Zoubek A, Haas O A, Ladenstein R, Gadner H, Radaszkiewicz T
St. Anna Kinderspital, Vienna, Austria.
Pediatr Hematol Oncol. 1986;3(2):135-42. doi: 10.3109/08880018609031209.
We report the case history of a 6 1/2-month-old girl with a hemophagocytic syndrome, pancytopenia, and excessive hepatosplenomegaly. Some extraordinary histological features present in this case--restricted organ involvement, excessive hemosiderosis, and fibrosis of the spleen--further contributed to the well-known problem of distinguishing between infection-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis.
我们报告了一名6个半月大患有噬血细胞综合征、全血细胞减少和肝脾肿大的女孩的病例史。该病例中出现的一些特殊组织学特征——器官受累受限、铁沉积过多和脾脏纤维化——进一步加剧了区分感染相关性噬血细胞综合征和家族性噬血细胞性淋巴组织细胞增生症这一众所周知的难题。
