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病理报告滤泡性淋巴瘤1-2级
就诊医院北京协和
目前状态康复10-20年
最后登录2024-11-21
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发表于 2024-1-22 09:28:39
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来自: 中国北京
https://doi.org/10.1002/ajh.22171
Although the coexistence of chronic lymphocytic leukemia (CLL) and myeloproliferative neoplasms (MPN) has been sporadically reported in the literature, no systematic studies on this disease association are available. We retrospectively analyzed 46 patients affected by CLL/MPN referred by 15 Italian GIMEMA centers. The aim of this retrospective multicenter study was to define the following: clinico-biological characteristics, possible familiarity, clinical course of both diseases, and influence of MPN chemotherapy on the course of CLL. Among 46 patients, 30 patients were males, 16 patients were females; median age was 71 years. Only one case had familiar CLL. Myeloproliferative disorders consisted of essential thrombocytemia in 18 cases, polycythemia vera in 10 cases, chronic myeloid leukemia in 9 cases, primary myelofibrosis in 6 cases, and MPN/myelodysplastic syndrome in 3 cases. The lymphoproliferative disorder was diagnosed as monoclonal B-cell lymphocytosis in 8 patients and as Binet Stage A CLL in 38 patients. After a median follow-up of 49 months, 9 patients experienced progressive CLL and only 6 patients required treatment after a median of 57.5 months. The biological profile confirmed a subset of low-risk CLL. Twenty patients received chemotherapy for MPN without influence on the course of CLL: lymphocyte counts remained unchanged after 3, 6, and 12 months of treatment. This series is the largest so far reported in literature. The diagnosis of concomitant CLL/MPN is a rare event and lymphoproliferative disorders present a clinical indolent course with a low-risk biological profile. MPN therapy does not interfere with the prognosis of patients with CLL. Am. J. Hematol. 2011. © 2011 Wiley-Liss, Inc.
尽管文献中偶尔报道慢性淋巴细胞白血病(CLL)和骨髓增生性肿瘤(MPN)共存,但尚未对这种疾病的相关性进行系统研究。我们回顾性分析了15个意大利GIMEMA中心转诊的46例CLL/MPN患者。这项回顾性多中心研究的目的是定义以下内容:临床生物学特征、可能的关联、两种疾病的临床病程以及MPN化疗对CLL病程的影响。46例患者中,男性30例,女性16例;中位年龄71岁。只有一个病例有CLL家族史。骨髓增生性疾病包括18例原发性血小板增多症、10例真性红细胞增多症、9例慢性粒细胞白血病、6例原发骨髓纤维化和3例MPN/骨髓增生异常综合征。淋巴增生性疾病在8例患者中被诊断为单克隆B细胞淋巴细胞增多症,在38例患者中诊断为Binet A期CLL。中位随访49个月后,9名患者出现进展性CLL,中位随访57.5个月后只有6名患者需要治疗。生物学特征证实了低风险CLL的一个子集。20名患者在不影响CLL进程的情况下接受了MPN化疗:治疗3、6和12个月后,淋巴细胞计数保持不变。这个系列是迄今为止文献报道的最大的一个。伴发CLL/MPN的诊断是一种罕见事件,淋巴增生性疾病表现为低风险生物学特征的临床惰性过程。MPN治疗不影响CLL患者的预后。Am.J.血液学。2011年。©2011 Wiley-Liss, Inc. |
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