Department of Leukemia.
Blood. 2014 May 1;123(18):2783-90. doi: 10.1182/blood-2013-11-536169. Epub 2014 Mar 10.
Richter syndrome (RS) is associated with poor outcome. The prognosis of patients with histologically aggressive chronic lymphocytic leukemia (CLL), or HAC, has not been studied. We aimed to correlate 2-deoxy-2-[(18)F]fluoroglucose/positron emission tomography (FDG/PET) data, histological diagnosis, clinical characteristics, and survival in patients with CLL. A total of 332 patients with CLL were histologically classified as: 95 RS, 117 HAC, and 120 histologically indolent CLL (HIC). HAC and RS patients had higher maximum standardized uptake value (SUVmax), more frequent constitutional symptoms, poorer performance status (PS), lower hemoglobin and platelets, and higher lactate dehydrogenase and β-2-microglobulin. An SUVmax ≥10 strongly correlated with mortality (overall survival [OS], 56.7 vs 6.9 months in patients with SUVmax <10 vs ≥10). Survival of patients with RS and HAC was similar among patients with SUVmax <10 or ≥10. SUVmax ≥10, PS ≥2, bulky disease, and age ≥65 were independently associated with shorter OS. In patients undergoing both fine-needle aspiration and biopsy, the former proved diagnostically inadequate in 23%, 29%, and 53% of HIC, HAC, and RS, respectively. FDG/PET is a useful diagnostic tool in patients with CLL and suspected transformation. Patients with HAC show different characteristics and worse prognosis compared with those with HIC. Patients with different CLL phases, but similar SUVmax have similar outcome. Tissue biopsy should be preferred for diagnosing RS.
里希特综合征(RS)与不良预后相关。尚未研究组织学侵袭性慢性淋巴细胞白血病(CLL)或 HAC 患者的预后。我们旨在将 2-脱氧-2-[[18]F]氟葡萄糖/正电子发射断层扫描(FDG/PET)数据、组织学诊断、临床特征和 CLL 患者的生存情况进行关联。共对 332 例 CLL 患者进行了组织学分类:95 例 RS、117 例 HAC 和 120 例组织学惰性 CLL(HIC)。HAC 和 RS 患者的最大标准化摄取值(SUVmax)更高,更频繁出现全身症状,体能状态(PS)更差,血红蛋白和血小板水平更低,乳酸脱氢酶和β-2-微球蛋白水平更高。SUVmax≥10 与死亡率(总生存期 [OS],SUVmax<10 的患者为 56.7 个月,SUVmax≥10 的患者为 6.9 个月)强烈相关。SUVmax<10 或≥10 的 RS 和 HAC 患者的生存情况相似。SUVmax≥10、PS≥2、肿块疾病和年龄≥65 与较短的 OS 独立相关。在同时进行细针抽吸和活检的患者中,前者在 HIC、HAC 和 RS 患者中的诊断分别不足 23%、29%和 53%。FDG/PET 是疑似转化的 CLL 患者的有用诊断工具。与 HIC 相比,HAC 患者表现出不同的特征和更差的预后。不同 CLL 阶段但 SUVmax 相似的患者具有相似的结果。组织活检应优先用于诊断 RS。