Asare George Awuku, Amin Coker, Fiador Kate, Hagbevor Israel, Tsede Ernest, Ampofo Blessing, Mensah Benjamin
Department of Medical Laboratory Sciences, College of Health Sciences University of Ghana Accra Ghana.
Bone Marrow Transplant (BMT) Unit Accra Ghana.
Clin Case Rep. 2025 May 7;13(5):e70483. doi: 10.1002/ccr3.70483. eCollection 2025 May.
The most prevalent genetic disease in Africa and sub-Saharan Africa is sickle cell disease. Crisis often leads to severe pain in the limbs and chest region accompanied by oxygen deprivation to tissues. In children and adults, acute chest syndrome (ACS) which is a complication of vaso-occlusive crisis (VOC), is a life-threatening condition. A 24-year-old female with known Sickle Cell Disease (SCD) complained of generalized diffuse chest pains and was administered diclofenac at a peripheral hospital. The pains subsided. However, the condition relapsed and at another clinic, she was treated with . paracetamol, morphine, pethidine, and . fluids. Three (3) days had already gone by since the crisis started before a further transfer was made to the Greater Accra Regional Hospital. On arrival, she was diagnosed with chest and leg pains, shortness of breath, and VOC. Chest x-ray demonstrated patchy consolidation, infiltrates bilaterally, with right atrial enlargement. Clexane 80 mg BD, Omeprazole 40 mg ., Azithromycin tablets 500 mg, morphine syrup 10 mg/5 mL, Injection Rocephin 2 g, and infusion paracetamol 1 g were administered, followed by a continuous treatment review plan. Oxygen saturation was however generally constant at 99%. On day five (post admission-D5) the patient was moaning and restless. Her WBC started increasing [5.9-10.1 × 10/μL (D5-D9)], platelets were still low (45 × 10/μL), with Hb showing signs of fluctuations [6.3-9.0 g/dL (D2-D9)]. Blood culture results showed Gram-positive bacillus. Acute phase reactants, CRP (103.65 mg/dL) and ferritin (741.3 μg/L) were very high. RBC exchange transfusion by apheresis commenced at 10 pm (for 3 h). By 6 am the following day, the patient was described as having a calm night. Further drug modifications were made and by day 9 (D9) patient was only on Cap Omeprazole 20 mg once for 5 days, and tab dexamethasone 6 mg daily. The patient was discharged on day 11 post admission. Thus, from the onset of the crisis to the application of apheresis, 8 days had elapsed. However, five (5) hours after the RBC exchange transfusion by apheresis, pains subsided considerably. The key clinical message is that prompt resolution of ACS by therapeutic apheresis application will subsequently reduce the long-term pulmonary complications in SCD patients, hospitalization time, the overall financial burden, anxiety, and discomfort to patient and family.
镰状细胞病是非洲和撒哈拉以南非洲最普遍的遗传疾病。危机常常导致四肢和胸部剧烈疼痛，并伴有组织缺氧。在儿童和成人中，作为血管闭塞性危机（VOC）并发症的急性胸部综合征（ACS）是一种危及生命的病症。一名已知患有镰状细胞病（SCD）的24岁女性主诉全身弥漫性胸痛，在外围医院接受了双氯芬酸治疗。疼痛缓解。然而，病情复发，在另一家诊所，她接受了对乙酰氨基酚、吗啡、哌替啶和补液治疗。自危机开始已经过去了三天，之后她才被进一步转至大阿克拉地区医院。到达后，她被诊断为胸痛、腿痛、呼吸急促和VOC。胸部X光显示有斑片状实变、双侧浸润以及右心房扩大。给予克赛80毫克每日两次、奥美拉唑40毫克、阿奇霉素片500毫克、吗啡糖浆10毫克/5毫升、罗氏芬注射液2克以及对乙酰氨基酚输液1克，随后制定持续的治疗复查计划。然而，血氧饱和度总体上一直稳定在99%。在入院第五天（D5），患者呻吟且烦躁不安。她的白细胞开始增加[5.9 - 10.1×10/微升（D5 - D9）]，血小板仍然很低（45×10/微升），血红蛋白显示有波动迹象[6.3 - 9.0克/分升（D2 - D9）]。血培养结果显示革兰氏阳性杆菌。急性期反应物，CRP（103.65毫克/分升）和铁蛋白（741.3微克/升）非常高。晚上10点开始通过单采进行红细胞置换输血（持续3小时）。到第二天早上6点，患者被描述为度过了平静的一晚。进一步调整了药物，到第9天（D9），患者仅服用奥美拉唑胶囊20毫克，每日一次，共5天，以及地塞米松片6毫克每日一次。患者在入院后第11天出院。因此，从危机开始到应用单采，已经过去了8天。然而，通过单采进行红细胞置换输血5小时后，疼痛明显减轻。关键的临床信息是，通过治疗性单采迅速解决ACS将随后减少SCD患者的长期肺部并发症、住院时间、总体经济负担、焦虑以及患者和家属的不适。
