Hemoglobin sickle cell disease complications: a clinical study of 179 cases
Hemoglobin sickle cell disease complications: a clinical study of 179 cases
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DOI：
10.3324/haematol.2011.055202
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发表时间：
2012-08-01
期刊：
影响因子：
--
通讯作者：
Haymann, Jean-Philippe
中科院分区：
文献类型：
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作者：
Lionnet, Francois;Hammoudi, Nadjib;Haymann, Jean-Philippe
背景血红蛋白镰状细胞病是最常见的血红蛋白病之一。令人惊讶的是，很少有研究致力于这种疾病，目前被认为是纯合镰状细胞病的轻度变异。本研究的目的是更新我们对血红蛋白镰状细胞病的认识。设计和方法该研究涉及 179 名患者的单中心系列研究。收集临床和生物学数据，特别关注肺动脉高压和肾病的评估。结果血红蛋白镰状细胞诊断被推迟，并在29%的病例中在成年时进行。住院期间疼痛性血管闭塞危象、急性胸部综合征和阴茎异常勃起的发生率分别为 36%、20% 和 20%。最常见的慢性器官并发症是视网膜病变和感觉神经性耳科疾病，分别占 70% 和 29% 的病例。事实上，纯合镰状细胞病的并发症发生率相当低，例如肾病、疑似肺动脉高压、中风和腿部溃疡（分别为 13%、4% 和 1%）。该人群中有 36% 进行了放血（基线血红蛋白 11.5 g/dL），预防了 71% 病例的急性事件复发。结论我们的数据表明，血红蛋白镰状细胞病不应被视为镰状细胞性贫血的轻度形式，而应被视为一种单独的疾病，特别强调与粘性相关的耳科和眼科疾病，并且血管病（中风、肺动脉高压、溃疡和糖尿病）患病率较低。肾病）。放血术有助于减少急性事件，应进一步研究该手术的更广泛应用。
BackgroundHemoglobin sickle cell disease is one of the most frequent hemoglobinopathies. Surprisingly, few studies have been dedicated to this disease, currently considered to be a mild variant of homozygous sickle cell disease. The aim of this study was to update our knowledge about hemoglobin sickle cell disease.Design and MethodsThe study involved a single center series of 179 patients. Clinical and biological data were collected with special attention to the assessment of pulmonary arterial hypertension and nephropathy.ResultsHemoglobin sickle cell diagnosis was delayed and performed in adulthood in 29% of cases. Prevalence of hospitalized painful vasoocclusive crisis, acute chest syndrome and priapism was 36%, 20% and 20%, respectively. The most common chronic organ complications were retinopathy and sensorineural otological disorders in 70% and 29% of cases. Indeed, prevalence of complications reported in homozygous sickle cell disease, such as nephropathy, suspicion of pulmonary hypertension, strokes and leg ulcers was rather low (13%, 4% and 1%, respectively). Phlebotomy performed in 36% of this population (baseline hemoglobin 11.5 g/dL) prevented recurrence of acute events in 71% of cases.ConclusionsOur data suggest that hemoglobin sickle cell disease should not be considered as a mild form of sickle cell anemia but as a separate disease with a special emphasis on viscosity-associated otological and ophthalmological disorders, and with a low prevalence of vasculopathy (strokes, pulmonary hypertension, ulcers and nephropathy). Phlebotomy was useful in reducing acute events and a wider use of this procedure should be further investigated.