Aplastic anaemia and bone-marrow failure: Viva

This is a presentation of severe aplastic anaemia in a nine-year-old boy, meeting the Camitta criteria. The structured oral runs from the severity grading through the pathophysiology, the exclusion of the inherited syndromes, the treatment fork, and the trial evidence, and it ends with the long term counselling. ^[1]

Opening: grade the severity

Examiner. This boy has pancytopenia and a hypocellular marrow. How do you grade the severity, and what does the grade mean for the treatment? ^[1]

Candidate. The boy meets the Camitta criteria for severe aplastic anaemia, which is a marrow cellularity under 25 percent together with at least two of three peripheral blood thresholds. He has a cellularity of 15 percent, which meets the marrow criterion, and he meets all three blood thresholds: the neutrophils of 0.3 times ten to the nine per litre are under 0.5, the platelets of 12 times ten to the nine per litre are under 20, and the reticulocytes of 10 times ten to the nine per litre are under 60. The grade is severe, and it is close to very severe, which is the neutrophils under 0.2 times ten to the nine per litre. The grade matters because the severe and the very severe disease are treated aggressively with the transplant or the immunosuppression, while the non-severe disease is often managed supportively. ^[1]