Sharma Saniya, Sachdeva Man Updesh Singh, Rastogi Pulkit, Prabhakar Nidhi
Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012 India.
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012 India.
Indian J Hematol Blood Transfus. 2022 Apr;38(2):432-433. doi: 10.1007/s12288-021-01501-1. Epub 2021 Oct 27.
A 40-year-old female presented with progressive fatigue and left upper abdominal discomfort for 1 month. CT scan revealed ill-defined hypervascular lesions in the liver and diffuse enlargement of the spleen. She had severe anemia with leukoerythroblastic reaction. Bone marrow smears revealed clusters and discrete spindle cells, few plump and round cells. Trephine biopsy revealed an infiltrative spindle-cell neoplasm replacing the marrow with the focal presence of epithelioid cell clusters. On immunostaining, the tumor cells were positive for CD34, CD31, Friend Leukemia Virus Integration-1 (FLI1), and vimentin, consistent with metastatic angiosarcoma. There were multiple small aggregates of 5-10 MIB-1 positive tumor cells (overall positivity < 1%). She succumbed to her illness before initiation of any therapy. Bone marrow metastasis of angiosarcoma is extremely rare. The aggressive clinical course and judicious use of immunohistochemical markers are required for definitive diagnosis.
一名40岁女性因进行性疲劳和左上腹不适1个月前来就诊。CT扫描显示肝脏有边界不清的高血管病变，脾脏弥漫性肿大。她患有严重贫血并伴有幼稚粒-幼红细胞反应。骨髓涂片显示有簇状和散在的梭形细胞，少数饱满的圆形细胞。骨髓活检显示浸润性梭形细胞瘤取代骨髓，并有局灶性上皮样细胞簇存在。免疫染色显示肿瘤细胞CD34、CD31、Friend白血病病毒整合-1（FLI1）和波形蛋白呈阳性，符合转移性血管肉瘤。有多个由5-10个MIB-1阳性肿瘤细胞组成的小聚集灶（总体阳性率<1%）。她在开始任何治疗前就因病去世。血管肉瘤的骨髓转移极为罕见。明确诊断需要侵袭性的临床病程及明智地使用免疫组化标志物。
