The LUST Project: Laboratory Urine Sickle Test

Patients with sickle cell disease (SCD) have a baseline level of intravascular haemolysis that varies between patients. Haemolytic transfusion reactions are common in this patient group and can be associated with antibody formation (delayed haemolytic transfusion reaction (DHTR)) or without – hyperhaemolysis (HH). Patients may have both at the same time and thus the presence of a new antibody does preclude the diagnosis of hyperhaemolysis. There needs to be a validated test to differentiate between the two so as to guide clinical management of the transfusion reaction which differs depending on the cause. In DHTR donor blood only is being haemolysed, in the case of SCD this would be HbA. In the case of HH both donor and recipient blood is haemolysed and therefore HbS (+/- another abnormal haemoglobin in a compound heterozygote) AND HbA are haemolysed. These haemoglobins, as the haemolysis is intravascular, should appear in the urine. 

As there is no current validated test for these haemoglobins in the urine, it is not really possible for clinical teams to request this test and so this impacts on clinical decision making. UK NEQAS is undertaking preliminary work to validate methods for the analysis of different haemoglobins (HbS, HbA, HbC and HbD) in urine to produce simulated quality assurance specimens for the use in inter-laboratory performance assessment of laboratories undertaking this analysis. 

We are asking NHSBT, hospital laboratory or clinical staff who are aware that a patient with SCD is having a haemolytic transfusion reaction to request the clinical team send a urine sample as a matter of urgency BEFORE the haemoglobinuria clears.

Consent for use of the material for quality assurance is not required under the HTA but is required in common law, in the same way as the removal of tissue for diagnostic testing. Approximately 10-20 mL of urine is needed from each patient.  Consecutive samples on the same can be sent as the haemolysis resolves/worsens. Samples should be kept at ambient temperature. There will be a process of pseudoanonymisation so that you will know whose samples have been provided but NEQAS will not.  However, you are asked to supply the patient’s gender, age, haemoglobinopathy disorder, recent blood transfusion history and circulating Hb S % if known.  Specimens will be collected by courier, arranged and paid for by UK NEQAS Haematology. 

On receipt of the sample in the hospital laboratory please email Barbara De la Salle (barbara.delasalle@nhs.net, with a cc to haem@ukneqas.org.uk), stating your contact details (contact name, phone number and road address) and the gender, age, haemoglobinopathy disorder, current Hb, current HbS%, date of last transfusion episode, date of causative transfusion episode if known of the patient.  We will contact you to arrange a convenient time for the courier collection. Sample labelling instructions are described above. 

The specimen should be packaged in the same way as for a ‘send away’ request and labelled:

For the attention of Barbara De la Salle
UK NEQAS Haematology and Transfusion
10 Millfield House
Woodshots Meadow
Croxley Industrial Park
Watford
Herts WD18 8YX

Tel: 01923  587111 

If you have any questions, please email sara.trompeter@nhs.net for more details.

Dr Sara Trompeter - Consultant Haematologist, NHSBT