A diagnostic nomogram for delayed hemolytic transfusion reaction in sickle cell disease

Abstract

Diagnosis of delayed hemolytic transfusion reactions (DHTR), one of the most dreaded complications of transfusion in patients with sickle cell disease (SCD), is challenging and not straightforward. Current diagnostic approaches are complex and not consensual; they are based on assessment of hemoglobin (Hb) drop and enhanced hemolysis, features also seen during classical vaso-occlusive events. In this observational study, we tested the hypothesis that the rate of decline in HbA after an index transfusion is a surrogate marker for the destruction of transfused RBC, which could be used diagnostically. We examined 421 transfusion episodes (in 128 patients of a French referral center for SCD) for which an Hb electrophoresis was obtained within 1 week following an index transfusion and repeated within 2 months (before a subsequent scheduled transfusion or during an acute complication). Chart review found DHTR to be present in 26 cases (6.2%), absent in 389 cases (92.4%), and possible in six cases (1.4%). As expected, DHTR was associated with accelerated hemolysis (increased serum bilirubin and lactic dehydrogenase concentrations) and a decline in total Hb as compared to the early post-transfusion value. However, the decline in HbA concentration appeared more effective in segregating between patients without DHTR and others. We propose a diagnostic nomogram for DHTR based on Hb A as a biologic marker of the survival of transfused RBCs.

Sickle cell disease and COVID‐19: Atypical presentations and favorable outcomes

“…SARS‐CoV‐2 test was still positive on nasal swab. Coombs test was negative but since she had been recently transfused, evaluation for the risk of DHTR was made according to the proposed model by the team of Habibi et al [3] and considered intermediate. We decided not to transfuse nor perform a second ET and rather observed her carefully.…”

“…Our third patient presented initially no lung lesions despite a severe pneumomediastin but after het exchange transfusion developed abnormality on CT scanner compatible with COVID‐19, in fact related to delayed hemolytic transfusion reaction. We encourage clinicians to treat SCD patients with COVID‐19 infection on a case by case basis and not consider them for transfusion invariably but if transfused, to always consider and score for the risk of DHTR [3]. We are hopeful that a careful analysis of the large registries of COVID‐19 among patients with hemoglobinopathies will allow us to better understand this intriguing relationship between COVID‐19 and SCD.…”

Increased Prevalence of Alloimmunization in Sickle Cell Disease? Should We Restore Blood Donation in French Guiana?

“…All of these data were recorded in an anonymous Excel database. A delayed hemolytic transfusion reaction was suspected when a patient presented several days after RBC transfusion with the development or intensification of symptoms suggestive of a painful crisis, hemolysis, reticulocytopenia, and worsening anemia, reversing the effects of the previous RBC transfusion (12).…”

Aloimunização eritrocitária e HLA em doença falciforme

“…Na DF, as reações transfusionais são em geral mais graves, podem desencadear CVOs e cursar com quadro de hiperhemólise (74). Além disso, a reação hemolítica tardia pode não ser diagnosticada, uma vez que os sinais e sintomas comumente podem ser confundidos com os da doença de base (75). A aloimunização pode também representar um marcador de gravidade da doença, tendo sido associada à dor crônica, à dependência de opioide, à lesão de órgão e à menor sobrevida (76).…”

“…A morbidade e a mortalidade relacionadas à aloimunização são provavelmente subestimadas. As RTHs não são reconhecidas, pois os sinais e sintomas se sobrepõem aos da própria doença de base e são em geral diagnosticadas como novas crises agudas da DF (66,75,176). Não obstante, as complicações secundárias aos atrasos para obter doadores compatíveis e as consequências da suspensão de procedimentos clínicos ou cirúrgicos não são em geral notificadas formalmente como um problema relacionado à aloimunização.…”