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尼日利亚一家三级医疗机构中镰状细胞病患者的急性白血病:病例系列研究。

Acute leukemia in sickle cell disease patients in a tertiary health facility in Nigeria: a case series.

机构信息

Haematology Department, Central Laboratory, Synlab Nigeria Limited, Lagos.

Department of Haematology, College of Medicine, University of Ibadan, Nigeria.

出版信息

Afr Health Sci. 2020 Sep;20(3):1304-1312. doi: 10.4314/ahs.v20i3.36.

DOI:10.4314/ahs.v20i3.36
PMID:33402979
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7751539/
Abstract

BACKGROUND AND OBJECTIVES

Sickle cell disease(SCD) is a disorder of red cells resulting from the co-inheritance of haemoglobin S (HbS) with another abnormal haemoglobin. The diagnosis of acute leukaemia is uncommon in our patients with sickle cell disease more so the patients have high morbidity and mortality due to the sickling process. Acute leukemia is a malignant clonal disorder of haemopoietic precursor cells resulting in accumulation of immature blood cells in the bone marrow and blood. The objective of the case series was to highlight the challenges of diagnosis and management of SCD patients with acute leukaemia, the importance of peripheral blood film review and propound a possible risk factor.

METHODS

Records of 58 patients diagnosed and managed for acute leukaemia over a 7 year period at the University College Hospital, Ibadan were reviewed. The diagnosis of acute leukaemia was based on clinical features in addition to peripheral and bone marrow smears findings. Microsoft excel version 2013 was used for statistical analysis.

RESULTS

Five (8.6%) of the patients with acute leukaemia also had sickle cell disease: 3 males and 2 females were described. Recurrent fever and anaemia were the most consistent presenting features in the patients. All the patients were not on any routine medications meant for SCD patients and had poor history of clinic attendance prior to the diagnosis of acute leukaemia. The diagnosis of acute leukaemia was not made until the patients were seen by a haematologist. The principal tool of diagnosis in all the patients was peripheral blood film review. Two patients were discharged against medical advice. The treatment period ranged between one month and one year in the remaining three patients.

CONCLUSION

SCD patients are not exempted from developing acute leukaemias and the diagnoses of the two conditions overwhelms the social and economic support of patients and care givers. The study also underscores the relevance of high level of suspicion and prompt review of peripheral blood film of SCD patients particularly when patients present with unremitting symptoms associated with anaemia and fever.

摘要

背景与目的

镰状细胞病(SCD)是一种由血红蛋白 S(HbS)与另一种异常血红蛋白共同遗传引起的红细胞疾病。在我们的 SCD 患者中,急性白血病的诊断并不常见,更常见的是,由于镰状细胞过程,患者的发病率和死亡率很高。急性白血病是一种造血前体细胞的恶性克隆性疾病,导致骨髓和血液中不成熟血细胞的积累。本病例系列的目的是强调 SCD 合并急性白血病患者的诊断和管理挑战,外周血涂片复查的重要性,并提出可能的危险因素。

方法

回顾了在伊巴丹大学教学医院诊断和管理急性白血病的 58 例患者的记录,在过去 7 年中。急性白血病的诊断基于临床特征,以及外周和骨髓涂片的发现。使用 Microsoft excel version 2013 进行统计分析。

结果

5 例(8.6%)急性白血病患者也患有镰状细胞病:3 例男性和 2 例女性。最常见的表现为反复发热和贫血。所有患者均未服用任何常规的 SCD 药物,且在诊断为急性白血病之前就诊记录不佳。只有在患者就诊于血液科医生时,才做出急性白血病的诊断。所有患者的诊断主要工具都是外周血涂片复查。有 2 例患者拒绝接受治疗后出院。其余 3 例患者的治疗时间在 1 个月至 1 年之间。

结论

SCD 患者并非不会发生急性白血病,这两种疾病的诊断给患者及其照顾者的社会和经济支持带来了巨大的压力。本研究还强调了对 SCD 患者外周血涂片进行高度怀疑和及时复查的重要性,特别是当患者出现与贫血和发热相关的持续症状时。

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