Atypical blast morphology of primary plasma cell leukemia with renal involvement and plasmablasts in urine
Atypical blast morphology of primary plasma cell leukemia with renal involvement and plasmablasts in urine
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肾脏受累的原发性浆细胞白血病的非典型母细胞形态和尿液中的浆母细胞
DOI：
10.1002/dc.23168
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发表时间：
2015
影响因子：
1.3
通讯作者：
Ika Kardum Skelin
中科院分区：
文献类型：
--
作者：
Antonia Pavlovic;S. Ostojić Kolonić;D. Radić Krišto;Biljana Jelić Puškarić;Ika Kardum Skelin
原发性浆细胞白血病（PCL）是浆细胞（PC）骨髓瘤的一种罕见的侵袭性变体，其特征在于循环中高水平的PC。临床表现与其他急性白血病相似，常见的并发症是各种组织和器官的髓外浸润。该病病程暴发，预后差。PCL中PC的形态包括一系列成熟，大多数病例具有淋巴浆细胞样或浆母细胞样形态。呈现为不类似PC的更原始的细胞是更罕见的，需要额外的形态学和免疫表型研究。我们报告一例79岁女性，因严重全血细胞减少和大叶性肺炎就诊。实验室和临床评价显示原发性非分泌性PCL伴非典型、不成熟的原始细胞形态、髓外肾受累和尿液中的浆母细胞。尽管做出了治疗努力，患者还是死于这种疾病。尿中PC的检测表明疾病的髓外扩散，特别是没有伴随血尿，并可能导致肾功能损害，这已经是这些患者的常见并发症。细胞病理学诊断。2015;43：158-162.© 2014 Wiley Periodicals，Inc.
Primary plasma cell leukemia (PCL) is a rare and aggressive variant of plasma cell (PC) myeloma characterized by high levels of circulating PCs. Clinical presentation is like other acute leukemia, with extramedullary infiltration of various tissues and organs being a frequent complication. The disease has a fulminant course and poor prognosis. Morphology of PCs in PCL includes a spectrum of maturity, with most cases having lymphoplasmacytoid or plasmablastic morphology. Presentation as more primitive cells that do not resemble PCs is even rarer and requires additional morphological and immunophenotypic studies. We present a case of 79‐year‐old woman who presented with severe pancytopenia and lobar pneumonia. Laboratory and clinical evaluation revealed primary, nonsecretory PCL with atypical, immature blast morphology, extramedullary renal involvement, and plasmablasts in urine. Despite therapeutic efforts the patient succumbed to the disease. Detection of PCs in the urine indicates extramedullary spread of disease, especially without accompanying hematuria, and may contribute to impairment of renal function, what is already a frequent complication in these patients. Diagn. Cytopathol. 2015;43:158–162. © 2014 Wiley Periodicals, Inc.