  4. University Medical Unit, Colombo South Teaching Hospital, Colombo, Sri Lanka

    F. H. D. S. Silva

Authors
  1. N. R. Muthumala
    View author publications

    Search author on:PubMed Google Scholar

  2. C. Kulathilake
    View author publications

    Search author on:PubMed Google Scholar

  3. N. N. Manawasinghe
    View author publications

    Search author on:PubMed Google Scholar

  4. K. H. B. P. Fernandopulle
    View author publications

    Search author on:PubMed Google Scholar

  5. C. S. Moonesinghe
    View author publications

    Search author on:PubMed Google Scholar

  6. K. Gonsalkorale
    View author publications

    Search author on:PubMed Google Scholar

  7. F. H. D. S. Silva
    View author publications

    Search author on:PubMed Google Scholar

Contributions

MNR, MNN, KC, FKHBP, MCS, GK and SFHDS were involved in the multidisciplinary management of this patient. MNR drafted the manuscript under primary supervision and review of SFHDS. All other authors reviewed the manuscript and contributed to corrections.

Corresponding author

Correspondence to F. H. D. S. Silva.

Ethics declarations

Ethical Approval

Our institutions do not require ethics review committee approval for the publication of case reports.

Consent to Participate

Informed written consent was obtained from the patient for the publication of this case report.

Consent for Publication

Informed written consent was obtained from the patient for the publication of this case report.

Competing interests

The authors declare no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Below is the link to the electronic supplementary material.

ESM 1

(758 KB PDF)

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Muthumala, N.R., Kulathilake, C., Manawasinghe, N.N. et al. Severe Hyperhaemolysis Syndrome in a Post-partum Woman with Haemoglobin E Disease: A Case Report. SN Compr. Clin. Med. 7, 317 (2025). https://doi.org/10.1007/s42399-025-02097-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s42399-025-02097-7

Keywords