[血红蛋白C病：16例突尼斯病例报告]
[Hemoglobin C disease: report of 16 Tunisian cases].
作者信息
Hafsia Raouf, Marrakchi Olfa, Ben Salah Naouel, Gouider Emna, Ben Lakhal Ryhane, Jeddi Ramzi, Aissaoui Lamia, Belhadjali Zaher, Ben Abid Hela, Meddeb Balkis, Hafsia Aïcha
机构信息
Service d'Hématologie Biologique, Hôpital Aziza Othmana, Tunis.
出版信息
Tunis Med. 2007 Mar;85(3):209-11.
AIM
was to provide the clinical and biological patterns hemoglobine disease in Tunisia.
METHODS
This retrospective study collected to 16 cases of hemoglobin C disease : 6 homozygotic Hb C and 10 heterozygotic Hb C/beta-thalassemia.
RESULTS
The clinical profile is characterized by mild hemolytic anemia (Hb = 11.7 g/dl) associated with splenomegaly and hypersplenism. Contrary to homozygous state, the Hb C/beta-thalassemia is associated with microcytosis and pseudopolycythemia. The diagnosis is based on target cells, specific intraerythrocytic Hb C crystals in blood smear and Hb C level at 100%.
CONCLUSION
The Hb C disease must be considered as a benign hemoglobinopathy which is associated with a long survival without major complications.
目的
旨在呈现突尼斯血红蛋白病的临床和生物学特征。
方法
这项回顾性研究收集了16例血红蛋白C病病例：6例纯合子血红蛋白C病和10例杂合子血红蛋白C/β地中海贫血。
结果
临床特征为轻度溶血性贫血（血红蛋白=11.7g/dl），伴有脾肿大和脾功能亢进。与纯合子状态相反，血红蛋白C/β地中海贫血与小红细胞症和假性红细胞增多症相关。诊断基于靶形细胞、血涂片上特异性红细胞内血红蛋白C晶体以及100%的血红蛋白C水平。
结论
血红蛋白C病必须被视为一种良性血红蛋白病，其与长期生存且无重大并发症相关。
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