management of this severe acute and rare condition. Diagnosis must be verified No By which specialty Exclusion Criteria Autoimmune haemolytic anaemia (AIHA) Alloimmune delayed haemolytic transfusion reaction with a positive direct agglutination test where haemolysis of only transfused red cells is suspected. Exclusion criteria have been defined to support evidence based prescribing practice. AIHA is excluded because it is a standalone condition in the Emerging role category (the system will link users to the correct condition). There is insufficient evidence of benefit for the use of Ig therapy in delayed allo-immune reaction. There have been only six cases reported in the literature across two papers with the evidence being very weak with four of the six cases being auto-antibodies. Qualifying Criteria Severe haemolytic transfusion reactions where there is suspected haemolysis of both donor and recipient red cells as evidenced by a fall in haemoglobin below pre-transfusion levels AND Ig therapy is given in conjunction with high dose corticosteroids Qualifying criteria have now been developed to align with the literature. The supporting evidence items will confirm that the drop in haemoglobin is related to the transfusion, compare the significance of drop in haemoglobin compared with the transfusion volume, and that ensure intravenous steroids are being given in combination with Ig therapy. Review Criteria Review is not mandated for this condition however the following criteria may be useful in assessing the effectiveness of therapy. Clinical effectiveness of Ig therapy can be demonstrated by: Review is not mandated for this condition because treatment is by one-off dose.
• Resolution of haemolysis • Stable haemoglobin • Rise in haemoglobin Dose Refer to the current product information sheet for further information. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. Induction Dose – Up to 2 g/kg over 1-2 days Additional dose may be requested when the patient’s haemoglobin has not improved. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. Refer to the current product information sheet for further information on dose, administration and contraindications. While a continuing treatment period of Ig therapy is not required, this condition is likely to involve prolonged haemolysis and Ig is seen as life-saving. The Specialist Working Group advised that uncommonly, a second dose might be required, when the patient has not improved, and may be requested up to six weeks after the first dose. References (most recent update: July 2016) Danaee A, Howard J, Roberston B and Robinson S (2014) Hyperhaemolysis in a patient with HbH disease. Transitional Medicine, 24:244-5. Ontario Regional Blood Coordinating Network (2016). Ontario Intravenous Immune Globulin (IVIG) Utilization Management Guidelines, Version 3.0. [online]. Available at: http://transfusionontario.org/en/download/ontario-intravenous-immune-globulin-IVIg-utilization-management-guidelines-2/. Stokes IC, Downie PA, Wood EM, Bowden DK, Monagle PT and Barnes CD (2001) Hyperhaemolysis in sickle cells disease – an unusual and potentially complication. Medical Journal of Australia, 192:281-2.