de Mol C L, Hermans M A W, Gerth van Wijk R, van Hagen P M, van Daele P L A
a Department of Internal Medicine, Section of Clinical Immunology , Erasmus University Medical Centre , Rotterdam , Netherlands.
b Department of Internal Medicine, Section of Allergology , Erasmus University Medical Centre , Rotterdam , Netherlands.
Hematology. 2017 Oct;22(9):544-547. doi: 10.1080/10245332.2017.1324377. Epub 2017 May 10.
Systemic mastocytosis (SM) is a myeloproliferative disease characterized by the accumulation of aberrant mast cells. Since advanced subtypes of SM can lead to organ dysfunction and shortened survival, timely recognition of progressive disease is important for the adequate treatment of SM patients.
Here, we report the results of our cohort study on the value of routine abdominal ultrasonography for the detection of progression of indolent systemic mastocytosis (ISM).
We included 88 patients with ISM, of whom 9 developed new hepatosplenomegaly during follow-up. In this group, the median serum tryptase level increased by 11.60 μg/l, compared with a decrease of -0.20 μg/l in the 79 patients with unchanged ultrasonography results (p = 0.016). A change in liver and/or spleen size never led to a change in clinical classification, nor management.
Based on the finding that a change in ultrasonography findings did not correlate to disease progression in general, it appears that isolated hepatosplenomegaly does not have prognostic implications in patients with ISM.
Routine abdominal ultrasonography is redundant in the follow-up of patients with ISM. A combination of physical examination with serum tryptase levels can be used to screen for hepatosplenomegaly.
系统性肥大细胞增多症(SM)是一种骨髓增殖性疾病,其特征为异常肥大细胞的积聚。由于SM的晚期亚型可导致器官功能障碍并缩短生存期,因此及时识别疾病进展对于SM患者的充分治疗至关重要。
在此,我们报告了关于常规腹部超声检查对惰性系统性肥大细胞增多症(ISM)病情进展检测价值的队列研究结果。
我们纳入了88例ISM患者,其中9例在随访期间出现了新的肝脾肿大。在这组患者中,血清类胰蛋白酶水平中位数升高了11.60μg/l,而超声检查结果未改变的79例患者血清类胰蛋白酶水平中位数下降了-0.20μg/l(p = 0.016)。肝脏和/或脾脏大小的改变从未导致临床分类或治疗的改变。
基于超声检查结果的改变通常与疾病进展不相关这一发现,看来孤立性肝脾肿大对ISM患者没有预后意义。
在ISM患者的随访中,常规腹部超声检查是多余的。体格检查与血清类胰蛋白酶水平相结合可用于筛查肝脾肿大。