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浆细胞白血病。我们诊治11例的诊断问题

Plasma cell leukaemia. Diagnostic problems in our experience with 11 cases.

作者信息

Toma V A, Retief F P, Potgieter G M, Anderson J D

出版信息

Acta Haematol. 1980;63(3):136-45. doi: 10.1159/000207385.

Abstract

11 patients with plasma cell leukaemia (PCL) are reported. Diagnostic clinical, haematological, immunological, biochemical and electron microscopical (TEM) data were analysed and compared to the largest series of PCL cases reported in the literature. Special attention was paid to four facets of this disease: (a) the clinical picture at admission; (b) the frequency of PCL; (c) the production of M components in relation to the maturity and type of the asynchronous plasma cells, and (d) the diagnostic problems of this entity of acute leukaemia of the afferent limb of the B lymphocyte transformation. In this series PCL emerges as a distinct clinical entity: patients are severely anaemic, hepatosplenomegaly is prominent, bone lessions are uncommun, but if present are usually non-osteolytic, and the response to treatment with an alkylating agent and glucocorticoid is poor. The diagnosis is difficult since the circulating plasma cells may have morphological features which only allows the diagnosis to be made after the TEM examination. If the peripheral blood of cases of acute leukaemias and immunocytic dyscrasias is routinely examined by TEM, PCL appears to be a not uncommon variant of plasma cell dyscrasia--in the present study it was 11%.

摘要

报告了11例浆细胞白血病(PCL)患者。对诊断性临床、血液学、免疫学、生化和电子显微镜(透射电镜,TEM)数据进行了分析,并与文献中报道的最大系列PCL病例进行了比较。特别关注了该疾病的四个方面:(a)入院时的临床表现;(b)PCL的发生率;(c)与不同步浆细胞的成熟度和类型相关的M成分产生情况;(d)B淋巴细胞转化传入支急性白血病这一实体的诊断问题。在本系列中,PCL表现为一种独特的临床实体:患者严重贫血,肝脾肿大明显,骨病变不常见,但如果存在通常是非溶骨性的,并且对烷化剂和糖皮质激素治疗反应较差。诊断困难,因为循环浆细胞可能具有形态学特征,只有在进行透射电镜检查后才能做出诊断。如果对急性白血病和免疫细胞发育异常病例的外周血常规进行透射电镜检查,PCL似乎是浆细胞发育异常中一种并不罕见的变异型——在本研究中其发生率为11%。

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