Sato Mai, Kurokawa Ryo, Okimoto Naomasa, Tokushige Junji, Ikemura Masako, Abe Osamu
Radiology, The University of Tokyo, Tokyo, JPN.
Hematology and Oncology, The University of Tokyo, Tokyo, JPN.
Cureus. 2024 Jul 18;16(7):e64879. doi: 10.7759/cureus.64879. eCollection 2024 Jul.
Aggressive systemic mastocytosis (ASM) is an advanced subtype of systemic mastocytosis characterized by organ involvement. In this article, we report a case with ASM in a 54-year-old woman with characteristic findings on computed tomography (CT) and fluorine-18-fluorodeoxyglucose positron emission tomography (F-FDG PET)/CT. Contrast-enhanced CT on admission revealed hepatosplenomegaly, generalized osteosclerosis, colonic edema, edematous thickening of the wall in the ascending colon and edema in the surrounding regions of these organs and mesentery, ileus, subcutaneous edema, periportal collar sign, and multiple mesenteric lymphadenopathies. There was no F-FDG uptake in the lesions other than mild F-FDG uptake in the vertebrae, making the possibility of differential diagnoses such as metastasis, lymphoma, and extramedullary leukemia lower. Based on bone marrow biopsy results and clinical findings, the diagnosis of ASM was established. ASM can be a potentially fatal disease with a poor prognosis, and understanding its distinctive clinical course and imaging findings is crucial for early therapeutic intervention.
侵袭性系统性肥大细胞增多症（ASM）是系统性肥大细胞增多症的一种晚期亚型，其特征为累及多个器官。在本文中，我们报告了一例54岁女性的ASM病例，该病例在计算机断层扫描（CT）和氟-18-氟脱氧葡萄糖正电子发射断层扫描（F-FDG PET）/CT上有特征性表现。入院时的增强CT显示肝脾肿大、全身性骨质硬化、结肠水肿、升结肠壁水肿增厚以及这些器官和肠系膜周围区域的水肿、肠梗阻、皮下水肿、门静脉周围环状征和多处肠系膜淋巴结肿大。除了椎体有轻度F-FDG摄取外，病变部位无F-FDG摄取，这使得转移瘤、淋巴瘤和髓外白血病等鉴别诊断的可能性降低。根据骨髓活检结果和临床表现，确诊为ASM。ASM可能是一种预后不良的潜在致命疾病，了解其独特的临床病程和影像学表现对于早期治疗干预至关重要。
