Riehl J, Schmitt H, Bergmann D, Fritz A, Sieberth H G
Medizinische Klinik II, Klinikums der Rheinisch-Westfälischen Technischen Hochschule Aachen.
Dtsch Med Wochenschr. 1996 Nov 1;121(44):1354, 1355-8. doi: 10.1055/s-2008-1043152.
A 17-year-old girl from Zaire was admitted to hospital with fever, cough, dyspnoea and severe chest pain. In addition to marked anaemia (haemoglobin 6.6 g/dl) she was known to have cavitary/exudative pulmonary tuberculosis (PTb) with bilateral basal infiltrations.
Blood gas analysis indicated partial respiratory failure (pO2 55 mm Hg, pCO2 36 mm Hg). Blood smear under air exclusion showed erythrocyte sickling. Haemoglobin electrophoresis demonstrated 92.7% HbS and thus confirmed sickle cell anaemia. A small spleen on sonography and the presence of Howell-Jolly bodies were interpreted as signs of functional asplenia. Microbiological and radiological tests confirmed exudative-cavitary PTb.
The findings were interpreted as due to an acute chest syndrome, caused by sickle cell thrombi in the pulmonary blood vessels, precipitated by the PTb. Transfusion of two units of erythrocyte concentrates led to improvement of the chest pain and the respiratory failure within a few hours. The PTb was successfully treated without any complications.
Acute chest syndrome is a vascular occlusive complication of sickle cell disease, pulmonary tuberculosis precipitating the development of this acute condition. Administration of erythrocyte concentrate rapidly improves the signs and symptoms.
一名来自扎伊尔的17岁女孩因发热、咳嗽、呼吸困难及严重胸痛入院。除显著贫血(血红蛋白6.6g/dl)外,已知她患有空洞/渗出性肺结核(PTb),双侧肺底部有浸润。
血气分析提示部分呼吸衰竭(pO2 55mmHg,pCO2 36mmHg)。在无氧条件下进行的血涂片检查显示红细胞镰变。血红蛋白电泳显示HbS占92.7%,从而确诊为镰状细胞贫血。超声检查发现脾脏较小且存在豪-焦小体,被解释为功能性无脾的征象。微生物学和放射学检查证实为渗出性空洞型PTb。
这些发现被解释为由急性胸部综合征所致,该综合征由肺血管中的镰状细胞血栓引起,PTb为其诱发因素。输注两单位红细胞浓缩液后,胸痛和呼吸衰竭在数小时内得到改善。PTb得到成功治疗,无任何并发症。
急性胸部综合征是镰状细胞病的一种血管闭塞性并发症,肺结核可促使这种急性病症的发生。输注红细胞浓缩液可迅速改善症状和体征。