Mukiibi J M, Nyirenda C M, Paul B, Adewuyi J O, Mzula E L B, Malata H N
Department of Haematology, University of Malawi, College of Medicine, Private Bag 360, Chichiri, Blantyre 3, Malawi.
East Afr Med J. 2003 Sep;80(9):470-5. doi: 10.4314/eamj.v80i9.8744.
To document the pattern of presenting clinical and haematological features of chronic myeloid leukaemia (CML) in central Africans and evaluate the clinical consequences of treating the disease with chemotherapy.
Prospective descriptive analysis of clinical and haematological data.
Departments of Haematology of tertiary referral centres and teaching hospitals.
Prospective clinical and haematological data were collected on 150 central Africans (90 Zimbabweans and 60 Malawians) using modern Coulter counters and standard up-to-date haematological procedures and the results analysed using predetermined criteria and the top-desk Scientific Calculator Model HP 48GX, Texas Instruments, USA.
There were 150 CML patients studied. Males predominated in a ratio of 1:5:1. The youngest patient was 10 years and the oldest 77 years with a mean +/- s.d. of 38.9 +/- 14.7 years. The peak age incidence of 47.3% occurred between 21 to 40 years. The Ph chromosome was found in 19 of the 20 patients studied. Although complaints attributed to splenic enlargement were the most common symptoms, several unusual clinical features were encountered viz: hepatomegaly (26%), bleeding (12%), significant lymphadenopathy (11.3%), purpura (3.3%), skin infiltration (2.7%), cardiac failure (2.7%) and 14.7% were diagnosed incidentally. Symptoms such as fatigue, headaches and weight loss were associated with greater degrees of leucocytosis, severe to gross splenomegaly and lower haemoglobin levels. The severe to gross splenomegaly which occurred in 68(45.3%) suggests that patients in this part of the world seek medical advice rather late in the disease. The median survival times of 65,47 and 39 months respectively for alpha interferon, hydroxyurea and busulphan are in agreement with those of previous larger series from other parts of the world.
The study has revealed that the presenting pattern of clinical and haematological features of CML is changing probably due to the advent of modern clinical practice coupled with increased physician density, greater awareness of disease among clinicians besides other reasons. However, optimal treatment is not possible for the majority of patients due to lack of chemotherapeutic agents and supportive care.
Referral centres in African health systems should be equipped for better management of CML patients.
记录中非地区慢性髓系白血病(CML)的临床和血液学特征表现模式,并评估化疗治疗该疾病的临床后果。
对临床和血液学数据进行前瞻性描述性分析。
三级转诊中心和教学医院的血液科。
使用现代库尔特计数器和标准的最新血液学程序,收集了150名中非地区患者(90名津巴布韦人和60名马拉维人)的前瞻性临床和血液学数据,并使用预定标准和美国德克萨斯仪器公司的HP 48GX型顶级桌面科学计算器对结果进行分析。
共研究了150例CML患者。男性占主导,比例为1.5:1。最年轻的患者为10岁,最年长的为77岁,平均年龄±标准差为38.9±14.7岁。47.3%的发病高峰年龄出现在21至40岁之间。在所研究的20例患者中,有19例发现了费城染色体。尽管归因于脾肿大的主诉是最常见的症状,但也遇到了一些不寻常的临床特征,即:肝肿大(26%)、出血(12%)、显著的淋巴结病(11.3%)、紫癜(3.3%)、皮肤浸润(2.7%)、心力衰竭(2.7%),14.7%为偶然诊断。疲劳、头痛和体重减轻等症状与更高程度的白细胞增多、严重至巨大脾肿大和更低的血红蛋白水平相关。68例(45.3%)出现的严重至巨大脾肿大表明,世界这一地区的患者在疾病晚期才寻求医疗建议。α干扰素、羟基脲和白消安的中位生存时间分别为65、47和39个月,与世界其他地区先前更大系列研究的结果一致。
该研究表明,CML的临床和血液学特征表现模式可能由于现代临床实践的出现、医生密度增加、临床医生对疾病的认识提高以及其他原因而正在发生变化。然而,由于缺乏化疗药物和支持治疗,大多数患者无法获得最佳治疗。
非洲卫生系统中的转诊中心应具备更好地管理CML患者的条件。