Soluble stem cell factor receptor (CD117) and IL-2 receptor alpha chain (CD25) levels in the plasma of patients with mastocytosis: relationships to disease severity and bone marrow pathology
Soluble stem cell factor receptor (CD117) and IL-2 receptor alpha chain (CD25) levels in the plasma of patients with mastocytosis: relationships to disease severity and bone marrow pathology
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DOI：
10.1182/blood.v96.4.1267.h8001267a_1267_1273
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发表时间：
2000-08-15
期刊：
影响因子：
20.3
通讯作者：
Metcalfe, DD
中科院分区：
文献类型：
--
作者：
Akin, C;Schwartz, LB;Metcalfe, DD
系统性肥大细胞增多症是一种肥大细胞增殖的疾病，可能与血液系统疾病有关，检查时没有允许诊断系统性疾病的特征，肥大细胞衍生的介质可能在尿液或血液中升高，也可能在严重过敏性疾病患者中升高。因此，诊断通常取决于骨髓活检的结果。为了便于评估，需要疾病范围和严重程度的替代标志物。由于肥大细胞增多症与血液病相关，因此测量了可溶性KIT（sKIT）和可溶性白细胞介素-2受体α链（sCD 25）的血浆水平，已知这两种受体从肥大细胞表面部分裂解，并在某些血液恶性肿瘤中升高。结果显示，两种可溶性受体的水平在系统性肥大细胞增多症中均升高。中位血浆sKIT浓度（以Au/mL表示）（1 Au =1.4 ng/ mt）：对照组176例（n = 60），无全身受累的色素性荨麻疹194例（n = 8），系统性惰性肥大细胞增多症511例（n = 30），系统性肥大细胞增多症伴相关血液学疾病1320例（n = 7）;侵袭性肥大细胞增多症，3390例（n = 3），全身性肥大细胞增多症患者血浆sCD 25水平升高;最高水平与广泛的骨髓受累相关。sKIT水平与总类胰蛋白酶水平、sCD 25水平和骨髓病理学相关。这些结果表明，sKIT和sCD 25是肥大细胞增多症患者疾病严重程度的有用替代标志物，应有助于诊断、选择需要骨髓活检的患者和记录疾病进展。（血。2000;96：1267-1273）（C）2000由美国血液学学会。
Systemic mastocytosis is a disease of mast cell proliferation that may be associated with hematologic disorders, There are no features on examination that allow the diagnosis of systemic disease, and mast cell-derived mediators, which may be elevated in urine or blood, may also be elevated in individuals with severe allergic disorders. Thus, the diagnosis usually depends on results of bone marrow biopsy. To facilitate evaluation, surrogate markers of the extent and severity of the disease are needed. Because of the association of mastocytosis with hematologic disease, plasma levels were measured for soluble KIT (sKIT) and soluble interleukin-2 receptor alpha chain (sCD25), which are known to be cleaved in part from the mast cell surface and are elevated in some hematologic malignancies, Results revealed that levels of both soluble receptors are increased in systemic mastocytosis. Median plasma sKIT concentrations as expressed by AU/mL (1 AU =1.4 ng/ mt) were as follows: controls, 176 (n = 60); urticaria pigmentosa without systemic involvement, 194 (n = 8); systemic indolent mastocytosis, 511 (n = 30); systemic mastocytosis with an associated hematologic disorder, 1320 (n = 7); aggressive mastocytosis, 3390 (n = 3), Plasma sCD25 levels were elevated in systemic mastocytosis; the highest revels were associated with extensive bone marrow involvement. Levels of sKIT correlated with total tryptase levels, sCD25 levels, and bone marrow pathology These results demonstrate that sKIT and sCD25 are useful surrogate markers of disease severity in patients with mastocytosis and should aid in diagnosis, in the selection of those needing a bone marrow biopsy, and in the documentation of disease progression. (Blood. 2000;96: 1267-1273) (C) 2000 by The American Society of Hematology.