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NEJM:浆细胞瘤和浆细胞白血病 -案例报道

2017-03-16 xing.T MedSci原创

浆细胞性白血病是一种罕见且高度侵袭性的浆细胞疾病,可表现为原发性疾病或多发性骨髓瘤的进展或复发。

患者为一名一个63岁的男性,患有IgGλ多发性骨髓瘤,因进行性无力和疲劳而就诊。4年前,他被诊断为IgGλ多发性骨髓瘤,并接受了多种治疗方案。

体格检查发现多个外生性皮下病变主要累及四肢(图A显示右手臂)和胸壁(图B)。一个病灶活检结果显示与浆细胞瘤相一致。



实验室检查显示白细胞计数为33500每立方毫米(参考范围为4000-11000),血红蛋白水平为7.1g/dL(参考范围为13.5-18),血小板计数为11000个每立方毫米(参考范围为150000-400000),肌酐水平为1.6mg/dL(140 umol/L;参考范围为0.7-1.5 mg/dL [60-130 umol/L]),并且钙水平为13.2 mg/dL(3.30 mmol/L;参考范围为8.4到10.5 mg/dL [2.10-2.62 mmol/L])。

外周血涂片显示浆细胞占25%(如图C,Wright-Giemsa 染色),诊断为浆细胞性白血病。浆细胞性白血病是一种罕见且高度侵袭性的浆细胞疾病,可表现为原发性疾病或多发性骨髓瘤的进展或复发。

该病人无法接受进一步的浆细胞白血病治疗,由于出现了重症脓毒症合并多器官功能衰竭。病人在ICU支持治疗几天后,决定采取舒适护理措施,病人在几天内就死亡了。

原始出处:

Uqba Khan, et al. Plasmacytomas and Plasma-Cell Leukemia.N Engl J Med 2017; 376:e19March 16, 2017DOI: 10.1056/NEJMicm1611205

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