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

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

doi:10.4084/mjhid.2016.053

Cited by 8 publications

(11 citation statements)

References 23 publications

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“…Further data are needed to conclusively determine whether this drug is safe to be used during pregnancy. Although there are published cases of HS complicating pregnancy, ours is the first published case of hyperhemolysis prophylaxis in a pregnant patient 53,54 …”

Section: Discussionmentioning

confidence: 87%

“…Although there are published cases of HS complicating pregnancy, ours is the first published case of hyperhemolysis prophylaxis in a pregnant patient. 53,54 2.5 | Delivery management By 36 weeks' gestation, the risks of prematurity were felt to be minimal while the maternal and fetal consequences of ongoing profound anemia were significant. Cesarean section used in this case given that worsening thrombocytopenia could preclude the administration of neuraxial anesthesia.…”

Section: Hyperhemolysis Prophylaxismentioning

confidence: 99%

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Peripartum hyperhemolysis prophylaxis and management in sickle cell disease: A case report and narrative review

et al. 2020

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Background: Sickle cell disease (SCD) is associated with hematologic complications including delayed hemolytic transfusion reactions (DHTRs) and pregnancy-related morbidity and mortality. Hyperhemolysis syndrome (HS) is the most severe form of DHTR in patients with SCD, in which both transfused and native red blood cells are destroyed. Further transfusions are avoided after a history of HS. Immunosuppressive agents can be used as prophylaxis against life-threatening hemolysis when transfusion is necessary. There is a paucity of evidence for the use of HS prophylaxis before transfusions, the continuation of hydroxyurea (HU) in lieu of chronic transfusion, and the use of erythropoiesisstimulating agents (ESA) in pregnant SCD patients. Case Report: We present a case of a pregnant patient with SCD and a previous history of HS. HS prophylaxis was given before transfusion with corticosteroids, intravenous immunoglobulin, and rituximab. In addition, HU was continued during pregnancy to control SCD, along with the use of concomitant ESA to maintain adequate hemoglobin levels and avoid transfusion. We describe a multidisciplinary approach to pregnancy and delivery management including tailored anesthetic and obstetric planning. Conclusion: This is the first published case of HS prophylaxis in a pregnant SCD patient, with good maternal and fetal outcomes after transfusion. HU and ESAs were able to control SCD and mitigate anemia in lieu of prophylactic transfusions during pregnancy. Further prospective studies are necessary to elucidate the ideal management of pregnant SCD patients with a history of HS or other contraindications to chronic transfusion.

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Section: Discussionmentioning

confidence: 87%

Section: Hyperhemolysis Prophylaxismentioning

confidence: 99%

Peripartum hyperhemolysis prophylaxis and management in sickle cell disease: A case report and narrative review

et al. 2020

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“…Treatments that have been used for HHS include corticosteroids, intravenous immunoglobulins, rituximab, blood exchange therapy and erythropoietin therapy 1,6,7,27,28 . Because of the lack of vigilance due to no previous case reports of HHS in China, the diagnosis of HHS in this case was delayed, and no other targeted therapy was given except for a single‐dose of intravenous immunoglobulin (1 g/kg) and dexamethasone (10 mg) given after the third and fourth blood transfusions, respectively.…”

Section: Discussionmentioning

confidence: 99%

“…Hyperhaemolysis syndrome (HHS) involves the accelerated destruction of red blood cells of the donor and recipient after blood transfusion, thus resulting in severe haemolytic anaemia syndrome in the recipient 1 . HHS is very rare, and most reported cases of HHS occur in patients with sickle cell disease (SCD) receiving transfusion treatment, 2‐5 whereas only a few cases have been reported in the patients with thalassemia 6‐8 and non‐haemoglobinopathy receiving transfusion treatment 9‐11 . The mechanism of HHS is still not completely clarified.…”

Section: Introductionmentioning

confidence: 99%

A case of hyperhaemolysis syndrome in a pregnant Chinese woman with β‐thalassemia during perinatal transfusion

Dai

et al. 2020

Transfusion Medicine

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Objectives To report a case of hyperhaemolysis syndrome (HHS) that occurred during perinatal blood transfusion in a pregnant Chinese woman with β‐thalassemia to deepen the understanding of HHS and the risk of transfusion therapy for patients with thalassemia. Background Most HHS cases occur in people with sickle cell disease. So far, no cases of HHS have been reported in the Chinese population. Here, we report a pregnant Chinese women with β‐thalassemia experiencing HHS. Methods The patient received ABO‐ and RhD‐matched red blood cell transfusion from six blood donors in four perinatal transfusions. Haemoglobinuria and lower haemoglobin levels compared to those before transfusion were observed after each transfusion, and the lactate dehydrogenase was consistently elevated. The blood samples were collected at different time points during the hospitalisation for direct antiglobulin test (DAT), antibody screening test and acid elution test. The antigens of six blood donors were identified, and the cross‐matching tests were repeated using the blood sample of the patient with specific irregular antibodies after the last transfusion. Results The DAT of the patient was negative for anti‐IgG and positive (1+) for anti‐C3d, and no red blood cell antibodies were detected in the eluent before, between and after transfusions. Before and between transfusions, blood samples were negative for red blood cell irregular antibodies, whereas IgM anti‐P1 and IgG anti‐Jka were detected in blood samples the next day after the last transfusion. In the six donors, two were negative for P1 and Jka, one was positive for P1 and negative for Jka, and three were negative for P1 and positive for Jka. The tentative cross‐matching tests using the indirect antiglobulin method in saline showed that only agglutination occurred in the blood samples of the patient collected after last transfusion and the three Jka‐positive blood donors. Discussion The clinical manifestations and laboratory test results suggested that HHS occurred in this patient with β‐thalassemia after each transfusion. Clinicians should be aware that HHS can occur with compatible blood transfusion.

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“…In 2016, anti-P1 alloimmunization in combination with an autoanti-I caused a delayed hyperhemolytic transfusion reaction in a pregnant patient with thalassemia intermedia. 3 When the implicated antibodies against P1 antigen are of immunoglobulin M (IgM) type, they risk being missed in antibody screening methods that primarily target IgG antibodies. This finding was well exemplified in a recent study involving a clinically mild acute hemolytic transfusion reaction caused by anti-P1.…”

Section: Antibodiesmentioning

confidence: 99%

The P1PK blood group system: revisited and resolved

Stenfelt

Hellberg²,

Westman

et al. 2020

Immunohematology

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This update on the P1PK blood group system (Hellberg Å, Westman JS, Thuresson B, Olsson ML. P1PK: the blood group system that changed its name and expanded. Immunohematology 2013;29:25–33) provides recent findings concerning the P1PK blood group system that have both challenged and confirmed old theories. The glycosphingolipids can no longer be considered the sole carriers of the antigens in this system because the P1 antigen has been detected on human red blood cell glycoproteins. New indications suggest that P1Pk synthase activity truly depends on the DXD motif, and the genetic background and molecular mechanism behind the common P1 and P2 phenotypes were found to depend on transcriptional regulation. Transcription factors bind the P1 allele selectively to a motif around rs5751348 in a regulatory region of A4GALT, which enhances transcription of the gene. Nonetheless, unexplained differences in antigen expression between individuals remain.

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

Cited by 8 publications

References 23 publications

Peripartum hyperhemolysis prophylaxis and management in sickle cell disease: A case report and narrative review

Peripartum hyperhemolysis prophylaxis and management in sickle cell disease: A case report and narrative review

A case of hyperhaemolysis syndrome in a pregnant Chinese woman with β‐thalassemia during perinatal transfusion

The P1PK blood group system: revisited and resolved

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