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Non-hematologic diagnosis of systemic mastocytosis: Collaboration of radiology and pathology
Blood Reviews ( IF 6.9 ) Pub Date : 2020-04-08 , DOI: 10.1016/j.blre.2020.100693
Kerem Ozturk 1 , Zuzan Cayci 1 , Jason Gotlib 2 , Cem Akin 3 , Tracy I George 4 , Celalettin Ustun 5
Affiliation  

Systemic mastocytosis (SM) is a hematologic disease with a wide range of clinical courses ranging from an indolent condition with normal life expectancy to exceedingly aggressive disorder with a poor prognosis. The symptoms and signs of SM result from the release of mast cell mediators with heterogeneous functions, and/or organ damage from neoplastic mast cell infiltration, or both. Diagnostic criteria for SM are well-defined by the World Health Organization (WHO). However, the diagnosis of SM can be difficult when especially it is not in the differential diagnosis. Routinely used radiologic techniques (e.g., X-ray, ultrasound, CT scans can show findings such as lytic-, sclerotic- or mixed-bone lesions, splenomegaly, hepatomegaly, retroperitoneal or periportal mesenteric lymphadenopathy, and omental thickening). It is essential to emphasize that the constellation of these radiologic findings should strongly concern of SM, especially in patients who also have a skin rash, allergic reactions, gastrointestinal tract symptoms (lasting, intermittent nausea, diarrhea), paroxysmal tachycardias, unexplained weight loss, persistent bone pain, cytopenias, liver dysfunction, eosinophilia. These findings, even coincidentally noted, will likely lead to a tissue biopsy, which reveals diagnosis (as we discussed and illustrated some tissue biopsies here). Moreover, the role of MRI and new techniques such as [18-fluorodeoxyglucose positron emission computed tomography, fibroscan] in the diagnosis of SM have been discussed. Furthermore, we reviewed the use of radiologic methods to evaluate treatment response and prognostication of SM. .



中文翻译:

系统性肥大细胞增多症的非血液学诊断:放射学和病理学的合作

系统性肥大细胞增多症 (SM) 是一种血液病,其临床病程范围广泛,从具有正常预期寿命的惰性状态到预后不良的极度侵袭性疾病。SM 的症状和体征是由具有异质功能的肥大细胞介质释放和/或肿瘤性肥大细胞浸润引起的器官损伤或两者兼而有之。SM 的诊断标准由世界卫生组织 (WHO) 明确定义。然而,特别是当 SM 不在鉴别诊断中时,SM 的诊断可能很困难。常规使用的放射学技术(例如,X 射线、超声、CT 扫描可以显示诸如溶骨性、硬化性或混合性骨病变、脾肿大、肝肿大、腹膜后或门静脉周围肠系膜淋巴结肿大和网膜增厚)等发现。必须强调的是,这些放射学发现的星座应该强烈关注 SM,特别是在同时有皮疹、过敏反应、胃肠道症状(持续、间歇性恶心、腹泻)、阵发性心动过速、不明原因的体重减轻、持续性骨痛、血细胞减少、肝功能障碍、嗜酸性粒细胞增多。这些发现,即使是巧合地注意到,也可能导致组织活检,从而揭示诊断(正如我们在此处讨论和说明的一些组织活检)。此外,还讨论了 MRI 和新技术(如 [18-氟脱氧葡萄糖正电子发射计算机断层扫描,纤维扫描])在 SM 诊断中的作用。此外,我们回顾了使用放射学方法来评估 SM 的治疗反应和预后。 .

更新日期:2020-04-08
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