Lane P A, O'Connell J L, Lear J L, Rogers Z R, Woods G M, Hassell K L, Wethers D L, Luckey D W, Buchanan G R
Colorado Sickle Cell Treatment and Research Center, University of Colorado School of Medicine, Denver, USA.
Blood. 1995 Apr 15;85(8):2238-44.
The incidence of functional asplenia in sickle-hemoglobin C (SC) disease has not been defined, and the use of prophylactic penicillin to prevent life-threatening septicemia in this disorder is controversial. The percentage of red blood cells with pits (pit count) is a reliable assay of splenic function in other disorders but has not been validated in hemoglobin SC disease. To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease. Baseline clinical data were recorded, and red blood cell pit counts were performed on 201 subjects, aged 6 months to 90 years, with hemoglobin SC; 43 subjects underwent radionuclide liver-spleen scanning. Pit counts greater than 20% were associated with functional asplenia as assessed by liver-spleen scan, whereas pit counts less than 20% were found in subjects with preserved splenic function. Pit counts greater than 20% were present in 0 of 59 subjects (0%) less than 4 years of age, in 19 of 86 subjects (22%) 4 to 12 years of age, and in 25 of 56 subjects (45%) greater than 12 years of age. Other subjects with hemoglobin SC, who had previously undergone surgical splenectomy, had higher pit counts (59.7% +/- 9.5%) than splenectomized subjects without hemoglobinopathy (38.5% +/- 8.8%) or with sickle cell anemia (20.5% +/- 1.9%; P < .001). Two subjects with hemoglobin SC disease (not splenectomized), ages 14 and 15 years, with pit counts of 40.3% and 41.7% died from pneumococcal septicemia. These data indicate that functional asplenia occurs in many patients with hemoglobin SC disease, but its development is usually delayed until after 4 years of age. The pit count is a reliable measure of splenic function in hemoglobin SC disease, but values indicative of functional asplenia (> 20% in our laboratory) are higher than in other disorders. The routine administration of prophylactic penicillin to infants and young children with hemoglobin SC disease may not be necessary.
镰状血红蛋白C（SC）病患者功能性无脾的发生率尚未明确，在这种疾病中使用预防性青霉素预防危及生命的败血症存在争议。有凹陷的红细胞百分比（凹陷计数）在其他疾病中是脾功能的可靠检测方法，但尚未在血红蛋白SC病中得到验证。为了解决这些问题，我们对血红蛋白SC病患者的脾功能进行了一项前瞻性、多中心研究。记录了基线临床数据，并对201名年龄在6个月至90岁之间的血红蛋白SC患者进行了红细胞凹陷计数；43名受试者接受了放射性核素肝脾扫描。凹陷计数大于20%与肝脾扫描评估的功能性无脾相关，而凹陷计数小于20%见于脾功能保留的受试者。59名4岁以下受试者中0名（0%）凹陷计数大于20%，86名4至12岁受试者中有19名（22%），56名12岁以上受试者中有25名（45%）。其他患有血红蛋白SC病且先前接受过手术脾切除术的受试者凹陷计数（59.7%±9.5%）高于无血红蛋白病的脾切除受试者（38.5%±8.8%）或镰状细胞贫血患者（20.5%±1.9%；P<0.001）。两名患有血红蛋白SC病（未脾切除）的受试者，年龄分别为14岁和15岁，凹陷计数分别为40.3%和41.7%，死于肺炎球菌败血症。这些数据表明，许多血红蛋白SC病患者存在功能性无脾，但其发展通常延迟至4岁以后。凹陷计数是血红蛋白SC病脾功能的可靠指标，但提示功能性无脾的值（在我们实验室中>20%）高于其他疾病。对患有血红蛋白SC病的婴幼儿常规使用预防性青霉素可能没有必要。
