-
骨髓纤维化分为原发性骨髓纤维化和继发性骨髓纤维化,其中以继发性骨髓纤维化更常见。导致继发性骨髓纤维化的病因多种多样,血液系统疾病则是其主要病因之一,诸如:白血病、淋巴瘤、多发性骨髓瘤等[1]。肥大细胞增生症虽然是血液系统疾病,并且也可导致继发性骨髓纤维化,但临床极为罕见,笔者拟通过该病例及相关文献学习加深对肥大细胞增生症伴继发性骨髓纤维化的认识。
系统性肥大细胞增生症致继发性骨髓纤维化PET/CT显像一例
Myelofibrosis secondary to systemic mastocytosis: a case report of PET/CT
-
摘要: 笔者报道了一例系统性肥大细胞增生症伴继发性骨髓纤维化的18F-FDG PET/CT显像病例。从临床症状、实验室检查、影像学检查以及骨髓穿刺等结果综合分析了该病的特点。并通过文献复习加深了对骨髓纤维化及肥大细胞增生症的认识。继发性骨髓纤维化的病因多种多样,肥大细胞增生症就是其病因之一。肥大细胞增生症是一种以肥大细胞多组织器官异常增生为特征的血液系统疾病,肥大细胞分泌的组胺会导致骨质密度弥漫性增高,肝脾淋巴结肿大一方面是因为肥大细胞的浸润,另一方面则是因为髓外造血所致。另外,此类患者出现的诸多临床症状也与肥大细胞分泌的化学介质密切相关。Abstract: A case of myelofibrosis secondary to systemic mastocytosis of 18F-FDG PET/CT imaging was reported. The characteristics of the disease were analyzed on the basis of clinical symptoms, laboratory examinations, imaging examinations, and bone trephine. The understanding of myelofibrosis and mastocytosis was deepened by literature review. Secondary myelofibrosis has numerous causes, one of which is mastocytosis. Mastocytosis is a blood system disease characterized by abnormal proliferation of mast cells in multiple tissues and organs. The histamine secreted by mast cells plays an important role in osteosclerosis. Lymphadenopathy characterized by enlarged liver and spleen is due to mast cell infiltration and extramedullary hematopoiesis. Symptoms of patients are also closely related to the chemical mediators secreted by mast cells.
-
Key words:
-
-
[1] 徐海萍, 高继勇, 宋凤娟, 等. 骨髓纤维化74例临床分析[J]. 白血病·淋巴瘤, 2006, 15(5): 369−371. DOI: 10.3760/cma.j.issn.1009−9921.2006.05.018.
Xu HP, Gao JY, Song FJ, et al. Clinical analysis of 74 cases of myelofibrosis[J]. J Leuk Lymphoma, 2006, 15(5): 369−371. DOI: 10.3760/cma.j.issn.1009−9921.2006.05.018.[2] Balkan C, Ersoy B, Nese N. Myelofibrosis associated with severe vitamin D deficiency rickets[J]. J Int Med Res, 2005, 33(3): 356−359. DOI: 10.1177/147323000503300311. [3] Butt YM, Chen WN. Myelofibrosis secondary to renal osteodystrophy[J]. Blood, 2016, 128(16): 2104. DOI: 10.1182/blood−2016−07−726182. [4] Lim DJ, Oh EJ, Park CW, et al. Pancytopenia and secondary myelofibrosis could be induced by primary hyperparathyroidism[J]. Int J Lab Hematol, 2007, 29(6): 464−468. DOI: 10.1111/j.1365−2257.2006.00877.x. [5] Del Porto F, Tatarelli C, Di Napoli A, et al. Systemic lupus erythematosus and myelofibrosis: a case report and revision of literature[J]. Leuk Res Rep, 2018, 9: 58−64. DOI: 10.1016/j.lrr.2018.04.004. [6] Valent P, Horny HP, Escribano L, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal[J]. Leuk Res, 2001, 25(7): 603−625. DOI: 10.1016/S0145−2126(01)00038−8. [7] Ozdemir D, Dagdelen S, Erbas T. Systemic mastocytosis[J]. Am J Med Sci, 2011, 342(5): 409−415. DOI: 10.1097/MAJ.0b013e3182121131. [8] Fritz J, Fishman EK, Carrino JA, et al. Advanced imaging of skeletal manifestations of systemic mastocytosis[J]. Skeletal Radiol, 2012, 41(8): 887−897. DOI: 10.1007/s00256−012−1374−9. [9] Djelbani-Ahmed S, Chandesris MO, Mekinian A, et al. FDG-PET/CT findings in systemic mastocytosis: a French multicentre study[J]. Eur J Nucl Med Mol Imaging, 2015, 42(13): 2013−2020. DOI: 10.1007/s00259−015−3117−3.