重型β地中海贫血患者肝脾肿大原因探析

Factors Affecting Hepatomegaly and Splenomegaly in Patients with Beta Thalassemia Major

目的探讨重型β地中海贫血(thalassemia major,TM)患者肝脾肿大的影响因素。方法 2008-07/2010-03月,171例TM患者接受腹部超声检查,观察其肝脾肿大情况及胆囊结石的发生,并记录一般情况、输血和去铁状况,测量血清铁蛋白浓度。结果 171例患者中113例肝脏增大,89例脾脏增大,15例曾行切脾术,4例有胆囊结石。地贫基因类型、是否高量输血是影响脾脏增大的因素。结论 TM患者中肝脾增大较为普遍,需加强规范化输血治疗,以维持输血前血红蛋白大于90g/L,尤其是β0/β0纯合子TM患者。

Objective To investigate the correlated factors of hepatomegaly and splenomegaly in patients with beta thalassemia major （TM）. Methods From July 2008 to March 2010, 171 TM patients underwent abdominal ultrasound examination.The status of hepatomegaly and splenomegaly and the occurrence of cholecystolithiasis were observed. Their general condition and the status of blood transfusion and iron chelator were recorded. Serum ferritin concentration was detected. Results Among 171 patients, 113 had hepatomegaly,89 had splenomegaly,15 underwent splenectomy,and 4 had cholecystolithiasis. Hemoglobin level before blood transfusion and type of TM genotype were the independent factors affecting the enlargement of spleen. Conclusion Hepatomegaly and splenomegaly are common in TM patients. Standardized blood transfusion should be intensified to keep hemoglobin level before transfusion higher than 90g/L, especially for β0/β0 patients.