General Updates | What should the optimal pre-transfusion hemoglobin target be in patients with thalassemia?

As for the pre-transfusion hemoglobin level in thalassemia patients, we had at ASCAT a very interesting debate between myself and doctor Sujit Sheth from Cornell University in New York, where we debated on maintaining the severe transfusion dependent thalassemia patient with the pre-transfusion hemoglobin between 9.5 and 10.5, or more than 10.5 or more than 11. So I was trying to explain the reason for being reasonable to keep a patient with a pre transfusion hemoglobin between 9.5 and 10.5. I based this schedule, which is also reported by the Thalassemia International Federation, even in the last recommendation, based on a paper back to several years ago by Professor Cazzola, who showed the clearly using the transferin receptor and the bone marrow activity, he showed that on that range of hemoglobin, 9.5-10.5, there was a sufficient bone marrow suppression. Because this is really the main purpose of transfusion – to try to reduce as much as possible the ineffective erythropoiesis that is the driver of the pathophysiology in severe thalassemia. So he showed that with a lower level, lower than 9.5, the suppression was not really adequate because still there was a lot of ineffective erythropoiesis, between 9.5 and 10.5 was only 1 or 2 times the residual ineffective erythropoiesis. So it was established that this was a good compromise. Also because we should consider that transfusion brings iron overload. So as much as we are transfusing the patients, the higher is the iron accumulation. So in this range, it was quite adequate.

But what I wanted to stress in my debate was, okay, we may also go up, and then I tell you something that Dr Sheth was explaining. But what I wanted to stress is that it’s fundamental, it is mandatory not being less than 9.5, because less than that, we have severe, ineffective erythropoiesis, and because of the anemia, we increase also the iron absorption. And I try to stress that, because based on evidences and data available, we know that unfortunately in some countries patients are under transfused.

What is the advantage of going up over 11? It has been shown recently by several papers, led by Dr Khaled Musallam, looking at a large cohort of Italian patients with different levels of hemoglobin, that was mainly based on non-transfusion-dependent thalassemia patients. Anyway, he found that patients with a higher level of hemoglobin, so more than ten more than 11, have less morbidities compared to those who have lower values.

So I think that I’m not particularly in favor of one of the other position. I want to say that we need to take into account that we don’t want to go below 9.5. I think we have to adapt the situation to patients according to their clinical status, their needs, and several other problems. So that was the debate for the hemoglobin.

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