巨核母细胞分化的粒细胞肉瘤并发慢性特发性骨髓纤维化

Granulocytic sarcoma of megakaryoblastic differentiation complicating chronic idiopathic myelofibrosis.

作者信息

Chan A C, Kwong Y L, Lam C C

机构信息

Department of Pathology, Queen Mary Hospital, Hong Kong.

出版信息

Hum Pathol. 1996 Apr;27(4):417-20. doi: 10.1016/s0046-8177(96)90116-1.

DOI:10.1016/s0046-8177(96)90116-1

PMID:8617486

Abstract

We report the development of soft tissue granulocytic sarcoma with megakaryoblastic differentiation in a patient with chronic idiopathic myelofibrosis, which has hitherto been undescribed. Although an artifactual signet ring appearance of the blasts was found in the formalin-fixed tissue and caused diagnostic problems, the histological appearance on B-5 fixed material and immunophenotyping confirmed the diagnosis. Cytogenetic analysis showed 47,XY,+der(1)del(1)(?p13) at initial presentation and 47,XY,del(1)(?13),+der(1)del(1)(?p13) at the time of soft tissue blastic transformation, indicating that partial trisomy 1 might be of pathogenetic significance.

摘要

我们报告了1例慢性特发性骨髓纤维化患者发生的伴有巨核母细胞分化的软组织粒细胞肉瘤，此前尚无此类病例的描述。尽管在福尔马林固定组织中发现原始细胞呈假的印戒样外观并导致诊断困难，但B-5固定材料的组织学表现及免疫表型分析证实了诊断。细胞遗传学分析显示，初诊时为47,XY,+der(1)del(1)(?p13)，软组织原始细胞转化时为47,XY,del(1)(?13),+der(1)del(1)(?p13)，提示1号染色体部分三体可能具有致病意义。