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首页> 外文期刊>Sao Paulo Medical Journal >Indolent systemic mastocytosis limited to the bone: a case report and review of the literature
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Indolent systemic mastocytosis limited to the bone: a case report and review of the literature

机译：惰性全身性肥大细胞增多症仅限于骨：一例病例报告并文献复习

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CONTEXT Systemic mastocytosis is defined as a clonal disorder of mast cells and their precursor cells and is currently classified as a myeloproliferative neoplasm. Its clinical course has a wide spectrum, ranging from indolent disease, with normal life expectancy, to highly aggressive disease, associated with multisystemic involvement and poor overall survival. The aim of this study was to report a case of indolent systemic mastocytosis, focusing on the diagnostic challenges, with a review of the literature. CASE REPORT A 79-year-old Caucasian woman with osteoporosis was evaluated at the Emergency Department because of complaints of low back pain. Before this, she had consulted an orthopedist and had undergone some imaging examinations, namely a bone scan that revealed a “superscan” pattern. Due to her pain complaints and these test results, the patient was admitted to the Department of Internal Medicine. After undergoing several analytical tests and some additional imaging examinations to rule out some important differential diagnoses, she then underwent bone marrow biopsy, which made it possible to identify indolent systemic mastocytosis. CONCLUSION Systemic mastocytosis is a rare entity that is difficult to diagnose. Its symptoms are often unspecific and frequently ignored. Skeletal changes may be the first and only manifestation of the disease and in some cases, like this one, the diagnosis is made only after histological examination. The key point for the diagnosis is to contemplate the possibility of systemic mastocytosis.
机译：背景系统性肥大细胞增多症定义为肥大细胞及其前体细胞的克隆性疾病，目前被分类为骨髓增生性肿瘤。它的临床过程范围很广，从惰性疾病，具有正常预期寿命的疾病到高度侵袭性疾病，与多系统参与和不良整体生存有关。这项研究的目的是报告一例缓慢的全身性肥大细胞增多症，重点是诊断挑战，并进行文献综述。病例报告由于腰痛的投诉，急诊科对一名患有骨质疏松症的79岁白人妇女进行了评估。在此之前，她曾咨询骨科医生并接受了一些影像学检查，即进行了“超级扫描”模式的骨扫描。由于她的疼痛症状和这些测试结果，该患者被送入内科。在经历了几次分析测试和一些额外的影像学检查以排除一些重要的鉴别诊断之后，她随后进行了骨髓活检，从而可以鉴别出惰性的全身性肥大细胞增多症。结论系统性肥大细胞增多症是一种罕见的难以诊断的实体。它的症状通常是不确定的，经常被忽略。骨骼变化可能是该病的第一个也是唯一的表现，在某些情况下，像这种情况，只有在组织学检查后才能做出诊断。诊断的关键是考虑全身性肥大细胞增多症的可能性。

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