Kors J W, Van Doormaal J J, Breukelman H, Van Voorst Vader P C, De Monchy J G
Department of Endocrinology, University Hospital, Groningen, The Netherlands.
J Intern Med. 1996 Feb;239(2):157-64. doi: 10.1046/j.1365-2796.1996.440787000.x.
To evaluate the natural course of indolent mastocytosis in adults.
A retrospective long-term follow-up study.
The Department of Endocrinology of a University Hospital.
Sixteen adult patients with a diagnosis of indolent mastocytosis and sufficient biochemical data for statistical analysis. One patient had paediatric-onset cutaneous mastocytosis, whilst the others had adult-onset systemic mastocytosis. Ages at the end of follow-up ranged from 23 to 79, median 50 years. Follow-up periods per patient lasted from 13 to 135 months, median 90 months.
Urinary excretions of the histamine metabolites N tau-methylhistamine (MH) and N tau-methylimidazoleacetic acid (MIMA), and signs and symptoms of the disease.
The excretion of MH but not MIMA increased in four patients (ages 37, 45, 61 and 65 years) and decreased in two patients (ages 26 and 48 years), including the only patient with paediatric-onset cutaneous mastocytosis. The excretion of MIMA but not MH increased in none and decreased in one patients (age 51 years). The excretions of both MH and MIMA increased in one patient (age 23 years) and decreased in two patients (ages 65 and 79 years). The excretion of MH and MIMA can be considered to have been stable in one patient (age 49 years). In the five remaining patients, observation periods were rather short. A definite judgement on the course of their disease could not be given. In the two patients in whom the excretion of both MH and MIMA decreased, the enlarged spleen decreased in size, whilst in the other patients, signs and symptoms did not change. There were no accompanying myeloproliferative disorders in any patient. No special treatment aiming at a reduction in mast cell load has been given. Rates of change over the whole follow-up period ranged from -8.4 to +25.1% per year.
The natural course of indolent adult-onset mastocytosis is not always progressive. Our data show that the activity of adult-onset indolent mastocytosis, as measured by urinary excretion of MH and MIMA and clinical signs and symptoms, can substantially decline, especially in older patients.
评估成人惰性肥大细胞增多症的自然病程。
一项回顾性长期随访研究。
一所大学医院的内分泌科。
16例诊断为惰性肥大细胞增多症且有足够生化数据用于统计分析的成年患者。1例患者为儿童期起病的皮肤肥大细胞增多症，其余患者为成人期起病的系统性肥大细胞增多症。随访结束时年龄范围为23至79岁，中位数为50岁。每位患者的随访期为13至135个月，中位数为90个月。
组胺代谢产物N-τ-甲基组胺（MH）和N-τ-甲基咪唑乙酸（MIMA）的尿排泄量以及疾病的体征和症状。
4例患者（年龄分别为37、45、61和65岁）MH排泄量增加而MIMA排泄量未增加，2例患者（年龄分别为26和48岁）包括唯一1例儿童期起病的皮肤肥大细胞增多症患者的MH排泄量减少。无患者MIMA排泄量增加，1例患者（年龄51岁）MIMA排泄量减少。1例患者（年龄23岁）MH和MIMA排泄量均增加，2例患者（年龄分别为65和79岁）MH和MIMA排泄量均减少。1例患者（年龄49岁）的MH和MIMA排泄量可认为稳定。其余5例患者观察期较短，无法对其疾病病程作出明确判断。在MH和MIMA排泄量均减少的2例患者中，肿大的脾脏体积缩小，而其他患者的体征和症状未改变。所有患者均无伴发的骨髓增殖性疾病。未给予旨在降低肥大细胞负荷的特殊治疗。整个随访期变化率为每年-8.4%至+25.1%。
成人期起病的惰性肥大细胞增多症的自然病程并非总是进行性的。我们的数据表明，以MH和MIMA尿排泄量及临床体征和症状衡量的成人期起病的惰性肥大细胞增多症的活性可大幅下降，尤其是在老年患者中。
