骨髓纤维化5例分析

骨髓纤维化５例分析哈励逊国际和平医院孙惠淑王学敏（衡水０５３０００）关键词骨髓纤维化诊断骨髓纤维化是骨髓发生弥漫性纤维组织和骨质增生伴髓外造血的一种骨髓增生性疾病,在临床上分为原发性和继发性２种。前者原因不明,后者可因肿瘤（包括急慢性白血病）、感染、...     

原发性骨髓纤维化的X线分析和诊断（附8例报告）

原发性骨髓硬化症又称骨髓纤维化，是一种血液病性的骨病变，以骨髓纤维化和髓外造血为特征，病因不明。此病较为少见，易与显示为骨硬化的有关疾病混淆。笔者对8例经骨髓活组织检查证实为骨髓纤维化的患者的X线平片作回顾性分析，以讨论该病的X线表现和诊断。     

肺性肥大性骨关节病的临床及X线表现

肥大性骨关节病又称马-斑(marie-bamberger)综合征[1],分特发性和继发性两种.特发性有家族史,病因不明,与遗传有关.Currarino等认为此病有自愈倾向,对临床影响不大.本文重点讨论继发性肥大性骨关节病.     

儿童肌肉,骨骼磁共振成象

文内介绍了儿童正常骨髓的MR表现,特别是红骨髓到黄骨髓的转化。讨论了儿童骨髓病变,如骨髓增生、骨髓置换、骨髓衰竭和骨髓纤维化的病因、病理,强调了MR影象与病理改变间的相互联系。     

肥大细胞与IgA肾病患者肾间质病变关系的研究

目的研究肥大细胞与IgA肾病患者肾间质病变之间的关系。方法收集49例IgA肾病患者的临床资料和肾活检标本,采用免疫组织化学技术检测肥大细胞在IgA肾病患者肾组织中的表达,并分析其意义。结果正常肾组织中偶见或无肥大细胞的存在,而在IgA肾病患者肾组织中肥大细胞表达明显增强（肥大细胞检出阳性率为42.86%）,并且随着肥大细胞的表达增多IgA肾病肾间质纤维化程度逐渐加重（χ2=9.1189,P〈0.05）;肥大细胞与临床指标的相关性：肥大细胞数量与血肌酐呈显著正相关（r=0.65,P〈0.05）,与尿蛋白定量无明显相关性（r=0.18,P〉0.05）。结论肥大细胞可能是IgA肾病肾间质纤维化的重要参与者,与IgA肾病肾间质病变的发生发展可能具有一定的关系。     

骨髓纤维化与巨核细胞数的关系

骨髓纤维化(MF)可在多种血液病中发生,其原因尚不清楚。虽有人发现MF局部有巨核细胞聚集现象,继发性骨髓纤维化亦有巨核细胞增多;但也有相反的报道。本文就此作了观察。1 材料与方法 1.1 对象为1989年9月以来我院住院的血液病患者及有骨髓转移的癌症患者,可供分析者136例,男92例,女44例,年龄范围15～78岁。 1.2 方法 采用上海产环钻骨髓活检针于髂后或髂前上嵴取骨髓组织约7mm     

飞行人员鼻腔结构异常的诊治和鉴定

目的 总结飞行人员鼻腔结构异常(鼻中隔偏曲和鼻甲肥大)的临床诊治和健康鉴定经验.方法 分析因鼻腔结构异常而住院诊治的飞行人员病历资料91份,对其进行分类和分型,总结鼻腔结构异常的分型与继发性气压伤之间的关系.结果 飞行人员鼻腔结构异常分类为Ⅰ类(地面有症状或体征)50例,Ⅱ类(地面无症状或体征、飞行中出现继发性气压伤,包括Ⅱa继发性鼻窦气压伤10例,Ⅱb继发性耳气压伤26例,Ⅱc继发性鼻窦气压伤+耳气压伤5例)41例.分型为鼻中隔偏曲型41例,下鼻甲肥大型29例,中鼻甲肥大型8例,鼻中隔偏曲复合鼻甲肥大型13例.鼻中隔偏曲24例次、下鼻甲肥大12例次、中鼻甲肥大14例次与继发性气压伤有关.85例飞行合格,1例由歼击机降为运输机飞行,2例暂时飞行不合格,3例永久停飞.结论 鼻腔结构异常在飞行人员中常见,根据对鼻窦和耳气压功能的影响进行分类和分型具有重要的临床航空医学意义,在飞行学员医学选拔、飞行人员疾病诊治和健康鉴定时统一操作规范,是提高招飞体检质量及疾病诊治和健康鉴定水平,保障航空兵部队战斗力的重要措施.     

老年血液病病因

国内唐鉴坤等分析233例65岁以上老年人贫血占老年住院病人的13．3％，其中以缺铁性贫血发病率最高，约占36～66％，再生障碍性贫血发病率高于日本和西欧，继发性自身免疫性溶血性贫血、营养性巨幼红细胞性贫血及骨髓病性贫血亦常见于老年人。其它骨髓增生性疾病，如真性红细胞增多症、原发性血小板增多症、骨髓纤维化等，也见于老年人。贫血等血液病对老年人的影响大，探讨其病因，对疾病的预防和治疗十分重要。门）随着年龄增长，红骨髓量减少，被黄和纤维组织代替，故骨髓造血功能减退。（2）睾丸酮分泌不足，因而对蛋白的同化作用和对红…     

肥大细胞,成纤维细胞与器官纤维化

近年来研究发现，肥大细胞不仅参与机床的变态反应、免疫病理学反应以及多种生理病理学过程，而且在机体器官纤维化的发生发展过程中也起着重要作用。本文着重综述肥大细胞成纤维细胞生长调节的相互依赖关系，肥大细胞及其介导剂对成纤维细胞生长增殖的影响，以及肥大细胞在器官纤维化中的作用。     

FDG positron emission tomography in patients with systemic mastocytosis

OBJECTIVE: Systemic mastocytosis is a hematologic neoplasm characterized by abnormal accumulation and growth of mast cells in one or more organ systems. We analyzed five patients with systemic mastocytosis referred for FDG positron emission tomography who had biopsy-proven mast cell infiltrates in various organs. CONCLUSION: Our findings indicate that FDG positron emission tomography is not useful for staging and follow-up of aggressive systemic mastocytosis.     

Systemic mastocytosis: MRI of bone marrow involvement

Systemic mastocytosis (SM) is an abnormal proliferation of mast cells, located in different structures: skin, bone marrow, spleen, liver and lymph nodes. Magnetic resonance imaging was prospectively performed in ten patients diagnosed by bone marrow biopsy in order to describe the different patterns of bone marrow involvement. Coronal T1-weighted spin-echo images were obtained in vertebral, pelvic, humeral and femoral bones. Depending on the extension of the cell infiltration, three patterns of bone marrow involvement were used: normal/no involvement (N), non-homogeneous (NH) and homogeneous (H). All ten patients presented bone infiltration. The patterns observed were: spine (50 % NH, 50 % H), pelvis (70 % NH), humerus 100(NH) and femur 40 % (NH). T1-weighted MR imaging is a sensitive technique for detecting marrow abnormalities in patients with systemic mastocytosis. There is no correlation between percentage of mast cells in bone marrow biopsy and extent or pattern of bone marrow involvement. Received: 5 June 1998; Revision received: 23 November 1998; Accepted: 15 January 1999     

Idiopathic myelofibrosis. Radiological aspects of bone changes

Idiopathic myelofibrosis is a chronic myeloproliferative disease characterized by skeletal lesions (30 to 70% of cases). We considered 49 patients with idiopathic myelofibrosis treated between 1972 and 1986 at the Institutes of Hematology and Radiotherapy, University of Bologna. Only 19 of these patients underwent roentgenographic skeletal surveys, associated with whole body bone scintigraphy in 4 cases, and with CT in 1 case. The most common bone change, as seen in 12 patients, was osteosclerosis, following two distinct patterns: pure, and mixed. Other types of bone involvement (osteoporosis and pure osteolysis) were seen in 2 cases only; in 5 patients radiological skeletal examinations did not show meaningful lesions. Conventional radiology is hardly ever conclusive in the diagnosis of idiopathic myelofibrosis. This is due partly to the often moderate degree of bone involvement, partly to the scanty specificity of the findings. However, a careful examination of the plain roentgenographs, completed when possible by other more recent imaging techniques, may be very important towards a more precise definition of the disease and, in some cases, for a correct diagnosis.     

Advanced imaging of skeletal manifestations of systemic mastocytosis

Systemic mastocytosis comprises a group of clonal disorders of the mast cell that most commonly involves the skeletal system. Imaging can be helpful in the detection and characterization of the osseous manifestations of this disease. While radiography and bone scans are frequently used for this assessment, low-dose multidetector computed tomography and magnetic resonance imaging can be more sensitive for the detection of marrow involvement and for the demonstration of the various disease patterns. In this article, we review the pathophysiological and clinical features of systemic mastocytosis, discuss the role of imaging for staging and management, and illustrate the various cross-sectional imaging appearances. Awareness and knowledge of the imaging features of this disorder will increase the accuracy of image interpretation and can contribute important information for management decisions.     

Nuclear medicine imaging of systemic mastocytosis

A 66-yr-old male with systemic mastocytosis is presented along with classic imaging findings of dense bone on radiographs, intense uptake on bone scan and marrow expansion on bone marrow scan. Dual photon absorptiometry (DPA) measurements of the skeleton revealed a very high bone mineral measurement of 1.678 grams per square centimeter (lumbar vertebrae 2 through 4). It is suggested that DPA may be useful to follow the development of mast cell disease in the bones.     

Multiple bee stings,peritumoral mast cell degranulation and anaphylaxis – Is there a relationship?

A case of a 58-year-old with fatal anaphylaxis due to multiple bee stings is reported. Supportive evidence for anaphylaxis included post-mortem serum tests, which demonstrated a markedly elevated tryptase level and increased sensitivity to bees on radioallergosorbent test (RAST). At autopsy a previously undiagnosed esophageal adenocarcinoma involving the gastroesophageal (GE) junction was also identified. Histology of the tumor demonstrated significant numbers of mast cells, many of which were degranulating. Increased numbers of mast cells, as in mastocytosis, are known to predispose to an allergic sensitivity to Hymenoptera. The finding of a significant peritumoral mast cell population with degranulating forms in this case, therefore, raises the possibility that death due to anaphylaxis was contributed to by mast cell proliferation in an occult esophageal carcinoma.     

Functional asplenia in malignant mastocytosis

A 47-yr-old man with malignant mastocytosis, a malignant neoplasia of mast cells, presented with anemia. Ultrasonography revealed an enlarged spleen. A liver-spleen scan using [99mTc]sulfur colloid failed to show any splenic uptake consistent with the diagnosis of functional asplenia. Functional asplenia related to this condition has not been reported previously. Since the spleen was heavily infiltrated with space occupying mast cells, a mass displacing effect along with impaired perfusion are discussed as underlying mechanism.     

Imaging findings in patients with myelofibrosis

The purpose of this review is to illustrate the wide range of radiological abnormalities in myelofibrosis. Myelofibrosis, also called myeloid metaplasia, is a myeloproliferative disorder of unknown etiology. The common imaging findings in patients with myelofibrosis are osteosclerosis, hepatosplenomegaly, and lymphadenopathies. In addition, extramedullary hematopoiesis may develop in multiple sites such as chest, abdomen, pelvis, and central nervous system, simulating malignant disease. Selected plain-film, CT, and MR images in patients with myelofibrosis are shown as pictorial essay to allow ready recognition of the most common imaging abnormalities of the disease. Received: 6 August 1998; Revision received: 15 October 1998; Accepted: 20 November 1998     

Open quiz solution: Case report 705

In summary, Sarcoidosis is a rare but well-recognized cause of diffuse osteosclerosis. The differential diagnosis of osteosclerosis is limited and includes osteoblastic metastases, sclerotic myeloma, myelofibrosis, and less common infiltrative bone marrow processes such as mastocytosis and sarcoidosis. In all of these entities the sclerosis is found most often in the axial (red marrow) skeleton. In this regard, it is interesting that cases of osseous sarcoid are usually lytic and located in the peripheral skeleton. In patients with osteosclerotic sarcoidosis, the diagnosis may be suggested by a past history of the disease or ancillary signs such as hilar node enlargement and subtle skin involvement. However, the specific diagnosis usually requires bone marrow biopsy with the demonstration of extensive, noncaseating granulomas.     

3T-MRI, elastography, digital mammography, and FDG-PET CT findings of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) of the breast

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous lymphoma, which is characterized by infiltration of neoplastic cytotoxic T cells into the subcutaneous tissue. We here report the case of a 66-year-old woman with SPTCL of the breast, which is a very uncommon location. Multiple suspicious irregular small masses in the subcutaneous fat were detected by mammography, and sonograms revealed hyperechoic masses. Elastography was useful to improve depiction and delineation of SPTCL in the hyperechoic subcutaneous fat, and dynamic contrast-enhanced MRI examinations showed multiple irregular rim enhanced masses with persistent enhancement. FDG-PET CT images showed hypermetabolism in areas corresponding to other imaging techniques. MRI can be useful for diagnosis of fat necrosis, which is a primary radiologic feature of SPTCL. However, fat necrosis has multitude of appearances by various imaging techniques, which typically indicate a benign disease, but may indicate a malignancy. Therefore, an ultrasonographically guided core needle biopsy is useful for a diagnosis of SPTCL of the breast. The presence of multiple subcutaneous nodules throughout the body on CT imaging may be an important finding that suggests a diagnosis of SPTCL.