López Guillermo A, Martí J M, Bladé J, Nomdedeu B, Montserrat E, Rozman C
Sangre (Barc). 1989 Feb;34(1):28-31.
Ten patients with plasma cell leukaemia (PCL), out of 259 cases of multiple myeloma diagnosed in the Haematology Service of the University Hospital of Barcelona in the last 18 years, are presented. Of the 10 PCL cases, 5 were primary and 5 were secondary. Anaemia and thrombocytopenia, along with massive plasma cell infiltration of the bone marrow, were the most striking findings. Osteolytic lesions were present in 9 of the cases and liver involvement in two. Chemotherapy including vincristine and prednisone was administered to eight patients, associated to alkylating agents (melphalan and/or cyclophosphamide) in six of them. Four of these patients received also adriamycin and BCNU. Two objective responses were achieved, lasting for 10 and 3 months, the remaining six patients failed to respond. The median survival for all the PCL patients was less than one month (ranging between 0.2 and 14 months). None of the secondary PCL patients survived for 2 months after diagnosis. Infection (3 cases of septicaemia and 3 of pneumonia), renal failure (2 cases) and liver insufficiency (1 case) were the causes of death in the nine deceased patients. The therapeutic possibilities for this severe haemopathy are discussed.
本文报告了巴塞罗那大学医院血液科在过去18年中诊断的259例多发性骨髓瘤患者中的10例浆细胞白血病(PCL)患者。在这10例PCL病例中,5例为原发性,5例为继发性。贫血和血小板减少症,以及骨髓中大量浆细胞浸润,是最显著的发现。9例患者出现溶骨性病变,2例有肝脏受累。8例患者接受了包括长春新碱和泼尼松在内的化疗,其中6例联合使用了烷化剂(美法仑和/或环磷酰胺)。这6例患者中有4例还接受了阿霉素和卡氮芥治疗。2例患者获得客观缓解,缓解持续时间分别为10个月和3个月,其余6例患者无反应。所有PCL患者的中位生存期不到1个月(范围在0.2至14个月之间)。继发性PCL患者在诊断后均未存活2个月。感染(3例败血症和3例肺炎)、肾衰竭(2例)和肝功能不全(1例)是9例死亡患者的死因。本文还讨论了这种严重血液病的治疗可能性。