Sideroblastic anaemia in a patient with sickle cell disease.

Sideroblastic anaemia is a rare condition. We report a unique case of concomitant sideroblastic anaemia in a patient with sickle cell disease with long-standing blood transfusion history. Due to a low prevalence of sideroblastic anaemia, the diagnosis of sideroblastic anaemia is often difficult, especially when coexisting with common types of anaemia, including sickle cell disease. This case highlights the detrimental effects of anchoring bias. Rare causes of refractory anaemia should be considered in patients with haemoglobin disorders as the therapeutic approaches for these conditions are different. High suspicion on the part of the clinician and low threshold for workup of anaemia often aids in the diagnosis of coexisting conditions such as sideroblastic anaemia. Early diagnosis and treatment of sideroblastic anaemia improves patient outcomes and prevents long-term complications.

Continuous infusion factor replacement in haemophilia B during and after cardiac surgery: the better choice?

A 57-year-old man with mild haemophilia B was admitted for coronary artery bypass graft surgery. His factor IX (FIX) activity was 15% on admission. Our goal was to maintain his FIX activity at 80%-100% for post-op days (PODs) 0-3, and at 60%-80% for PODs 4-14. Preoperatively, the patient was given recombinant FIX (rFIX) bolus using the formula:Dosage needed=%(desired FIX level-current level of FIX)×weight (kg)×1.3.This increased his activity to 100%. One IU of rFIX increased FIX activity by 0.8%; the half-life of rFIX is 18-24 hours. The rFIX infusion was started intraoperatively and continued after surgery to maintain target FIX activity. He was discharged on POD 9 on rFIX bolus dosing of 5000 IU every 12 hours for an additional 5 days. Using continuous factor infusion, we managed to decrease the amount rFIX used by >60% while maintaining steady state FIX activity level.