Robinson R G, Adler R, Swanson V L, Williams K O, Siegel S E
Am J Pediatr Hematol Oncol. 1982 Fall;4(3):341-4.
A 19-year-old boy with congenital hypoplastic anemia (CHA) treated with long-term steroid therapy, presented with septic shock caused by Streptococcus pneumoniae. Peripheral blood smears revealed Howell-Jolly bodies and postmortem examination revealed the absence of the spleen, splenic vein and splenic artery. We have found no association of CHA and congenital asplenia previously reported in the literature or in a review of 12 additional patients with CHA at this institution. The case is reported to bring attention to the concurrence of these two rare conditions in one individual and to discuss the possible implications of this association.
一名19岁患有先天性再生障碍性贫血（CHA）的男孩，长期接受类固醇治疗，因肺炎链球菌感染引发感染性休克。外周血涂片显示有豪-乔小体，尸检发现脾脏、脾静脉和脾动脉均缺失。我们在先前的文献报道中以及对本机构另外12例CHA患者的回顾中，均未发现CHA与先天性无脾之间存在关联。报道该病例是为了引起对这两种罕见病症在同一患者身上并发情况的关注，并讨论这种关联可能产生的影响。
