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[一名急性巨核细胞白血病患者的股骨粒细胞肉瘤]

[Granulocytic sarcoma of the femur in a patient with acute megakaryoblastic leukaemia].

作者信息

Čolović Nataša, Bogdanović Andrija, Vidović Ana, Perunčić-Jovanović Maja, Čolović Milica

出版信息

Srp Arh Celok Lek. 2011 Nov-Dec;139(11-12):805-8. doi: 10.2298/sarh1112805c.

Abstract

INTRODUCTION

Granulocytic sarcoma, chloroma or myeloblastoma are observed in 3% to 7% of acute myeloid leukaemia and represents localized tumour composed of collection of immature leukaemic cells. It appears most frequently in patients with M2, M4 and M5 subtypes of acute myeloid leukaemia

CASE OUTLINE

A 58-year-old female presented with pain and oedema of the right upper limb in November 2009. After two months the patinet had fracture dislocation and numerous osteolytic lesions of the right femur. Immunohistochemistry of tumour biopsy showed megakaryoblastic granulocytic sarcoma which was CD31++, F-XIII++, CD34-, FVIII+++, S100-, aktin-, EMA++, Bcl2++, CD43++, with positive proliferative marker measured with Ki-67 positivity in more of 50% of cells. Aspirate of bone marrow and immunophenotyping with flowcytometry revealed diagnosis of acute megakaryoblastic leukaemia. The course of the disease was rapid and the patient died before commencing chemotherapy, five months after first complaints.

CONCLUSION

Granulocytic sarcoma is extramedullary localization of collection of leukaemia cells which can proceed, to arise concomitantly with leukaemia, or may be the only manifestation of the disease. The diagnosis can be established only with immunohystochemistry.

摘要

引言

粒细胞肉瘤、绿色瘤或成髓细胞瘤在3%至7%的急性髓系白血病患者中可见,是由不成熟白血病细胞聚集形成的局限性肿瘤。它最常出现在急性髓系白血病M2、M4和M5亚型的患者中。

病例概述

一名58岁女性于2009年11月出现右上肢疼痛和水肿。两个月后,患者出现右股骨骨折脱位及多处溶骨性病变。肿瘤活检的免疫组化显示为巨核细胞性粒细胞肉瘤,其表现为CD31++、F-XIII++、CD34-、FVIII+++、S100-、肌动蛋白-、EMA++、Bcl2++、CD43++,增殖标志物Ki-67阳性率在50%以上的细胞中呈阳性。骨髓穿刺及流式细胞仪免疫表型分析显示诊断为急性巨核细胞白血病。疾病进展迅速,患者在首次出现症状五个月后,在开始化疗前死亡。

结论

粒细胞肉瘤是白血病细胞聚集的髓外定位,可先于白血病出现、与白血病同时发生,或可能是该疾病的唯一表现形式。只有通过免疫组化才能确诊。

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