مرض HbH غير الحذفي: إدارة الخطوة التالية عندما لا يحقق استئصال الطحال الارتفاع المستهدف للهيموغلوبين

References

• Clinical phenotypes are diverse among affected individuals with non-deletional haemoglobin H (HbH) disease (--/α^Tα).
• Clinical symptoms of non-deletional HbH are generally more severe than those of deletional forms.
• Iron chelation should be started if LIC >5 mg/g dry weight or ferritin >500 ng/mL.
• Deferasirox doses in the range of 10 to 15 mg/kg/day have been suggested for NTDT.
• Basically, chelation should be started when the LIC is greater than 5 mg/g DW or the serum ferritin is greater than 500 ng/mL with a goal of keeping the LIC between 2 to 5 mg/g DW or the ferritin between 300 and 800 ng/mL.