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Background In the treatment of Rheumatoid arthritis (RA), early diagnosis and early treatment with tight control have become increasingly important with the advent of biological therapy. Ultrasonography (US) of the various joints enables the real-time evaluation of synovial hypertrophy, effusion and bone erosion, Power Doppler (PD) ultrasound is able to identify both subclinical synovitis and early erosive disease. Objectives The objectives of this study were to investigate whether the image of US at the operated joint reflect synovium histological findings or clinical indicators, and to compare the results in the patient treated by non-biological agent (NonBio) and biological agent (Bio) Methods RA related orthopaedic surgery was performed at 1287 joints including 17 shoulders, 160 knees, 96 elbows, 378 wrists, 364 fingers, 38 ankles and 234 toes during the period between January 2011 and December 2018 at our rheumatic center. Preoperatively, ultrasound evaluations were performed and grade of PD signal was determined at the part with the highest signal. PD signal consists 4 grades from grade 0 to 3. The operations were performed within one week after the ultrasound evaluations. Rooney score of the synovium histology, DAS28-ESR(4), MMP-3, CRP were investigated. Rooney score represents the histologic features in the synovium of RA. It includes 6 features i.e. synoviocytes hyperplasia, fibrosis, proliferating blood vessels, perivascular infiltrates of lymphocytes, focal aggregates of lymphocytes, diffuse infiltrates of lymphocytes. They were scored separately on a scale of 1-10.The treatments that the patients received included: biological agents (Bio) (n=389 30%), etanercept (ETN) (n= 102), tocilizumab (TCZ) (n=105), infliximab (IFX) (n=29), adalimumab (ADA) (n=28), certlizumab pegol (CZP) (n=12), golimumab (GLM) (n=56), and abatacept (ABT) (n=55), tofacitinib (TOF) (n=2). Results PD signal (0.97±0.88), DAS28 (3.23±1.13), CRP(0.5±1.25 mg/dL), MMP-3 (119.0±119.0 ng/mL) and Rooney score (23.5±7.7) in the patients treated with Bio were significantly lower than those (1.39±0.95, 3.66±1.07, 0.78±1.37 mg/dL, 146.3±150.0ng/dL, 28.5±9.26) in the patients treated with NonBio. Rooney score fibrosis (9.57±1.44) in patients treated with Bio was significantly higher than those (8.64±2.48) in patients treated by NonBio. Rooney score, synoviocyte hyperplasia (1.16±1.26) and three items of lymphocyte (1.59±2.91, 1.68±2.54, 1.11±2.20) in patients treated with Bio were lower than those (1.72±1.30, 3.79±3.56, 3.73±3.53, 2.40±2.98) in patients treated with NonBio. TCZ, ADA, ABT and IFX had some significant differences for Rooney score and Rooney item score between the patients treated with NonBio. Conclusion The activity of RA synovitis at operated site was suppressed in patients treated with Bio. There were some differences in clinical data, histological score, PD signal and DAS28 among Bio. Disclosure of Interests None declared

Methotrexate (MTX) is the first choice disease-modifying antirheumatic drug (DMARD) for rheumatoid arthritis (RA) and is referred to as an "anchor drug"; its use has been steadily increasing annually. However, MTX-related lymphoproliferative diseases (MTX-LPDs) have emerged as important complications in the patients with RA. There have been no reports of intra-articular MTX-LPDs of the patients with RA. Atypical cells were found in the patient's joint fluid by cytological examinations, and MTX-LPDs were suspected. The patient discontinued MTX and open synovectomy was performed. The histological findings and immunohistochemical staining of the specimens confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL) of MTX-LPDs. After the operation of the patient's left knee joint, pains and swollen joint disappeared with no relapse. The cytological examinations of the synovial fluid followed by knee operation were effective for early diagnosis of MTX-LPD. MTX discontinuation with no chemotherapy followed up with a knee operation improved the recovery of the MTX-LPD.

We report two cases of TAFRO syndrome, which is characterized by thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly. Magnetic resonance imaging (MRI) of the spine showed a dark medullary pattern in the bone marrow on the T1- and T2-weighted images of both patients. One patient showed complete resolution after treatment. Serial MRIs of the improved patient revealed a transition to a normal marrow pattern on both images, which might represent resolution of the disease.

An 80-year-old man was diagnosed with disseminated intravascular coagulation (DIC) and thrombotic microangiopathy (TMA) associated with mucin-producing gastric cancer with bone marrow metastasis. He died suddenly on the third day of hospitalization before chemotherapy. Microscopic autopsy findings revealed fibrin thrombi by phosphotungstic acid hematoxylin (PTAH) staining of the renal glomeruli, and platelet thrombi by von Willebrand Factor (Factor VIII Antigen) staining of the microvessels of the bleeding intestine. Tumor cells were negative for both stains. Staining of endothelial cells (EC) of the small vessels with thrombomodulin (TM) stain revealed destruction of EC structure. This patient was thought to have had systemic dissemination of solid tumor cells associated with DIC and TMA, the clinical course of which is extremely aggressive. Different types of thrombi were observed in different organs, such as the kidneys and small intestine, which supported the co-occurrence of DIC and TMA by microscopic pathological findings. These findings provide pathological evidence for the pathology of the concurrent development of DIC and TMA and show differences in the types of thrombi according to the blood vessel localization. Furthermore, the findings were highly suggestive of the mechanisms causing organ dysfunction, such as renal dysfunction, and gastrointestinal bleeding.

症例は83歳，女性．83歳から慢性腎不全のため，血液透析を導入．透析導入時の血液検査で，高ガンマグロブリン血症と抗核抗体320倍を認めたが，その他自己免疫疾患を示唆する所見は認めなかった．また，慢性腎不全の原疾患は不明であった．週3回の外来血液透析を継続中，平成20年6月末から水様性下痢（5～6行/日）が出現．整腸剤，塩酸ロペラミド，バンコマイシンなどの内服を行ったが，下痢は慢性再発性に出現を繰り返していた．下痢に伴う低アルブミン血症，浮腫を認めた．それまで服用していたランソプラゾールを塩酸ラニチジンに変更したが，下痢は出現を繰り返し，大腸内視鏡検査を行った．肉眼では異常所見を認めずほぼ正常の粘膜所見であり，横行結腸からの粘膜生検で粘膜表層部上皮直下にエオシン好性で，マッソントリクローム染色では25～35μm幅の沈着物を帯状に認め，粘膜固有層全層に小型リンパ球，形質細胞といった慢性炎症性細胞浸潤に加え，好酸球浸潤が目立ち，collagenous colitisと診断した．塩酸ラニチジンを中止後，下痢は消失し，低アルブミン血症も徐々に改善した．Collagenous colitisは1976年にLindstromによって報告され，慢性水様性下痢，肉眼的にほぼ正常な大腸所見，特徴的組織所見を有する臨床的症候群である．欧米では数百例の報告があり，慢性水様性下痢の原因として決して稀ではないと報告されている．本邦での報告例はまだ少ないが，年々増加傾向にある．病因は不明だが，自己免疫や薬剤の関与などが考えられている．本例も薬剤中止後に下痢が消失したことから，その関与が考えられた．透析患者での報告はまだないが，慢性下痢の鑑別疾患の一つとして念頭に置くことが必要と考え，報告した．

The objective of this study is to investigate whether ultrasonography (US) images of joints that underwent surgery reflected the synovial histological findings or clinical indicators and to compare the results of the findings related to large joints (LJs) with those of small joints (SJs).The operations were performed on 215 joints in 177 patients with rheumatoid arthritis (RA). The 215 joints included 64 LJs and 151 SJs. The joints with the power Doppler (PD) signal grades 0 and 1 were assigned to group L, while those with grades 2 and 3 were assigned to group H. The Rooney score, Disease Activity Score-erythrocyte sedimentation rate (DAS28), serum matrix metallopeptidase 3 (MMP-3), and C-reactive protein (CRP) levels were determined.The Rooney score, DAS28, MMP-3, and CRP levels of the LJs were significantly lower in group L than in group H. In group H, similar results were found in the LJs and SJs, with a significant increase in the disease activity, CRP and MMP-3 levels and the histological findings in comparison to group L.The PD signal grade was one of the indicators that reflected the degree of synovitis in the histological findings of the active joints of RA patients.

症例は75歳の男性で, 下血を主訴に入院した. 十二指腸第二部乳頭対側の粘膜下腫瘤からの出血の診断で内視鏡的止血術, 血管造影下塞栓術を試みたが止血困難であり, 緊急手術となった. 十二指腸部分切除にて腫瘤を切除し, 術中迅速病理検査で十二指腸壁内の迷入腺管から発生した高分化型腺癌と診断されたため, 幽門輪温存膵頭十二指腸切除を追加施行した. 術後の免疫組織学的検索にて異所性膵より発生した膵癌と診断した. 異所性膵に発生した悪性腫瘍の報告は非常にまれであり, 術前に正確な診断をつけることは困難である. 異所性膵の手術においては悪性化の可能性を念頭におき, 術中迅速病理検査を行い手術方針を決定することが必要である.

The site of methylguanidine (MG) production in the kidney was investigated using animal models of renal disease and cultured renal epithelial cells. In rats with proximal tubular injury induced by adenine, the blood and urinary levels of MG increased as the severity of injury increased. In contrast, in cases of glomerular injury, there were no such changes in MG levels. Thus, it was apparent that proximal tubular injury served to promote MG production. In addition, a marked increase was observed in the intensities of bands attributable to 5,5-dimethyl-1-pyrroline-N-oxide (DMPO)-OH in the electron spin resonance spectrum of the kidney in the rats given adenine. In these rats, the activity of the radical-scavenging enzymes superoxide dismutase, catalase, and glutathione peroxidase was decreased. This suggests that the formation of excessive radicals and deterioration of defense mechanisms that contribute to the development of oxidative stress underlie the enhanced MG production. The experiments using cultured cells revealed that an oxide of adenine, 2,8-dihydroxyadenine (DHOA), directly induced renal tubular injury. These findings indicate that the accumulation of creatinine due to DHOA, combined with oxidative stress, resulted in increased MG production.

A pCW vector harboring rat liver serine dehydratase cDNA was expressed in Escherichia coli. The expressed level was about 5-fold higher in E. coli BL21 than in JM109 cell extract; the former lacked two kinds of proteases. Immunoblot analysis revealed the occurrence of a derivative other than serine dehydratase in the JM109 cell extract. The recombinant enzyme was purified to homogeneity. Staphylococcus aureus V8 protease and trypsin cleaved the enzyme at Glu-206 and Lys-220, respectively, with a concomitant loss of enzyme activity. Spectrophotometrically, the nicked enzyme showed a ∼50% reduced capacity for binding of the coenzyme pyridoxal phosphate and no spectral change of circular dichroism in the region at 300–480 nm, whereas circular dichroism spectra of both enzymes in the far-UV region were similar, suggesting that proteolysis impairs the coenzyme binding without an accompanying gross change of the secondary structure. Whereas the nicked enzyme behaved like the intact enzyme on Sephadex G-75 column chromatography, it was dissociated into two fragments on the column containing 6 m urea. Upon the removal of urea, both fragments spontaneously refolded. These results suggest that serine dehydratase consists of two folding domains connected by a region that is very susceptible to proteases.

Synovial tissue specimens obtained from the knee joints of 40 patients with rheumatoid arthritis (RA) and from 22 patients with osteoarthritis (OA) were examined histologically. The histopathological features of RA synovitis and OA synovitis were then compared. Seven criteria items of histopathological features characteristic to RA synovitis were given a score of 1-3 points each in order to evaluate the histological severity of the seven items. Their total scores were then calculated. A comparison of the total RA synovitis score and the total OA synovitis score revealed that RA synovitis showed more than 11 points (maximum 20 points), while OA synovitis showed less than 10 points in all but two cases. Furthermore, the total scores of RA synovitis were then determined in the same manner for other joints, where it was confirmed that five other joints had scores of more than 11 points as well; that is, the intercarpal, wrist, elbow, ankle and hip joints in 52 patients with RA. From these results, it was concluded that in the histological examination of biopsied synovial tissue of RA, if the total score for synovitis is more than 11 points (maximum 20 points), an histological diagnosis of RA synovitis can be confirmed.

上眼瞼に発生したメルケル細胞癌の1例を経験し, サイトケラチン20の有用性について他の2例のメルケル細胞癌を加えた3例で検討したので報告する. 症例は74歳, 女性, 農業. 上眼瞼腫瘍の捺印細胞診では, 小円形の均一な腫瘍細胞がシート状に多数出現していた. 核は円形～類円形で, N/C比大, クロマチンは細顆粒状で, 小さな核小体が数個認められた. 頸部腫瘤の吸引細胞診では, 小円形の腫瘍細胞はインディアンファイル様配列やモールディングをなし, 核の大小不同や核分裂像が認められた. なおcytoplasmic button-like globulesは明らかでなかったが, CK20による免疫染色では核近傍に滴状ならびに核周囲性の陽性所見を認めた. 免疫染色ではサイトケラチン (CK20, AE1), NSE, Chromogranin A, EMAが強陽性, Neurofilament, Synaptophysinは一部陽性, S-100蛋白, Vimentin, サイトケラチン (AE3), CEA, HMB 45, LCAは陰性だった。電顕像では細胞質内に少量の神経内分泌顆粒と, 核の傍に中間系フィラメントを, 細胞膜の一部にデスモゾーム様構造を認めた. 以上の所見からメルケル細胞癌と診断した. メルケル細胞癌と鑑別を要する他の8組織型の腫瘍における免疫組織学的検討の結果, CK20がび慢性に陽性を示すことがメルケル細胞癌の診断, 鑑別上有用であった.

Anomalies of the internal jugular vein are uncommon and not frequently experienced. Either a fusiform or saccular dilatation of the veins has been termed phlebectasia by some investigators and venous aneurysm by others. In 1928 Harris was the first to describe this condition using the term "congenital venous cyst." Relatively few cases of head and neck venous aneurysms have since been reported. We herein report a case of congenital internal jugular vein malformation while discussing both the diagnosis and pathology.

小児の右副咽頭にまれに発生する胎児型横紋筋肉腫の捺印細胞診を経験したので報告する.患者は1歳1ヵ月の男児で主訴は喘鳴.生検時の捺印細胞診Papanicolaou (Pap.) 染色では, 多数の腫瘍細胞が散在性にみられ, その多くは紡錘形および卵円形で, 一部にラケット状, おたまじゃくし状, 細胞質の不明瞭なものが存在した.クロマチンは増量し, 細胞質は厚みのあるライトグリーン好染色性で, ごく一部の腫瘍細胞に横紋構造が認められた.PAS染色では細胞質内に陽性穎粒を認め, H-E染色ではエオジン好性の細胞質がみられた.組織標本では, 大多数の細胞は紡錘形および卵円形で, それらに混じってラケット状の好酸性細胞質を有する細胞が散在性に出現し, 渦巻き状の流れをもって密に増殖していた.リンタングステン酸ヘマトキシリン (PTAH) 染色では一部の腫瘍細胞に横紋構造を認めた.免疫組織学的に腫瘍細胞は, デスミン, ミオグロビンが強陽性を示し, α-平滑筋アクチン, ビメンチンは一部陽性, ミオシン, アクチン (HHF 35, sarcomeric) は陰性だった.電子顕微鏡的所見では横紋筋芽細胞やZ帯を有するミオフィラメントの所見を認めた.以上の所見より, 比較的低分化の胎児型横紋筋肉腫と診断し, 化学療法および放射線照射後の生検から4ヵ月目に腫瘍摘出術を施行.手術標本では腫瘍細胞は紡錘形, 卵円形のものが消失し, 横紋筋芽細胞からなる分化型のものだけになっていた.

A significant increase in high-density lipoprotein-cholesterol and a tendency for total cholesterol and low-density lipoprotein-cholesterol to decrease were found in parous rats given Hokoei-to orally for 25 days, resulting in improvement of the atherogenic index. In experiments using cells of vascular origin, significantly increased proliferation of endothelial cells and suppressed proliferation of smooth muscle cells were obtained in the presence of Hokoei-to. When the action of each component galenical of Hokoei-to was examined, all five components, i.e. Taraxaci Radix, Angelicae Radix, Cyperi Rhizoma, Moutan Cortex and Dioscoreae Rhizoma, promoted the proliferation of endothelial cells. However, Taraxaci Radix and Angelicae Radix also promoted the growth of smooth muscle cells, showing inconsistency with the effect of Hokoei-to as a whole.

Experimental sensitization by repeated intramuscular injection of Ascaris suum antigen (Ag-As) supplemented with Freund's incomplete and complete adjuvants was carried out in 50 BALB/c CrSl c male mice (sensitized group) for 24 weeks, and the results were compared with those in a control group of 25 mice. At the injection sites of the sensitized group, granulomatous angiitis with eosinophil infiltration was observed in all mice, and fibrinoid angiitis in only four. By light and electron microscopic examinations pulmonary granulomatous vasculitis with a few eosinophils was observed at a high frequency (80%) after 12 experimental weeks. Immunohistochemical examination revealed pulmonary vascular and perivascular infiltration of L3/T4 (CD4) positive cells, B cells, IgG and C3 positive cells in addition to activated macrophages, Thy-1 T cells, IgE positive cells, and IgM positive cells after 12 experimental weeks. There were significant increases in the eosinophil cell count of the peripheral blood, the hemagglutination titers of the sheep erythrocytes, IgE and IgM antibodies to Ag-As by ELISA and Western blotting after 8 experimental weeks. After 12 experimental weeks the IgG antibody to the Ag-As was low, but it increased significantly, and the sera showed multiple precipitation lines to the Ag-As by the Ouchterlony method. In conclusion, the pulmonary granulomatous vasculitis in this study is considered to consist of allergic reactions of type IV and probably type III based on type I.

Adherent cells derived from human palatine tonsils were isolated and cultivated. Exponentially growing adherent cells (TAC) were observed by phase-contrast microscopy and transmission electron microscopy. Immunocytochemical studies were also performed. TAC were composed of relatively monotonous cells with polygonal or spindle shapes and high proliferative activity. In addition to the development of rough endoplasmic reticulum and lysosomes, the TAC possessed a moderate amount of pinocytotic vesicles and a few microfilaments. All of the TAC strongly expressed fibroblastic markers and partial monocyte/macrophage markers, such as beta-subunit of prolyl 4-hydroxylase (DAKO-fibroblast), lysozyme, anti-alpha-1-antichymotrypsin (alpha ACT), and CD68 (KP-1, EBM/11). It was noted that, as the TAC were cultured for a longer period, they gradually increased the reactivity with the monoclonal antibody PG-M1. Furthermore, the TAC expressed myocytic phenotype, such as alpha-smooth muscle actin (alpha SMA) with various intensity. Moreover, as to extracellular matrix, TAC stained for collagen type I, collagen type III, laminin, and fibronectin. Collagen type IV was weakly positive. The results presented here showed that the TAC expressed three different phenotypes of fibroblasts, histiocytes and smooth muscle cells at the same time. The monoclonal antibody raised against the TAC reacted strongly with the subendothelial pericytes and/or smooth muscle cells in the extrafollicular area in human tonsils. The present results also suggested that the origin of the TAC was probably subendothelial pericytes and/or smooth muscle cells of the microvasculatures in the tonsil.

Wistar rats (4-week-old) were administered with streptozotocin (45 mg/kg) through tail veins. After 3 months, diabetic rats were divided into 2 groups. One group (EPA group, n = 16) was fed a lipid-free diet (90%, w/w) plus lard (8%) and 90% pure eicosapentaenoic acid ethyl ester (2%) for 6 months. The other group (control group, n = 16) was fed in the same way except that eicosapentaenoic acid ethyl ester was replaced by safflower oil. Twenty-four-hour urine was collected just before starting the experimental diets and during the 6-month experimental period at monthly intervals. There were no differences in food intake and body weight between the two groups throughout the experiment. The mean microalbuminuria of the EPA group became significantly lower than that of the control group after 4 months on the diets through the end of the study (6 months). The mean microalbuminuria levels at the end of the study were 1.38 mg/day in the EPA group (n = 9) and 5.19 mg/day in the control group (n = 6) (p < 0.01). Eicosapentaenoic acid administration might retard the progression of diabetic nephropathy by reducing microalbuminuria.

It is relatively rare that schwannomas arise in the vagal trunk of the neck or brachial plexus. This paper presents a case of heterochronous schwannomas arising in the left vagal trunk of the neck and brachial plexus. A 52-year-old woman was seen at the hospital because of enlarging left subclavicular tumor with a spontaneous pain, which was noticed 5 years before. There was a history of undergoing enucleation of a schwannoma which occurred in the left left vagus nerve on the neck 17 years earlier. On palpation the tumor was elastic hard and hen's egg-sized. CT, ultrasonography, and needle aspiration biopsy revealed that the tumor was a schwqnnoma of the left brachial plexus. Tumorectomy (intracapsular shelling out) was performed under general anesthesia. Postoperative examination revealed no further neurological deficit of the left upper extremity. Tumorectomy of the schwannoma from the brachial plexus can cause postoperative neurological deficit of the upper extremity. So it seems better to enucleate intracapsularly for thepostoperative quality of life.

Clear cell meningioma is a rare morphologic form of meningioma. This case report describes a very rare case of multifocal clear cell meningioma in the thoracic and lumbar spine. A 9 year old patient presented with back pain and popliteal pain, which were aggravated by trunk motion and knocking. Magnetic resonance imaging (MRI) suggested a meningioma at the L2 level. An intradural extramedural tumor attached to the cauda equina was surgically removed and pathological diagnosis was clear cell meningioma. The patient began to complain of back pain at the Th12 level 4 months postoperatively. Retrospective analysis of MRI before the first operation revealed the small tumors at Th12 and L5 levels; comparison with a new MRI showed the rapid growth in 4 months. Adjunctive surgical resection was carried out and the back pain disappeared. These tumors had the same pathological findings. In conclusion, in young patients with suggested meningioma in the lumar spine, clear cell meningioma should also be considered, and the possibility of multifocal occurrence and postoperative recurrence must be taken into account.

Injection of subtotally nephrectomized rats with streptozotocin produced metabolic abnormalities resembling diabetic nephropathy in humans. These abnormalities were hyperglycemia, hypoinsulinemia, azotemia, hypertriglyceridemia and hypercholesterolemia, accompanied with an increase in glycosylated protein. Extraordinary changes in the urinary excretion of glucose and protein were also observed in rats that received streptozotocin treatment after subtotal nephrectomy. In addition, the level of creatinine clearance was significantly decreased. The pathological findings in the kidney of these rats revealed lesions of the glomerular capillary loops, mesangial area and Bowman's capsule. Coagulation was also found in the glomerular capillaries. Our results suggest that this rat model would be useful for studies of diabetic nephropathy.

We report the characteristics of magnetic resonance imaging (MRI) of cystic intradural extramedullary spinal cord tumors (cystic neurilemmoma, epidermoid cyst, and enterogenous cyst). T1-weighted MRI enhanced with gadolinium-DTPA clearly demonstrated the rim morphology of these tumors. The comparison between the rim enhancement pattern and histopathological findings offered possible qualitative diagnosis of these cystic spinal cord tumors by MRI.

Three cases of lung carcinomas with unusual histologic appearances that have received little or no comment in the literature are presented. They were initially confused with malignant lymphoma because of a diffuse proliferation of relatively monotonous cells simulating large-cell immunoblastic lymphoma. In each case, the possibility of malignant lymphoma was excluded with confidence after the immunohistochemical study (leucocyte common antigen negative and cytokeratins positive), although with conventional microscopy several foci of cohesive groups of tumor cells were observed. The tumors were ranked at the clinical stage II or III when they were initially discovered, but all patients died of disease within 1 year. The present three tumors show an aggressive behavior and could be classified into a peculiar variant of 'large cell' carcinoma. It is necessary for surgical pathologists to have an idea of these variants of lung carcinoma in order to avoid erroneous diagnosis.

This paper describes a 72-year-old male patient with megacolon successfully undergone artificial anus This paper describes a 72-year-old male patient with megacolon successfully undergone artificial anus cerebral infarction and suffered from incontinence of urine and constipation since 4 years ago. In the resected specimen psuedomelanosis coli was found on mucous membrane but no organic stricture was shown. Histologic feature of the dilated sigmoid colon revealed a decrease in mumber and atrophy of nerve plexus, vacuolation of nerve cells, and hypertrophy of muscular tunics. Idiopathic megacolon is a relatively rare disease and 30 cases have been reported in Japan during the past 10 years. In this case his history of cerebral infarction and a decreased number and degeneration of nerve plexus of enteric wall suggested a possible denervation due to cerebral infarction. However, from two, facts, no organic lesion on the colon and a negative result in acetylcholine esterase stain by colon biopsy, a possibility of adult megacolon could mot be denied. Further accumulation of experience for this disease including neurological investigation might be essential.

A 34-year-old woman visited our hospital complaining of a rapid enlargement of masses at the right breast and right axilla, Biopsy of the breast revealed malignant lymphoma, and Br+Ax+Mj+Mn was performed. Histologically the tumor was a diffuse lymphoma in LSG classification, mixed type malignant lymphoma, and Pre-B-originated one. Postoperative VEPA and radiation therapies resulted in a transient remission, however, after 7 months she experienced a recurrence in the peritoneum, resisted various kinds of therapies, and died of general metastasis after 11 months. It might be diagnosed as systemic malignant lymphoma forming a mass in the breast. Malignant lymphoma originating in the mammary duct is relatively rare, and 121 cases have been reported in Japan. The avaraged age (47.7) when the patients first had the disease was slightly younger than that of brast cancer patients. The masses as many as about 1/4 of them were found in the right side C resion, 1/4 of the patients were accompanied by a rapid enlargement, and about a half of them had tumors with the maximum diameter of 5 cm or more. Operation + chemotherapy regimen was the most common. It has poorer prognosis than that of breast cancer. Moreover, a change in LDH appers to reflect the stage of the disease. Eleven cases originated in B cell alone have been reported up to now.

The eldest autopsied case (a 23-year-old man) of infantile form of cystinosis with uremia and myxoedema was reported. The cystine content per gram wet tissue of various organs was arranged as follows: the thyroid (4.61 mg), kidney (1.71 mg), eye (0.75 mg), spleen (0.65 mg), liver (0.49 mg), and brain (0.016 mg). In polariased light microscopy, cystine crystals were detected in the epithelial cells and epithelial lumina of both kidneys by Wollaston test, and foam cells with cystine crystals were histologically found in the reticuloendothelial and other organs, especially in the bone marrows. Moreover, electron microscopic findings revealed square, rectangular or lozenge-shaped small cystine crystal profiles in osmophilic dense bodies of the histiocytic cells and in the cytoplasm of the foam cells. In some of them, positive reaction for acid phosphatase activity was specifically localized at the periphery of the cystine crystal profiles.

An autopsy case of rheumatoid arthritis with active polyarthritis, systemic necrotizing arteritis, pleuritis, pericarditis, rheumatoid nodules in a few organs and a healing gastric ulcer was reported. Histologically, systemic necrotizing arteritis was characterized by vascular changes of the following three types: Granulomatous arteritis with a characteristic arrangement of mesenchymal cells forming a palisade around coagulation necrosis of media and some of them formed a rheumatoid nodule-like lesion in the wall (RA type); Fibrinoid arteritis very similar to the Kussmaul-Maier type periarteritis nodosa (PN type); and chronic arteritis with endarterial proliferation (Ep type). Although it is hard to distinguish arteritis of PN type from the Kussmaul-Maier type periarteritis nodosa, arteritis of RA type with rheumatoid nodule-like lesion in the wall may be interpreted as an extremely developed form of vasculitis in rheumatoid arthritis.

An autopsy case was presented of isolated myocarditis (Fiedler's myocarditis) which was probably affected by fatal generalized infection of 2 rarely encountered infective agents, namely, infectious mononucelosis and cytomegalic inclusion disease.

A left renal artery aneurysm was found in a 45-year-old normotensive man. In an attempt to evade the possible occurrence of aneurysmal rupture, aneurysmectomy in addition to left renal biopsy (first surgery) was performed. This vascular operation led to a virtually complete renal artery stenosis concomitant with the development of hypertension. The renin-angiotensin-aldosterone system and levels of plasma prostaglandins were also increased following this failed surgery. Reconstruction of the affected renal artery was technically so difficult that left nephrectomy was carried out (second surgery). Renal specimens obtained at the first surgery revealed no histological abnormalities. Discriminating histological findings of the kidney obtained at the second surgery were remarkable; hyperplasia of the juxtaglomerular cells producing renin and hyperplasia of the renomedullary interstitial cells which had pecularities similar to cells known to secrete renal prostaglandins. High levels of the renin-angiotensin-aldosterone system and plasma prostaglandins after the first surgery were reduced following the second surgery. It is suggested that acute constriction of the renal artery led to a hyperplastic change of the the juxtaglomerular cells and the renomedullary interstitial cells and stimulated an inappropriate release of renin and renal prostaglandins.

An immunopathological study was carried out on subcutaneous nodules (rheumatoid nodules) biopsied from 16 patients with rheumatoid arthritis. Sixteen rheumatoid nodules were histologically classified into three stages, the 1st stage (acute inflammatory stage, 3 cases), the 2nd stage (granulomatous stage, 10 cases), and the 3rd stage (scar-formed stage, 3 cases). In necrotic or granulation tissue of the nodules in the 3 stages, immunofluorescent analysis using FITC-labeled rabbit anti-human immunoglobulins, beta 1C, and fibrinogen serum gamma-globulins and FITC-labeled aggregated human IgG gave results that were strongly positive in the 1st stage, moderately positive in the 2nd stage, and only slightly positive or almost negative in the 3rd stage. Acute or chronic thrombotic endoarteritis was observed around rheumatoid nodules in 6 out of the 16 cases. Among them, 2 cases of the 1st stage showed acute thrombotic endoarteritis with marked infiltration of neutrophils in the wall, and 2 cases of the 2nd and the 3rd stages respectively showed chronic endoarteritis with organized thrombi.

An autopsy case with SLE suffering from Raynaud's phenomenon and pulmonary hypertension was reported. Histological examinations revealed systemically marked fibrous intimal thickening of arteries and arterioles with or without thrombus throughout the whole body, especially of the pulmonary arteries and arterioles. Pulmonary arterial changes in the present case were compared with those in 52 autopsied cases with SLE without pulmonary hypertension, but there were no cases with such marked arterial changes as the present case. In addition, the incidence of pulmonary thrombosis was significantly higher in the cases with Raynaud's phenomenon than the cases without this phenomenon. However, the relation between pulmonary hypertension and Raynaud's phenomenon, pulmonary thrombosis, fibrous pericarditis, or type of lupus nephritis in SLE could not be clarified with a significant difference. ACTA PATHOL. JPN. 34: 593–604, 1984.