Hyperhemolysis in Patients With Hemoglobinopathies: A Single-Center Experience and Review of the Literature

Danaee, Anicee; Inusa, Baba; Howard, Jo; Robinson, S.

doi:10.1016/j.tmrv.2015.06.001

Cited by 54 publications

(87 citation statements)

References 56 publications

(65 reference statements)

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“…Recurrence of hyperhaemolysis may be prevented by the administration of IVIG and methylprednisolone prior to subsequent transfusions (Danaee et al, 2015).…”

Section: Guidelinementioning

confidence: 99%

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

et al. 2016

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“…Recurrence of hyperhaemolysis may be prevented by the administration of IVIG and methylprednisolone prior to subsequent transfusions (Danaee et al, 2015).…”

Section: Guidelinementioning

confidence: 99%

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

et al. 2016

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“…Four hypotheses have been exposed1: (1) ‘bystander haemolysis’, with a higher risk of formation of C5-9 membrane attack complex in patients with SCD; (2) macrophage activation because reticulocytes and mature sickle RBCs adhere more avidly to macrophages than normal RBC. The rise in macrophage activity could cause reticulocytopenia via peripheral consumption, and is consistent with the increased erythroid activity shown in bone marrow aspirates.…”

Section: Discussionmentioning

confidence: 99%

“…In the study at Guys and St. Thomas’ Hospital,1 only nine cases were recorded for 4 years in a population of 1000 patients with SCD, 100 of whom were on a transfusion programme. Of the 79 reported patients (77 reported by Danae1 and 2 more recent cases18 19), 29 (39.7%) appeared in the paediatric age with a median of 7.8 years, with no gender difference; 22 children (75.8%) had SCD, 4 (13.8%) had β-thalassaemia major and 3 (10.3%) thalassaemia intermedia. Nine children (31%) had antibodies before transfusion and 11 (37.9%) developed them later.…”

Section: Discussionmentioning

confidence: 99%

“…It is therefore advisable in patients where the transfusion may be vital and cannot be avoided to administer both treatments before. Rituximab may play a role as a preventive treatment 1. Other treatments such as erythropoietin,1 6 15 18–20 22 rituximab1 2 18 19 23 and eculizumab1 6 have been tried in the event, but with limited success.…”

Section: Discussionmentioning

confidence: 99%

“…It can appear acutely (before 7 days) or delayed (after 7 days) after a red blood cell (RBC) transfusion. It can be mistaken for a delayed haemolytic transfusion reaction (DHTR) although direct antiglobulin test (DAT) is negative, or when positive it does not explain the haemolytic crisis 1. Most patients suffered from haemoglobinopathies, but this syndrome has also been described in other pathologies 1 3.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

When a transfusion in an emergency service is not really urgent: hyperhaemolysis syndrome in a child with sickle cell disease

Langeber¹,

Marco

Benedit

et al. 2018

BMJ Case Reports

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A 13-month-old boy with sickle cell disease (SCD) from Equatorial Guinea, who had recently arrived in Spain, presented with fever. He had suffered from malaria and had received a blood transfusion. Following physical examination and complementary tests, intravenous antibiotics and a red blood cell (RBC) transfusion were administered. Soon after a second transfusion 5 days later, the haemoglobin level fell below pretransfusion levels, together with reticulocytopenia, and haematuria-the so-called hyperhaemolysis syndrome-requiring intensive care and treatment with intravenous immunoglobulins and corticosteroids, with resolution of the complication. We want to emphasise the importance of suspecting this rare, though severe complication that can appear after any RBC transfusion especially in patients with SCD, as the clinical syndrome can simulate other more common complications of these patients and a further transfusion is contraindicated. There is no standardised treatment, but intravenous immunoglobulin and corticosteroids are usually effective.

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Incidence and predictive score for delayed hemolytic transfusion reaction in adult patients with sickle cell disease

et al. 2017

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Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication of transfusion in sickle cell disease (SCD). The frequency of DHTR is underestimated because its symptoms mimic those of vaso-occlusive crisis and antibodies (Abs) are often not detectable. No predictive factors for identifying patients likely to develop DHTR have yet been defined. We conducted a prospective single-center observational study over 30 months in adult sickle cell patients. We included 694 transfusion episodes (TEs) in 311 patients, divided into occasional TEs (OTEs: 360) and chronic transfusion program (CTEs: 334). During follow-up, 15 cases of DHTR were recorded, exclusively after OTEs. DHTR incidence was 4.2% per OTE (95% CI [2.6; 6.9]) and 6.8% per patient during the 30 months of the study (95% CI [4.2; 11.3]). We studied 11 additional DHTR cases, to construct a predictive score for DHTR. The DHTR mortality is high, 3 (11.5%) of the 26 DHTR patients died. The variables retained in the multivariate model were history of DHTR, number of units previously transfused and immunization status before transfusion. The resulting DHTR-predictive score had an area under the ROC curve of 0.850 [95% CI: 0.780-0.930], a negative-predictive value of 98.4% and a positive-predictive value of 50%. We report in our study population, for the first time, the incidence of DHTR, and, its occurrence exclusively in occasionally transfused patients. We also describe a simple score for predicting DHTR in patients undergoing occasional transfusion, to facilitate the management of blood transfusion in SCD patients.

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Hyperhemolysis in Patients With Hemoglobinopathies: A Single-Center Experience and Review of the Literature

Cited by 54 publications

References 56 publications

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

When a transfusion in an emergency service is not really urgent: hyperhaemolysis syndrome in a child with sickle cell disease

Incidence and predictive score for delayed hemolytic transfusion reaction in adult patients with sickle cell disease

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