Inflammatory malignant fibrous histiocytoma associated with leukemoid reaction or leukocytosis: a comprehensive review.
Inflammatory malignant fibrous histiocytoma associated with leukemoid reaction or leukocytosis: a comprehensive review.
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DOI：
10.5402/2012/946019
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发表时间：
2012
期刊：
影响因子：
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通讯作者：
Frieri M
中科院分区：
文献类型：
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作者：
Hurtado-Cordovi J;Pathak P;Avezbakiyev B;Frieri M
炎性恶性纤维组织细胞瘤（IMFH）合并白血病样反应(LR)/白细胞增多是一种罕见的疾病。在本文中，我们在PubMed检索了所有已知的与LR/白细胞增多症相关的IMFH病例，试图得出关于这种变异对治疗的反应及其病理生理的结论。以恶性纤维组织细胞瘤、类白血病反应、白细胞增多等关键词检索Medline电子数据库。共16例，男12例（75%），女4例（25%），平均年龄62.6岁，年龄47 ~ 77岁。平均生存期770天，14 ~ 6570天不等。4例患者在末次随访时存活，分别为6570天、1095天、335天和180天。在12例死亡的患者中，死亡发生在LR或白细胞增多症发病后约92天，时间从3天到334天不等。我们得出结论，与LR/白细胞增多相关的IMFH对放化疗没有完全反应。IMFH细胞过量产生生长因子和细胞因子，并与炎症浸润相互作用，似乎促进了免疫效应细胞的功能障碍，并证实了该肿瘤的发展和生长。为了确定潜在的治疗靶点，清楚地了解这些分子途径是至关重要的。
Inflammatory malignant fibrous histiocytoma (IMFH) associated with leukemoid reaction (LR)/leukocytosis is a rare entity. In this paper, we search PubMed for all known cases of IMFH associated with LR/leukocytosis in an attempt to draw conclusions about this variant's response to treatments and its pathophysiology. Medline electronic database was searched using key words such as malignant fibrous histiocytoma, leukemoid reaction, and leukocytosis. A total of 16 patients were found, twelve males (75%) and 4 female (25%), with a mean age of 62.6 years, ranging from 47 to 77. The mean survival was 770 days, ranging from 14 to 6570 days. Four patients were alive at last follow-up: 6570 days, 1095 days, 335 days, and 180 days, respectively. Of the 12 patients that expired, death occurred approximately 92 days after the onset of LR or leukocytosis, ranging from 3 to 334 days. We conclude that IMFH associated with LR/leukocytosis does not completely respond to chemoradiation. Overproduction of growth factors and cytokines by IMFH cells and their interactions with the inflammatory infiltrate seem to promote immunological effector cell's dysfunction and substantiate the development and growth of this neoplasm. A clear understanding of these molecular pathways is crucial in order to identify targets for potential therapy.