Naseem Shano, Kaur Sukhpreet, Gupta Ritu, Kashyap Rajesh, Nityanand Soniya
Department of Hematology, Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGIMS), Lucknow, 226014 India.
Indian J Hematol Blood Transfus. 2012 Mar;28(1):10-4. doi: 10.1007/s12288-011-0097-8. Epub 2011 Jul 21.
Plasma cell leukemia is an unusual manifestation of multiple myeloma, reported to occur in 2% of newly diagnosed patients. It may either present at the time of diagnosis (primary) or evolve as a late feature in the course of multiple myeloma (secondary). Most clinical signs of myeloma are observed in plasma cell leukemia, although osteolytic lesions and bone pain are less frequent and lymphadenopathy, organomegaly and renal failure are more often present. The immunophenotype of plasma cell leukemia differs typically from that of myeloma by lack of aberrant CD56 expression. An abnormal karyotype is more frequently found in plasma cell leukemia and there is higher incidence of unfavourable cytogenetics. Plasma cell leukemia is an aggressive disease, characterized by a fulminant course and a short survival. We are reporting cases of this rare condition which presented at our center over 3 years along with review of literature.
浆细胞白血病是多发性骨髓瘤的一种罕见表现形式,据报道在新诊断患者中的发生率为2%。它可以在诊断时出现(原发性),也可在多发性骨髓瘤病程中作为晚期特征演变而来(继发性)。虽然溶骨性病变和骨痛较少见,而淋巴结病、器官肿大和肾衰竭更常出现,但骨髓瘤的大多数临床体征在浆细胞白血病中均可观察到。浆细胞白血病的免疫表型通常与骨髓瘤不同,表现为缺乏异常的CD56表达。浆细胞白血病中更常发现异常核型,且不良细胞遗传学的发生率更高。浆细胞白血病是一种侵袭性疾病,其特点是病程迅猛且生存期短。我们报告了过去3年在我们中心出现的这种罕见疾病的病例,并对文献进行了综述。