Causes of massive tropical splenomegaly in Ghana

Causes of massive tropical splenomegaly in Ghana
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DOI:
10.1016/s0140-6736(02)09680-0
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发表时间:
2002-08-10
期刊:
影响因子:
168.9
通讯作者:
Bates, I
Bates, I
中科院分区:
医学1区
文献类型:
--
作者:
Bedu-Addo, G;Bates, I

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背景热带巨脾的病因和诊断尚未得到很好的研究,尤其是现代研究方法。我们的目的是确定功能,这将有助于当地的临床医生区分之间的基础conditions.Methods我们收集了前瞻性的临床和实验室数据,221加纳患者的脾脏大小至少10厘米。我们通过分子和免疫学研究以及常规检查确定了与巨脾相关的疾病。结果高反应性疟疾性脾肿大(HMS; 91例[41%])和B淋巴细胞增生性疾病(48例[22%])是最常见的疾病与巨大的脾肿大。其余患者中,32例(14%)有血液系统疾病,50例(23%)我们无法确定脾肿大的原因。除HMS和热带脾淋巴瘤外,所有诊断组均以男性为主。年龄小于40岁和绝对淋巴细胞计数(小于10 × 10(9)/L)是区分HMS患者和淋巴组织增生性疾病患者的唯一有用和广泛可用的鉴别指标。这一发现对巨脾肿大的治疗有重要意义。这种疾病的不常见原因的诊断通常是简单的,但B淋巴细胞增生性疾病和HMS之间的区分可能是困难的。与淋巴组织增生性疾病相比,HMS与年龄较小、女性比例较高和淋巴细胞绝对计数较低相关。
Background The causes and diagnosis of massive tropical splenomegaly are not well studied, especially with modern investigative methods.. We aimed to identify features that would help local clinicians differentiate between the underlying conditions.Methods We collected prospective clinical and laboratory data on 221 Ghanaian patients with spleen size of at least 10 cm. We identified conditions associated with massive splenomegaly with molecular and immunological investigations as well as routine tests. Patients were assigned to diagnostic categories on the basis of these test results and predetermined criteria.Findings Hyper-reactive malarial splenomegaly (HMS; 91 patients [41%]) and B-lymphoproliferative disorders (48 [22%]) were the most common disorders associated with massive splenomegaly. Of the remaining patients, 32 (14%) had haematological disorders, and in 50 (23%) we could not identify the cause of splenomegaly. Male sex predominated in all diagnostic groups except HMS and tropical splenic lymphoma. Age less than 40 years and absolute lymphocyte count (less than 10 x 10(9)/L) were the only useful and widely available discriminators for distinguishing patients with HMS from those with lymphoproliferative disorders.Interpretation B-lymphoproliferative disorders are a previously unrecognised cause of massive tropical splenomegaly. This finding has major implications for management of massive splenomegaly. Diagnosis of the less common causes of this disorder is usually straightforward, but differentiating between B-lymphoproliferative disorders and HMS can be difficult. HMS is associated with younger age, a higher proportion of women, and lower absolute lymphocyte counts than lymphoproliferative disorders.