مرض HbH غير الحذفي (--/α^Tα): ماذا تفعل عندما تفشل عمليات نقل الدم المنتظمة في الحفاظ على مستوى الهيموغلوبين المستهدف

References

• Clinical phenotypes are diverse among affected individuals with non-deletional haemoglobin H (HbH) disease (--/αTα).
• Clinical symptoms of non-deletional HbH are generally more severe than those of deletional forms.
• Therefore, splenectomy is recommended in selected patients with non-deletional HbH disease. These indications include severe anaemia that impedes growth and development in the patients with limited access or poor adherence to regular blood transfusions and/or iron chelation, hypersplenism with anaemia, leukopenia, or thrombocytopenia that results in infections or bleeding, or massive splenomegaly with left upper quadrant pain that increases the risk of splenic rupture.
• Splenectomy should generally be avoided in patients younger than 5 years.
• Studies have shown that splenectomy can increase haemoglobin levels by 10-30 g/L and decrease or eliminate the need for blood transfusions in HbH-CS.