BIOLOGY 2F03 Chapter Notes - Chapter 9-10: Splenectomy, Deferasirox, Iron Overload
20 May 2018
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• Pulmonary HTN - can occur in upto 60% of patients that are not regularly transfused as a result
of free Hb and NO depletion
• Hemochromatosis
o As a physiological response to chronic anaemia + ineffective erythropoiesis as well as
due to regular transfusions
o It is difficult to assess Fe loading, T2* MRI is now used to image heart and liver
o Non-transferrin-bound-iron only drops for the duration of chelation; so short of 24/7
infusions, a patient will always develop iron overload
• Psychosocial - different for different people
o Regular hospital visits = loss of education, activity, integration
o Bony changes and jaundice = self consciousness
o Anxiety
o Sexual maturation/function problems
o 81% said they would have chosen rpe natal diagnosis and abortion in hindsight
1. Other treatment methods in beta thal intermedia
• The OPTIMALCARE study 2010 said people who are regularly transfused and chelated have a
lowed incidence of complications than those with no treatment
o But you must make the decision based on patient preferences because there is no clear
life expectancy difference with either avenue
• Oral Chelators
o Deferriprone (TDS) is good for decreasing cardiomyopathy but has SFX of
agranulocytosis, arthritis and GI intolerance
o Deferasirox (OD) also have SFX of GI intolerance as well as renal and liver trouble
o Evidence is not sure whther these are better than normal infusions
• Splenectomy - can riase Hb by 2g/dl and is indicated for
o Hypersplenic patients; painfuk/bulky spleen
o Where transfusion/chelation is not available/doable
o Watch out for risk of thrombosis and sepsis after splenectomy
• Hydroxyurea may increase HbF levels but clinically, it is inconsistent
• Mini allografts are basically partial BM transplants
• Gene therapy may be an option for the future
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