Hyperhemolytic Syndrome complicating a Delayed Hemolytic Transfusion Reaction due to anti-P1 alloimmunization, in a pregnant woman with HbO-Arab?-thalassemia

Main Article Content

Zoe Bezirgiannidou
Anna Christoforidou
Eftychia Kontekaki
Athanasios Anastasiadis
Spyros Papamichos
Helen Menexidou
Dimitrios Margaritis
George Martinis
Elpidoforos Mandadakis


Hyperhemolysis, anti-P1, cold autoantibody, HbO-Arab/β-thalassemia



Background: Hyperhemolytic Syndrome or Hyperhemolytic Transfusion Reaction (HHTR), a life-threatening subset of Delayed Hemolytic Transfusion Reaction (DHTR) is characterized by destruction of both transfused and autologous erythrocytes evidenced by a fall in post transfusion hemoglobin below the pre-transfusion level.

Case report: We describe a case of DHTR due to anti-P1 alloimmunization manifesting with hyperhemolysis in a 30-year-old Greek Pomak woman with thalassemia intermedia (HbO-Arab/?-thalassemia), during the11th week of her first gestation. She was successfully managed with avoidance of further transfusions and administration of IVIG and corticosteroids.

Conclusion: A high index of suspicion for HHTR is of vital importance among clinicians especially since optimal methods for its prevention and treatment remain yet to be defined. Early recognition of HHTR leading to prompt cessation of additional transfusions and initiation of immunosuppressive treatment can be life-saving, especially in clinical settings where limited therapeutic options are available, such as in pregnancy.


Download data is not yet available.

Abstract 2593
PDF Downloads 567
HTML Downloads 1057
Figure 1 Downloads 0


1. Chadebech P, Habibi A, Nzouakou R, Bachir D, Meunier-Costes N, Bonin P, Rodet M, Chami B, Galacteros F, Bierling P, Noizat-Pirenne F: Delayed hemolytic transfusion reaction in sickle cell disease patients: evidence of an emerging syndrome with suicidal red blood cell death. Transfusion. 2009; 49:1785-1792.

2. Win N: Hyperhemolysis syndrome in sickle cell disease. Expert Rev Hematol. 2009; 2:111-115.

3. Santos B, Portugal R, Nogueira C, Loureiro M: Hyperhemolysis syndrome in patients with sickle cell anemia: report of three cases. Transfusion. 2015; 55:1394-1398.

4. Hannema SE, Brand A, van Meurs A, Smiers FJ: Delayed hemolytic transfusion reaction with hyperhemolysis after first red blood cell transfusion in child with beta-thalassemia: challenges in treatment. Transfusion. 2010; 50:429-432.

5. Morawakage LR, Perera BJ, Dias PD, Wijewardana SK: Hyperhemolysis in a patient with beta-thalassemia major. Asian J Transfus Sci. 2009; 3:26-27.

6. Grainger JD, Makar Y, McManus A, Wynn R: Refractory hyperhaemolysis in a patient with beta-thalassaemia major. Transfus Med. 2001; 11:55-57.

7. Gupta S, Fenves A, Nance ST, Sykes DB, Dzik WS: Hyperhemolysis syndrome in a patient without a hemoglobinopathy, unresponsive to treatment with eculizumab. Transfusion. 2015; 55:623-628.

8. Azarkeivan A, Ahmadi MH, Zolfaghari S, Shaiegan M, Ferdowsi S, Rezaei N, Lotfi P: RBC alloimmunization and double alloantibodies in thalassemic patients. Hematology. 2015; 20:223-227.

9. Thakral B, Bhattacharya P, Agnihotri N, Sharma RR, Marwaha N, Gopalan S: Acute hemolytic transfusion reaction by anti-P1 antibody in pregnancy. Am J Hematol. 2005; 78:163-164.

10. Arndt PA, Garratty G, Marfoe RA, Zeger GD: An acute hemolytic transfusion reaction caused by an anti-P1 that reacted at 37 degrees C. Transfusion. 1998; 38:373-377.

11. Pohl B, Nicodemus P, Cronin-Ladd C: Delayed hemolytic transfusion reaction caused by anti-P1. Transfusion. 1981; 21:758-759.

12. Antibody Detection,Antibody Identification, and Serologic Compatibility Testing. 15 edn. Bethesda, Maryland, USA, AABB. 2005;764-765.

13. 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; 9:433-436.

14. Papadopoulos V, Dermitzakis E, Konstantinidou D, Petridis D, Xanthopoulidis G, Loukopoulos D: HbO-Arab mutation originated in the Pomak population of Greek Thrace. Haematologica. 2005; 90:255-257.

15. Garratty G: The James Blundell Award Lecture 2007: do we really understand immune red cell destruction? Transfus Med. 2008; 18:321-334.

16. Petz LD: Bystander immune cytolysis. Transfus Med Rev. 2006; 20:110-140.

17. Bartolmas T, Yurek S, Balola AH, Mayer B, Salama A: Evidence Suggesting Complement Activation and Haemolysis at Core Temperature in Patients with Cold Autoimmune Haemolytic Anaemia. Transfus Med Hemother. 2015; 42:328-332.

18. Vanderlugt CL, Begolka WS, Neville KL, Katz-Levy Y, Howard LM, Eagar TN, Bluestone JA, Miller SD: The functional significance of epitope spreading and its regulation by co-stimulatory molecules. Immunol Rev. 1998; 164:63-72.

19. Proudfit CL, Atta E, Doyle NM: Hemolytic transfusion reaction after preoperative prophylactic blood transfusion for sickle cell disease in pregnancy. Obstet Gynecol. 2007; 110:471-474.

20. Mechery J, Abidogun K, Crosfill F, Jip J: Hyperhemolysis syndrome complicating pregnancy in homozygous deltabeta-thalassemia. Hemoglobin. 2012; 36:183-185.