Lowenthal E A, Wells A, Emanuel P D, Player R, Prchal J T
Division of Hematology/Oncology, Department of Medicine, University of Alabama at Birmingham, 35294, USA.
Am J Hematol. 1996 Mar;51(3):207-13. doi: 10.1002/(SICI)1096-8652(199603)51:3<207::AID-AJH5>3.0.CO;2-0.
Acute Chest Syndrome (ACS) continues to be a major source of morbidity and mortality among patients with sickle cell disease. It is characterized by the presence of pleuritic chest pain, fever, raises on lung auscultation, and pulmonary infiltrates on chest x-ray [Castro et all: Blood 84:643-649]. The pathophysiology of this disorder remains poorly understood leading to the descriptive term "Acute Chest Syndrome" designated by Charache et al. [Arch Intern Med 139:67-69, 1979]. Typical bacterial pathogens are seldom isolated in adults, although they play a significant role in the pathogenesis of this entity in children. Until recently, the technology to accurately study viral infection as a precipitating cause of ACS has been unavailable. Parvovirus B19 is being increasingly recognized as an important human pathogen, and has been established as the cause of transient "aplastic crisis" in patients with sickle cell diseases [Saarien et al: Blood 67:-11411-11417, 1986; Young: Sem Hematol 25:159-172, 1988]. We present three patients with hemoglobin SC variant of sickle cell disease who developed ACS in association with acute parvovirus B19 infection, one of which died of respiratory failure. Parvovirus B19 infection was established by polymerase chain reaction for parvovirus B19 DNA, and the presence of parvovirus B19 specific IgM antibodies. These cases suggest that parvovirus B19 may be associated with more than self-limited illness in patients with sickle cell disease, and that this ubiquitous virus may merit further study as a precipitating cause of ACS.
急性胸综合征(ACS)仍然是镰状细胞病患者发病和死亡的主要原因。其特征为胸膜炎性胸痛、发热、肺部听诊啰音及胸部X线显示肺部浸润[卡斯特罗等人:《血液》84:643 - 649]。这种疾病的病理生理学仍知之甚少,因此才有了查拉奇等人提出的描述性术语“急性胸综合征”[《内科学文献》139:67 - 69,1979]。典型的细菌性病原体在成人中很少分离到,尽管它们在儿童该疾病的发病机制中起重要作用。直到最近,才有技术可准确研究病毒感染作为ACS的诱发原因。细小病毒B19越来越被认为是一种重要的人类病原体,并且已被确定为镰状细胞病患者短暂“再生障碍危象”的病因[萨里恩等人:《血液》67:11411 - 11417,1986;扬:《血液学专题》25:159 - 172,1988]。我们报告了3例患有镰状细胞病血红蛋白SC变异型的患者,他们在感染急性细小病毒B19后发生了ACS,其中1例死于呼吸衰竭。通过针对细小病毒B19 DNA的聚合酶链反应以及细小病毒B19特异性IgM抗体的检测确定了细小病毒B19感染。这些病例表明,细小病毒B19在镰状细胞病患者中可能导致的不仅仅是自限性疾病,这种普遍存在的病毒作为ACS的诱发原因可能值得进一步研究。