Thalassaemia Intermediate and Splenectomy

[kat]

Hi,


I would like to ask Anton these questions please (was referred by Josephine Bila) :

I would like to ask about hypertransfusion (getting 4 units of blood every 2 weeks) with the goal of shrinking the spleen. Is there the chance the spleen will shrink from 26 cm currently to smaller size?

It is expected that 6 months of continuous hypertransfusion is needed in this treatment plan.

What about the iron overload that will occur during this period? Is this dangerous eventhough chelation is being followed?

Also, another question is about pain and aches from hypertransfusion. Is the bone marrow reacting to the increased blood being given? I have aches in the morning all over, but more so in the upper body. And on occasion cramps in foot during the night which is sudden and awakes me from sleep. Cramp lasts around 5 minutes.


Thank you for your assistance with this.

[antonskafi]

Dear Friends;

Please find below the reply of Dr. Perla Eleftheriou;


1) Hypertransfusion can indeed lead to spleen shrinking and is an option I recommend to my patients if their larger spleen causes abnormalities of their counts ( ie low platelets, low white cells) or abdominal discomfort with reduced appetite. The response to the hypertransfusion treatment ( in terms of how much the spleen will shrink) varies from patient to patient but is certainly expected to be beneficial. Whilst on hypertransfusion, iron chelation doses might have ( most likely will have to) be adjusted. That is to say, the doses might have to be increased and this is fine. As a guidance, the ferritin trend will have to be closely monitored ( eg every 2 months).



2) Pain in thalassaemia patients is a big problem, very common and often multifactorial. Pain before blood transfusion is very common ( when the haemoglobin drops low). Extramedullary haematopoiesis, osteoporosis and early spinal/joint degenerative disease can all contribute to this. If worse and persistent upper body pains ( suspect you mean upper spine), perhaps an MRI thoracic spine would help. If someone has bony/joint aches which are worse in the morning in addition to an enlarging spleen I would also recommend for the auto immune profile to be checked as well as a protein electrophoresis. Cramps in the feet/legs can be caused by electrolyte disturbance which can be a side effect of some chelators. Check your Magnesium and Zinc levels!





Hope this helps

Best wishes