Localized AL amyloidosis: A suicidal neoplasm?

Abstract

Although AL amyloidosis usually is a systemic disease, strictly localized AL deposits are not exceptionally rare. Such case reports form a considerable body of published articles. Although both AL amyloidosis types are formed from an N-terminal segment of a monoclonal immunoglobulin light chain, a typical localized AL amyloid differs from the systemic counterpart by the morphological appearance of the amyloid, and presence of clonal plasma cells and of giant cells. In this article it is pointed out that localized AL amyloidosis (‘amyloidoma') represents a true plasma cell neoplasm and not a pseudotumor. The pathogenesis of localized AL amyloidosis may differ from that of the systemic type, a suggestion underlined by the fact that localized AL amyloidosis of kappa type is as common as that of lambda origin, in contrast to the systemic form where lambda chains constitute the overwhelming majority of cases. It is suggested that oligomeric assemblies of the produced immunoglobulin light chain are toxic to plasma cells, which in this way commit suicide.     

Renal Amyloidosis Secondary to ANCA-Associated Vasculitis: A Case Report

Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.     

72例多发性骨髓瘤单克隆蛋白分析

用速率散射比浊法测定 72例多发性骨髓瘤 (MM)血清与尿中单克隆蛋白 (M蛋白 )。结果显示 ,与IgG型 ,IgA型对应的免疫球蛋白 (Ig)均显著增高 ,不对应的Ig均明显降低。无论κ或λIgG ,IgA型 ,其对应的血清轻链显著增高 ,不对应的血清轻链显著降低。无论LC κ或是LC λ型 ,对应的血轻链在正常范围 ,不对应的血轻链低于正常 ,而尿中对应的轻链显著增高。在IgG ,IgA ,LC三型中 ,血清与尿中轻链κ/λ均显著异常。提示 ,血清中κ轻链 >2 0g·L 1或 <5g·L 1,λ轻链 >10g·L 1或 <2g·L 1,血、尿中κ/λ >5或 <0 .75时 ,任一项指标对MM的诊断均具有重要价值。     

72例多发性骨髓瘤单克隆蛋白分析

Serum and urinary monoclonal proteins (M protein) were measured in 72 cases of multiple myeloma (MM) using rate nephelometry. In IgG and IgA types of MM, the level of immunoglobulin (Ig) corresponding to the malignant isotype was significantly higher and that of Ig uncorresponding to the malignant isotype lower than the normal level. The light chain corresponding to the malignant isotype in serum was increased and the light chain uncorresponding to that in serum was decreased either kappa-IgG, IgA types or lambda-IgG, IgA types. Either kappa-LC or lambda-LC type of MM, the serum light chain corresponding to the malignant isotype was in the normal range and uncorresponding to that was decreased, and the corresponding light chain in urine was significantly elevated. Kappa/lambda ratio in serum and urine was all significantly abnormal in IgG, IgA, and LC types of MM. Our data suggest that any quota among kappa light chain > 20 g.L-1 or < 5 g.L-1, lambda light chain > 10 g.L-1 or < 2 g.L-1 in serum and kappa/lambda ratio > 5 or < 0.75 in serum and urine has an important value for diagnosing MM.     

免疫固定电泳技术在多发性骨髓瘤诊断及分型中的应用

[目的]探讨免疫固定电泳技术在多发性骨髓瘤(MM)诊断及分型中的应用.[方法]收集30例患有MM的患者的血清进行相关检测.免疫固定电泳采用美国海伦那电泳仪进行检测及扫描;免疫球蛋白定量及血轻链检测:用西门子BNⅡ免疫分析仪以全自动方式进行速率散射比浊分析对MM患者的血清中免疫球蛋白(IgG、IgA、IgM)含量及血轻链...     

急性肾炎患儿尿IgG轻链检测及其临床意义

目的:探讨急性肾炎患儿尿IgG轻链与临床的关系。方法:使用闪射比浊法研究急性肾炎患儿尿IgGKappa、lambda轻链急性期、恢复期与对照组的比较以及与临床的关系。结果:急性肾炎患儿尿IgGLambda轻链急性期、恢复期与对照组比较,差异无统计学意义。急性肾炎患儿尿kappa轻链急性期与恢复期及对照组差异有统计学意义(P<0.05)。尿kappa轻链与蛋白尿呈正相关。结论:尿IgGKappa轻链升高可作为急性肾炎患儿肾炎发生时的炎症反应指标及估计疾病程度和预后的指标。     

Clinical pathology of nodal micrometasteses in non-small cell lung cancer

ObjectiveTo explore whether the conventional pathologic stages of some non-small cell lung cancer (NSCLC) patients were underestimated.Methods195 lymph node samples were taken from 25 NSCLC patients during the operations. Firstly, each resulting tissue block was processed for routine paraffin embedding. Then the 6～10 serial sections were chosen, each 5 μm thick, from every paraffin block of the lymph node. Finally, the first and the second last sections of each lymph node were stained by hematoxylin eosin (HE), and the other serial sections were used for the immunohistochemical (IHC) staining examination with the monoclonal antibody against cyokeratin 19.ResultsWith HE staining, 30 of the 195 regional lymph nodes revealed dominant nodal metastases, and none showed micrometastases. IHC staining was performed on 135 lymph nodes that were identified as free of metastases by HE staining, 31 showed micrometastases; none showed gross nodal metastases. There was a significant difference between HE staining staging and IHC staining staging (P<0.05).ConclusionConventional HE staining can accurately detect gross nodal metastases in the lymph nodes of NSCLC patients, but is unfit for detecting lymph nodal micrometastases. IHC staining analysis can significantly facilitate the detection of occult micrometastatic tumor cells in lymph nodes, and its assessment of nodal micrometastases can provide a refinement of TNM stage for NSCLC patients. Our results provide a rationale for extensive lymph nodes sampling.     

血清Kappa与Lambda轻链的检测在恶性肿瘤中的表达

目的探讨血清中Kappa和Lambda轻链在恶性肿瘤患者中的表达情况。方法采用免疫比浊法对80例恶性肿瘤患者和20例健康体检者血清中Kappa和Lambda轻链进行检测。结果肝癌组患者血清中κ和λ值均显著高于健康对照组,且差异有统计学意义(P0.01);而κ/λ比值与健康对照组比较,差异无统计学意义(P0.05)。胃癌、肺癌、乳腺癌、食道癌、大肠癌组患者血清中κ、λ、κ/λ比值分别与健康对照组比较,差异均无统计学意义(P0.05)。结论血清中Kappa和Lambda轻链的测定,可作为原发性肝癌的诊断指标和对患者免疫功能的监测。     

κ和λ轻链标记对淋巴网状组织病变病理诊断的意义

对86例淋巴网状组织疾病进行抗κ和λ轻链抗血清标记。结果表明,淋巴组织反应性增生均为多克隆性。35例B细胞恶性淋巴瘤中,单克隆9例,大致单克隆15例,多克隆11例。提示单克隆和大致单克隆对确诊B细胞性恶性淋巴瘤有重要价值,多克隆性标记并不能完全排除恶性淋巴瘤的诊断。     

A study of Hodgkin/Reed-Sternberg cells using single cell polymerase chain reaction

Objective To investigate the characteristics of Hodgkin/Reed- Sternberg (H/R-S) cells found in patients with various types of Hodgkin’s disease (HD). Methods H/R- S cells were micropicked from frozen sections of tissues affected by HD. The DNA from these cells was amplified by polymerase chain reaction (PCR) using immunoglobulin heavy chain gene FRⅢa/JH primers and light chain gene family- specific primers. Results A total of 52/135 (35. 8%) isolated cells showed the specific products in the reactions. IgH and V(κ4) rearrangements were repeatedly found in many cells from a lymphocyte predominance type sample; repeated V(κ4) and individual IgH/V(κ2,4) rearrangements and individual IgH, V(λ 3) / V(κ4) rearrangements were found in two different cases of the nodular sclerosis type; repeated IgH/ V(λ3) and individual V(λ2,4) rearrangements, repeated V(κ2,4) rearrangements, repeated V(κ4) and individual IgH/ V(κ3) rearrangements, repeated IgH and individual V(κ3) / V(λ4) rearrangements were detected in 3 cases of the mixed cellularity type. Repeated and individual IgH rearrangements were found in other 2 cases. Conclusion The H/R- S cells isolated from the lymphocyte predominance subtypes of HD have IgH and V(λ4) gene rearrangements. This suggests that the lymphocyte predominance type is a proliferation of neoplastic B cells. The cells isolated from the mixed cellularity and nodular sclerosis types derive from B lineage cells at various stages of differentiation because of the presence of their IgH, κ and/or λ gene rearrangements. To our knowledge, this is the first time that the λ gene rearrangement was detected in H/R- S cells.     

原位杂交和免疫组化双标记技术在大鼠全脑脑片培养中的应用

目的探讨联合应用原位杂交(in situ hybridization,ISH)和免疫组化(immunohistochemistry,IHC)技术检测全脑脑片特定核酸和蛋白的表达。方法在静态全脑脑片培养的基础上,将培养的脑片进行冰冻切片,然后联合应用原位杂交和免疫组化技术检测特定基因的表达情况。结果脑片冰冻切片的质量可以进行后续的实验;与单纯应用一种方法相比,联合应用两种技术可同时在核酸水平和蛋白水平检测待测基因,两种显色方法不相互冲突,且可以更直观地显示核酸和蛋白的表达模式。结论原位杂交和免疫组化双染技术明显优于单纯的原位杂交技术或免疫组化;该双染技术可用于脑片培养中的基因和蛋白检测。     

Do extraglomerular microvasculature and mesenchymal interstitial cell proliferation indicate a stable course of lupus nephritis?

BackgroundLupus Nephritis (LN) is a major and frequent manifestation of Systemic Lupus Erythematosus (SLE), an autoimmune disease. Renal biopsy has a pivotal role in the diagnosis, prognosis, and management of the LN. The aim of this study was to count the mesenchymal interstitial cells utilizing CD34 immunohistochemistry (IHC) and morphometric analysis, correlate them with clinical parameters, class, activity, and chronicity indices and see if it can predict the course of the disease.MethodsA total of 32 renal biopsy blocks were analyzed by H&E stain, special stains, and CD34 IHC. Microvasculature density and interstitial stem cells were highlighted by CD34. These were then counted using a previously standardized computerized digital photomicrograph system (Dewinter Optical Inc) and manual count, respectively.ResultsOut of the 32 cases, Lupus class 3 comprised of 11 (34.38%) cases, class 4 comprised of 16 (50%) cases, and mixed class 4 + 5 had 5 (15.62%) cases. It was found that CD34 expression in the microvasculature (for both microvascular density and mean vascular lumen diameter) decreased in patients of Lupus Nephritis with higher disease activity (p < 0.05). Although not statistically significant, the number of interstitial stem cells increased with lower disease activity. A statistical significance was found between serum total protein, serum albumin, and serum creatinine among the three groups of LN.ConclusionImmunohistochemical staining of renal biopsy with CD34 may be used as a surrogate marker of disease activity in Lupus Nephritis patients.     

精子相关抗原9(SPAG9)在乳腺癌中的表达及其临床意义

目的 研究乳腺癌患者组织中精子相关抗原9（sperm associated antigen 9,SPAG9）和血清中SPAG9抗体的表达水平,了解其对乳腺癌诊断的价值。方法 免疫组化（immunohistochemistry,IHC）法检测40例乳腺癌患者病灶及其癌旁组织中SPAG9表达水平,分析SPAG9表达水平与临床病理分期（tumor node metastasis stage,TNM）的关系。酶联免疫吸附试验（enzyme-linked immunosorbent assay,ELISA）检测53例初诊乳腺癌、48例良性病变和43例健康对照血清SPAG9抗体浓度,分析血清SPAG9抗体浓度与患者临床指标的关系;并绘制受试者工作特征（receiver operating characteristic,ROC）曲线,评价血清SPAG9抗体对乳腺癌诊断的价值及意义。结果 乳腺癌病灶组织中SPAG9蛋白表达高于癌旁组织,二者差异有统计学意义（P=0.000）。乳腺癌血清SPAG9抗体浓度[10.22（5.055~16.660）mU/ml]显著高于良性病变组[6.666（3.369~11.517）mU/ml]（P<0.05）和健康对照组[5.079（2.857~8.571）mU/ml]（P=0.000）,且与年龄和TNM分期明显有关。ROC曲线下面积（area under the curve,AUC）为0.715,得出SPAG9抗体最佳临界值为10.185mU/ml时,SPAG9抗体诊断乳腺癌的敏感度为50.9%,特异性为88.4%。结论 SPAG9及其抗体在乳腺癌病灶组织和血清中高表达,血清SPAG9抗体对乳腺癌诊断具有一定的价值。     

κ及λ轻链测定在多发性骨髓瘤诊断中的应用

目的：寻求准确，可靠的免疫球蛋白及κ和λ轻链测定方法，提高多发性骨髓瘤分型和鉴别诊断水平。     

桥本甲状腺炎与黏膜相关淋巴组织的病理学特征

目的阐明桥本甲状腺炎（hashimoto thyroiditis,HT）的组织形态学及免疫组织化学特征,分析轻链限制在鉴别HT与黏膜相关淋巴组织（mucosa associated lymphoid tissue,MALT）淋巴瘤中的意义。方法 30例HT的石蜡标本和2例MALT淋巴瘤,分别用HE染色行组织病理学检查以及免疫组织化学检测CD20、CD3、lgκ、lgλ和细胞角蛋白,评价淋巴细胞的浸润及淋巴上皮病变（lymphoepithelial lesion,LEL）,轻链限制用于鉴别甲状腺早期淋巴瘤和HT。2例甲状腺原发性淋巴瘤作为对照。结果所有HT标本均显示HT特征性的组织病理特点。免疫组织化学染色显示了形态不显著的LEL,其中B细胞少见,而T细胞大量增生。轻链限制检测显示浆细胞多克隆性增生。2例MALT淋巴瘤显示破坏性淋巴上皮病变、B细胞免疫表型,其中浆细胞样细胞群中显示lgκ轻链限制。结论 HT与淋巴瘤的组织病理学及免疫组织化学特征显著不同,免疫组织化学有助于鉴别。     

巨细胞病毒在炎症性肠病中的临床意义及其诊治策略

目的 探讨巨细胞病毒（cytomegalovirus,CMV）在炎症性肠病（inflammatory bowel disease,IBD） 临床意义及其诊治策略。方法 从IBD患者[其中溃疡性结肠炎（UC）39例,克罗恩病（CD）15例]及对照组患者（肠易激综合征患者55例）血液中检测CMV抗体,并从 UC患者（其中手术标本11例,肠镜下活检标本39例）,以及克罗恩病患者（其中手术标本4例,肠镜下活检标本15例）,及对照组肠镜下活检标本55例,检测CMV抗体及CMV包涵体。结果 IBD伴CMV感染肠道病变更广泛,多为全结肠炎或右半结肠炎。IBD组CMV抗体阳性率高于对照组（P<0.01）,重度、激素难治性UC组全身激素治疗多于非激素难治性UC组（P<0.05）,重度、激素难治性UC组CMV IgM抗体阳性率高于非重度和（或）激素难治性UC组（P<0.05）,手术UC组血清CMV抗体阳性率高于非手术IBD组（P<0.01）,重度、激素难治性UC组CMV包涵体（HE染色）阳性率与非重度和（或）激素难治性UC组比较,差异无统计学意义（P>0.05）,重度、激素难治性UC组CMV包涵体（IHC）阳性率高于非重度和（或）激素难治性UC组（P<0.01）,手术UC组CMV包涵体（IHC）阳性率高于非手术IBD组（P<0.01）,重度或难治性IBD常合并或继发有CMV感染经适当的抗病毒治疗可明显改善病情。结论 血清学和组织学（特别是IHC） 联合检测可提高CMV感染诊断的准确性。在重症或激素难治性UC患者检测CMV是特别重要的,如果检测到CMV感染,更昔洛韦抗病毒治疗应立即开始,逐渐减弱激素剂量,并停用免疫抑制剂。     

IMA与SCUBE1联合检测在急性肺栓塞诊断中的价值

目的 探讨联合检测缺血修饰性白蛋白(IMA)与新型血小板活化蛋白血浆内皮生长因子样单链蛋白1相关补体Clr/Cls单肽(SCUBE1)在急性肺栓塞(APE)诊断中的价值.方法 收集2013年6月至2017年3月邢台市第三医院呼吸科就诊的APE患者130例为病例组;同时选取同期健康体检者为对照组(48例).比较分析病例组和对照组血清中IMA和血浆中SCUBE1的水平,并用ROC曲线分析IMA、SCUBE1指标单独和联合应用对诊断APE的价值.结果 病例组患者IMA和SCUBE1水平均高于对照组,差异有统计学意义(P<0.05).IMA单独诊断APE的敏感度和特异度分别为66.92％、77.08％;SCUBE1单独诊断APE的敏感度、特异度分别为67.69％与72.91％;IMA与SCUBE1联合检测诊断APE的敏感度、特异度分别为76.92％和85.42％.IMA、SCUBE1指标单独及联合应用诊断APE的ROC曲线的曲线下面积分别为0.796、0.839和0.888.结论 血清IMA和血浆SCUBE1均可作为诊断APE的生物标志物,在APE诊断中具有重要价值,同时检测二者有利于APE的诊治.     

Lymphoplasmacytoid lymphoma elaborating lambda chain paraprotein with amyloid deposition in Sj?gren's syndrome.

A woman presented with painful enlargement of her parotid and submandibular glands. She was shown to have the previously unreported combination of idiopathic thrombocytopenic purpura, Sjögren''s syndrome, Hashimoto''s disease, and myasthenia gravis. Parotid gland biopsy and bone marrow examination showed the presence of a rare lymphoplasmacytoid lymphoma. There was amyloid deposition in the parotid glands, gums and on muscle biopsy. Immunohistochemical staining of the parotid lymphoma and amyloid was monotypic for lambda light chains, and there was also a lambda chain paraprotein. It is felt that the lymphoplasmacytic lymphoma was responsible for the light chain amyloidosis.     

Light chain deposition disease presenting with hepatomegaly: an association with amyloid-like fibrils

We report an unusual case of lambda light chain deposits without overt plasma cell dyscrasia. The clinical presentation was hepatomegaly without biochemical sign of renal involvement. Portal hypertension, spontaneous rupture of the spleen and fracture of the 12th thoracic vertebra occurred during the course of the disease. Ultrastructural studies showed that lambda light chain deposits were associated with amyloid-like deposits. This case suggests that light chain deposition disease and amyloidosis could be two expressions of the same disease.