Singh Namrata, Mott Sarah L, Sutamtewagul Grerk, McCarthy Ashley, Slager Susan L, Cerhan James R, Ballas Zuhair, Link Brian K
Division of Rheumatology University of Washington Seattle Washington USA.
Holden Comprehensive Cancer Center (HCCC) University of Iowa Iowa City Iowa USA.
EJHaem. 2020 Sep 1;1(2):537-544. doi: 10.1002/jha2.95. eCollection 2020 Nov.
To examine the prevalence of hypogammaglobulinemia in chronic lymphocytic lymphoma (CLL) patients and to test the hypothesis that patients with hypogammaglobulinemia have a distinct clinical profile and outcome.
Immunoglobulin levels (IgA, IgG, IgM, IgE) were measured in newly diagnosed, treatment naïve banked samples of 150 patients with CLL followed prospectively for outcomes. Cox regression models were used to assess the effects of clinical variables on overall survival (OS).
The median age of the selected CLL cohort was 64 years with a male predominance; 96.2% of the patients were white. Fifty-nine deaths occurred during a median follow up of 6.8 years. Hypogammaglobulinemia in CLL was common in our cohort with 88 (58.7%, 95% CI: 50.4-66.6%) patients having a measurable isotype deficiency. The most common Ig deficiency was IgM (44.0%). IgA deficiency or low IgE was associated with higher Rai stages as well as with higher white blood cell counts at presentation. Any immunoglobulin deficiency was not associated with overall survival.
A significant proportion of treatment-naïve CLL patients had underlying Ig deficiencies - both in isolation and in isotype combinations. Although a deficiency of IgA or IgE was associated with more severe disease at presentation, the impact of this association was mild.
检测慢性淋巴细胞白血病(CLL)患者低丙种球蛋白血症的患病率,并验证低丙种球蛋白血症患者具有独特临床特征和预后的假设。
在150例初诊、未接受过治疗且有样本储存的CLL患者中检测免疫球蛋白水平(IgA、IgG、IgM、IgE),并对其预后进行前瞻性随访。采用Cox回归模型评估临床变量对总生存期(OS)的影响。
所选CLL队列的中位年龄为64岁,男性居多;96.2%的患者为白人。在中位随访6.8年期间有59例死亡。CLL患者中的低丙种球蛋白血症在我们的队列中很常见,88例(58.7%,95%CI:50.4 - 66.6%)患者存在可测量的同种型缺乏。最常见的Ig缺乏是IgM(44.0%)。IgA缺乏或低IgE与较高的Rai分期以及就诊时较高的白细胞计数相关。任何免疫球蛋白缺乏均与总生存期无关。
相当比例的初治CLL患者存在潜在的Ig缺乏——既有单一缺乏,也有同种型组合缺乏。虽然IgA或IgE缺乏与就诊时病情更严重相关,但这种关联的影响较轻。