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婴儿急性巨核细胞白血病(AMKL)伴严重骨髓纤维化。经肝活检确诊及治疗反应。

Acute megakaryocytic leukemia (AMKL) with major myelofibrosis in an infant. Diagnosis by liver biopsy and response to treatment.

作者信息

Garel D, Mielot F, Gaulard P, Quillard J, Dommergues J P, Tchernia G

机构信息

Laboratory d'Hématologie, Hôpital Bicêtre, Kremlin-Bicêtre, France.

出版信息

Nouv Rev Fr Hematol (1978). 1991;33(1):5-8.

PMID:1945824
Abstract

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.

摘要

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。

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