Altura R A, Head D R, Wang W C
Department of Hematology/Oncology, Columbus Children's Hospital, Columbus, OH 43205, USA.
Br J Haematol. 2000 May;109(2):459-62. doi: 10.1046/j.1365-2141.2000.01977.x.
Idiopathic myelofibrosis can develop in children as well as adults. However, the disease appears to be much more aggressive in adults, being characterized by poor survival rates and a high frequency of malignant transformation. Here, we describe three cases of idiopathic myelofibrosis in infants, two of whom were followed for 16 and 22 years after diagnosis. Neither of these patients required more than minimal supportive care, and both have had spontaneous erythropoietic recovery as early as 2-3 years after diagnosis. There have been no indications of malignant transformation or clinical deterioration. Thus, idiopathic myelofibrosis may have a different pathogenesis and clinical course in infants from adults, requiring a more conservative approach to management.
特发性骨髓纤维化可发生于儿童和成人。然而，该疾病在成人中似乎更具侵袭性，其特征为生存率低和恶性转化频率高。在此，我们描述了3例婴儿特发性骨髓纤维化病例，其中2例在诊断后分别随访了16年和22年。这2例患者均仅需极少的支持治疗，且均在诊断后2 - 3年即出现自发性红细胞生成恢复。均无恶性转化或临床恶化的迹象。因此，婴儿特发性骨髓纤维化的发病机制和临床过程可能与成人不同，需要采取更保守的管理方法。
