Garel D, Mielot F, Gaulard P, Quillard J, Dommergues J P, Tchernia G
Laboratory d'Hématologie, Hôpital Bicêtre, Kremlin-Bicêtre, France.
Nouv Rev Fr Hematol (1978). 1991;33(1):5-8.
An infant presented with enlarged liver and spleen and profound pancytopenia. Severe marrow fibrosis impeded the diagnosis which was achieved by liver biopsy: Fibrosis surrounded foci of blasts which were shown by immunologic markers to originate from the megakaryocytic lineage. Complete sustained remission was obtained by low-dose aracytine therapy. The child was disease-free at 40 months' follow up.
一名婴儿出现肝脏和脾脏肿大以及严重全血细胞减少。严重的骨髓纤维化妨碍了诊断,最终通过肝脏活检得以确诊:纤维化围绕着原始细胞灶,免疫标记显示这些原始细胞起源于巨核细胞系。通过低剂量阿糖胞苷治疗获得了完全持续缓解。在40个月的随访中,该患儿无病生存。