Hyperhemolytic Syndrome Complicating a Delayed Hemolytic Transfusion Reaction due to anti-P1 Alloimmunization, in a Pregnant Woman with HbO-Arab/β-Thalassemia

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Abstract

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/β-thalassemia), during the11 ^th 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.

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Most cited references 26

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Delayed hemolytic transfusion reaction/hyperhemolysis syndrome in children with sickle cell disease.

Stacey L. Gottschall, Diane M Baylerian, Julie An M Talano … (2003)

Alloimmunization in patients with sickle cell disease (SCD) has a reported incidence of 5% to 36%. One complication of alloimmunization is delayed hemolytic transfusion reaction/hyperhemolysis (DHTR/H) syndrome, which has a reported incidence of 11%. In patients with SCD, clinical findings in DHTR/H syndrome occur approximately 1 week after the red blood cell (RBC) transfusion and include the onset of increased hemolysis associated with pain and profound anemia. The hemoglobin (Hb) often drops below pretransfusion levels. In many reported adult cases, the direct antiglobulin test (DAT) remains negative and no new alloantibody is detected as the cause for these transfusion reactions. To date, few pediatric cases have been reported with this phenomenon. The objective of this study was to describe the clinical and laboratory findings of a case series in children who had SCD and experienced a DHTR/H syndrome at our institution. An 11-year retrospective chart review of patients with discharge diagnosis of SCD and transfusion reaction was performed. DHTR/H syndrome was defined as the abrupt onset of signs and symptoms of accelerated hemolysis evidenced by an unexplained fall in Hb, elevated lactic dehydrogenase, elevated bilirubin above baseline, and hemoglobinuria, all occurring between 4 and 10 days after an RBC transfusion. Patient characteristics, time from transfusion, symptoms, reported DAT, new autoantibody or alloantibody formation, laboratory abnormalities, and complications were recorded. Patients with acute transfusion reactions were excluded. We encountered 7 patients who developed 9 episodes of DHTR/H syndrome occurring 6 to 10 days after RBC transfusion. Each presented with fever and hemoglobinuria. All but 1 patient experienced pain initially ascribed to vaso-occlusive crisis. The DAT was positive in only 2 of the 9 episodes. The presenting Hb was lower than pretransfusion levels in 8 of the 9 events. Severe complications were observed after the onset of DHTR/H: acute chest syndrome, n = 3; pancreatitis, n = 1; congestive heart failure, n = 1; and acute renal failure, n = 1. DHTR/H syndrome occurs in pediatric SCD patients, typically 1 week posttransfusion, and presents with back, leg, or abdominal pain; fever; and hemoglobinuria that may mimic pain crisis. Hb is often lower than it was at the time of original transfusion, suggesting the hemolysis of the patient's own RBCs in addition to hemolysis of the transfused RBCs; a negative DAT and reticulocytopenia are often present. Severe complications including acute chest syndrome, congestive heart failure, pancreatitis, and acute renal failure were associated with DHTR/H syndrome in our patients. DHTR/H in the pediatric sickle cell population is a serious and potentially life-threatening complication of RBC transfusion. It is important to avoid additional transfusions in these patients, if possible, because these may exacerbate the hemolysis and worsen the degree of anemia. DHTR/H syndrome must be included in the differential of a patient who has SCD and vaso-occlusive crisis who has recently had a transfusion.

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The association of anti-P and early abortion.

R Shirey, P Ness, T Kickler … (2015)

The current report details the serologic findings in a case reported previously of a P1k woman, para 0 gravida 13, who was treated during her fourteenth pregnancy with plasmapheresis to reduce the anti-P titer. These studies suggest that anti-P can induce early abortion in Pk women and that the abortions are immunologically mediated. Further, this case supports the disputed proposal that the anti-P component of anti-PP1Pk is responsible for pregnancy loss in p women.

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The functional significance of epitope spreading and its regulation by co-stimulatory molecules.

K Neville, W S Begolka, J Bluestone … (1998)

Epitope spreading is a process whereby epitopes distinct from and non-cross-reactive with an inducing epitope become major targets of an ongoing immune response. This phenomenon has been defined in experimental and natural situations as a consequence of acute or persistent infection and secondary to chronic tissue destruction that occurs during progressive autoimmune disease. We have investigated the functional significance of this process in the chronic stages of both autoimmune and virus-induced central nervous system (CNS) demyelinating disease models in the SJL/J mouse. During the relapsing-remitting course of experimental autoimmune encephalomyelitis (R-EAE) induced with defined encephalitogenic myelin peptides, CD4+ T cells specific for endogenous epitopes on both the initiating myelin protein (intramolecular epitope spreading) and distinct myelin proteins (intermolecular epitope spreading) are primed secondary to myelin destruction during acute disease and play a major functional role in mediating disease relapses. Similarly, epitope spreading to endogenous myelin epitopes appears to play a major functional role in the chronic-progressive course of Theiler's murine encephalomyelitis virus-induced demyelinating disease (TMEV-IDD), a virus-induced CD4+ T-cell-mediated immunopathology. In TMEV-IDD, myelin destruction is initiated by virus-specific CD4+ T cells which target virus epitopes persisting in CNS-derived antigen-presenting cells. However, the chronic stage of this progressive disease is associated with the activation of CD4+ T cells specific for multiple myelin epitopes. In both models, the temporal course of T-cell activation occurs in a hierarchical order of epitope dominance, spreading first to the most immunodominant epitope and progressing to lesser immunodominant epitopes. In addition, epitope spreading in R-EAE is regulated predominantly by CD28/B7-1 co-stimulatory interactions, as antagonism of B7-1-mediated co-stimulation using anti-B7-1 F(ab) fragments is an effective ameliorative therapy for ongoing disease. The process of epitope spreading has obvious important implications for the design of antigen-specific therapies for the treatment of autoimmune disease since these therapies will have to identify and target endogenous self epitopes associated with chronic tissue destruction.

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Author and article information

Journal

Journal ID (nlm-ta): Mediterr J Hematol Infect Dis

Journal ID (iso-abbrev): Mediterr J Hematol Infect Dis

Journal ID (publisher-id): Mediterranean Journal of Hematology and Infectious Diseases

Title: Mediterranean Journal of Hematology and Infectious Diseases

Publisher: Università Cattolica del Sacro Cuore

ISSN (Electronic): 2035-3006

Publication date Collection: 2016

Publication date (Electronic): 18 October 2016

Volume: 8

Issue: 1

Electronic Location Identifier: e2016053

Affiliations

[1 ]Blood Transfusion Centre, University General Hospital of Evros, Alexandroupolis, Greece

[2 ]Department of Hematology, Democritus University of Thrace Faculty of Medicine, Alexandroupolis, Greece

[3 ]Department of Pediatrics, Democritus University of Thrace Faculty of Medicine, Alexandroupolis, Greece

Author notes

Correspondence to: Zoe Bezirgiannidou, MD, Hematologist, Blood Transfusion Centre, University Hospital of Alexandroupolis, 68100, Greece. Tel: +302551351510. E-mail: zbezi@ 123456hotmail.com

Article

Publisher ID: mjhid-8-1-e2016053

DOI: 10.4084/MJHID.2016.053

PMC ID: 5111518

PubMed ID: 27872733

SO-VID: 41ec8ac8-9ca4-4c4a-9960-22a8482a2680

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Hyperhemolytic Syndrome Complicating a Delayed Hemolytic Transfusion Reaction due to anti-P1 Alloimmunization, in a Pregnant Woman with HbO-Arab/β-Thalassemia

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Abstract

Background

Case report

Conclusion

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Most cited references 26

Delayed hemolytic transfusion reaction/hyperhemolysis syndrome in children with sickle cell disease.

The association of anti-P and early abortion.

The functional significance of epitope spreading and its regulation by co-stimulatory molecules.

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