Accelerated erythrocyte destruction mimicking post‐transfusion hyperhaemolysis in the course of uncomplicated vaso‐occlusive crisis associated with sickle cell disease

Abstract

This report regards a 40-year-old man, diagnosed with sickle cell anaemia (SCA; HbSS), who was admitted to our emergency service with symptoms of vaso-occlusive crisis. The patient was not under chronic transfusion therapy, and the baseline haemoglobin was 7 g/dL. He was initially treated with opioid analgesics and hydration. The initial laboratory investigation showed Hb = 7.1 g/dL, Ht = 21.1%, lactate dehydrogenase (LDH) = 384 U/L, total bilirubin = 2.3 mg/dL and indirect bilirubin = 1.32 mg/dL. Anti-E and anti-Di a were identified in the antibody screening using the gel method (ID-DiaPanel and ID-DiaPanel-P, BIORAD, Lagoa Santa, Brazil). Previous immunohaematological investigation had shown only anti-E. He was transfused with two red blood cell (RBC) units (254 and 261 mL), without any clinical intercurrences during the infusion. The patient's phenotype was R 0 r; K−; Jk(a + b−); Fy(a + b−); S−, s+ and Dia−, and the transfused units had the same phenotype. In addition, molecular investigation for the most common RHD 1 and RHCE variants (Restriction Fragment Length Polymorphism-RFLP-PCR designed to detect c.733C>G) was performed and was negative. All transfused units were HbS-negative, and the patient's blood volume was 4.5 L. The laboratory tests performed approximately 8 hours after the transfusion revealed a sharp decrease in the haemoglobin levels (posttransfusion Hb = 5 g/dL) and an increase in LDH (550 U/L). Parvovirus polymerase chain reaction was performed and was negative.

Epidemiological and clinical features, therapeutic strategies and outcomes in patients with hyperhaemolysis: A systematic review

“…As the epidemiology and underlying pathophysiology have yet to be definitively elucidated, recognition of HHS can be challenging, as clinicians may not consider this condition in the absence of detectable antibodies or a haemoglobinopathy diagnosis. DHTRs and HHS may be difficult to distinguish, and symptoms of HHS may also mimic those of a vaso‐occlusive crisis 15 . Moreover, although American Society of Hematology (ASH) guidelines provide features of HHS, there are no published diagnostic criteria that define HHS for research purposes at an international level 16 .…”

“…DHTRs and HHS may be difficult to distinguish, and symptoms of HHS may also mimic those of a vaso-occlusive crisis. 15 Moreover, although American Society of Hematology (ASH) guidelines provide features of HHS, there are no published diagnostic criteria that define HHS for research purposes at an international level. 16 Thus, the literature is inconsistent, as cases of post-transfusion haemolysis have been diagnosed and reported as HHS despite the presence of reticulocytosis not attributed to ESA and/or haemoglobin values that do not decrease below the pretransfusion baseline haemoglobin.…”