Molderings Gerhard J, Afrin Lawrence B, Hertfelder Hans-Jörg, Brettner Stefan
Institute of Human Genetics, University Hospital Bonn, Bonn, D-53127, Germany.
Armonk Integrative Medicine, Armonk, NY, 10504, USA.
F1000Res. 2017 Dec 28;6:2182. doi: 10.12688/f1000research.13343.1. eCollection 2017.
Mast cell activation disease typically presents as chronic multisystem polymorbidity of generally inflammatory ± allergic theme. Presently, treatment of the rare, cytoproliferative variant systemic mastocytosis employs empirically selected therapies to impede mast cell mediator production and action and, when necessary, inhibition of proliferation. Some tyrosine kinase inhibitors (TKIs) have been used successfully in uncommon cases of systemic mastocytosis not bearing that disease's usual imatinib-resistant KIT mutation. Recently, sunitinib, a multi-targeted TKI, had been successful in a case of systemic mast cell activation syndrome. In addition, most allergy is principally a mast cell activation phenomenon, and sunitinib has been shown helpful in controlling a murine model of oral allergy syndrome. Here, we present the first use of sunitinib in systemic mastocytosis.
肥大细胞活化疾病通常表现为以炎症±过敏为主题的慢性多系统多发病。目前，对于罕见的细胞增殖性系统性肥大细胞增多症变体，治疗采用经验性选择的疗法来阻碍肥大细胞介质的产生和作用，并在必要时抑制增殖。一些酪氨酸激酶抑制剂（TKIs）已成功用于系统性肥大细胞增多症的罕见病例，这些病例不存在该疾病常见的对伊马替尼耐药的KIT突变。最近，多靶点TKI舒尼替尼在一例系统性肥大细胞活化综合征中取得了成功。此外，大多数过敏主要是肥大细胞活化现象，舒尼替尼已被证明有助于控制口腔过敏综合征的小鼠模型。在此，我们展示了舒尼替尼在系统性肥大细胞增多症中的首次应用。
