Epstein-Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab.

Histopathologic findings in the lymph nodes of patients with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome are similar to those of idiopathic multicentric Castleman's disease (iMCD), but TAFRO syndrome is different from iMCD in how it can progress rapidly and be fatal. These patients present scarce lymphadenopathy and low immunoglobulin levels. We present a case of cutaneous and systemic plasmacytosis (C/SP) that caused TAFRO syndrome-like symptoms which were successfully treated with rituximab. A 67-year-old woman presented with fever and a pruritic skin rash. Numerous plasma cells were observed in the peripheral blood and imaging revealed organomegaly, anasarca, and generalized lymphadenopathy. Subsequently, she rapidly developed thrombocytopenia as well as renal and heart failure. She tested positive for the Epstein-Barr virus (EBV), elevated immunoglobulins, and C/SP, which are also atypical for TAFRO syndrome, thereby complicating the diagnosis. However, after using the Japanese TAFRO Syndrome Research Group diagnostic criteria, we promptly administered rituximab to treat the C/SP with TAFRO-like symptoms and saved her life. Finally, histopathological observations of the lymph node biopsy helped confirm EBV-positive hypervascular-type iMCD. Therefore, diagnosing TAFRO-like syndromes based on the Japanese diagnostic criteria and following the associated treatment even without a confirmed diagnosis is crucial to improving the patient outcomes.

[A case of juvenile gastric polyposis with gastric cancer successfully treated by laparoscopic total gastrectomy -review of 36 reported cases in Japan].

A 43-year-old woman was suffering from epigastric pain. Her gastroscopy revealed polyposis of the stomach, and her biopsy revealed a hyperplastic polyp. During the 18-month follow-up, the polyps proliferated, and the patient was referred to our institute for further investigation and treatment. A juvenile gastric polyposis diagnosis was made. She refused to have the surgery despite the fact that it was necessary due to the anemia and hypoalbuminemia she was experiencing. Endoscopic biopsy results revealed gastric cancer at a follow-up visit 2 years and 3 months later; thus, a laparoscopic total gastrectomy was performed. Pathological examination revealed adenocarcinomas that were scattered and well-differentiated, with hyperplastic polyps in the background. No lymph node metastasis was found. Despite the fact that juvenile gastric polyposis is a pathologically benign disease, there have been numerous case reports of surgery being performed due to anemia, hypoalbuminemia, or gastric cancer associated with the disease. When gastric cancers are discovered in cases of juvenile gastric polyposis, they are usually in an early stage, making them a good candidate for laparoscopic total gastrectomy.

[Lymphocytic colitis caused by medicine ingredient modification].

A 49-year-old woman was referred to our hospital for further evaluation and treatment of diarrhea. Colonoscopic findings revealed indistinct vascular patterns and extensive edema in a colon segment, and white granular mucosa and crack-like appearance in the sigmoid colon and rectum. She was diagnosed with lymphocytic colitis (LC) based on lymphocytic infiltration into the epithelium on histopathological examination. Diarrhea symptoms resolved after long-term medication withdrawal. This medicine's composition was changed 4 years ago and this modification possibly triggered LC.

[A Case of Port Site Recurrence of Gallbladder Carcinoma after Laparoscopic Cholecystectomy].

The patient was a 76-year-old man who underwent laparoscopic cholecystectomy with a diagnosis of cholecystolithiasis. Since the definite diagnosis of gallbladder carcinoma was made after the operation, he underwent additional resection. Four years later, he revisited the hospital complaining of painful swelling of the port site of his epigastrium. Magnetic resonance imaging of the abdomen revealed a mass 3.4 cm in diameter at the epigastrium. Thus, port site recurrence of gallbladder carcinoma was the suspected diagnosis. We resected the peritoneum, rectus abdominis muscle, and skin, as well as the tumor, and the abdominal wall was reconstructed using synthetic composite mesh. Histological examinations revealed recurrence of gallbladder carcinoma. Port site recurrence of gallbladder carcinoma is known to have a poor prognosis, but long survival can be expected in patients after complete resection of the metastatic lesion, if the recurrence develops more than a year after the initial operation.

Salvage surgery after chemotherapy with S-1 plus cisplatin for α-fetoprotein-producing gastric cancer with a portal vein tumor thrombus: a case report.

BACKGROUND: Patient with α-Fetoprotein (AFP)-producing gastric cancer usually has a short survival time due to frequent hepatic and lymph node metastases. Gastric cancer with portal vein tumor thrombus (PVTT) is rare and has an extremely poor prognosis. CASE PRESENTATION: A 63-year-old man was found to have a huge Type 3 gastric cancer with a PVTT and a highly elevated serum AFP level. Chemotherapy with S-1 plus cisplatin was given to this patient with unresectable gastric cancer for 4 months. The serum AFP level decreased from 6,160 ng/mL to 60.7 ng/mL with chemotherapy. Since the PVTT disappeared after the chemotherapy, the patient underwent total gastrectomy. Histological findings of the primary tumor after chemotherapy showed poorly differentiated adenocarcinoma without hepatoid cells and viable tumor cells remaining in less than 1/3 of the neoplastic area of mucosa and one lymph node. The cancerous cells were immunohistochemically stained by anti-AFP antibody. The patient has survived for 48 month without recurrence. CONCLUSIONS: AFP-producing gastric cancer with a PVTT has an extremely poor prognosis, but long-term survival was achieved for this dismal condition by salvage surgery after chemotherapy.

[Vascular Calcification - Pathological Mechanism and Clinical Application - . The significance of arterial calcification in unstable plaques].

Plaque rupture or erosion with subsequent thrombus formation is the principal mechanism underlying the sudden onset of acute coronary syndromes. Plaque inflammation and increased oxidative stress play important roles in the pathogenesis of plaque destabilization. Macrophages, T lymphocytes, and neutrophils are the dominant types of inflammatory cells at human coronary unstable plaques, such as ruptured plaques or eroded plaques. Calcification is a common finding in human atherosclerotic lesions, and arterial calcification is generally classified into calcification within an atherosclerotic plaque, and Mönckeberg's medial calcific sclerosis characterized by calcific deposits within the media of small and medium-sized muscular arteries. It has been reported that a spotty pattern of calcification is associated with coronary unstable ruptured plaques in patients with acute myocardial infarction. Patients undergoing hemodialysis (HD) have a high prevalence of arterial calcification and cardiovascular events. We recently demonstrated that plasma oxidized low density lipoprotein (LDL) levels significantly increased after a single HD session. This HD session-related increase in plasma oxidized LDL levels could contribute to the progression and acceleration of atherosclerosis and arterial calcification, leading to the development of cardiovascular events in HD patients.

Myeloperoxidase and progression of aortic valve stenosis in patients undergoing hemodialysis.

BACKGROUND AND AIM OF THE STUDY: Currently, there is an increased incidence of aortic valve stenosis (AS) in patients undergoing hemodialysis (HD), though the exact mechanisms are not fully understood. Myeloperoxidase (MPO) is a leukocyte-derived enzyme that catalyzes the formation of reactive oxygen species and is an index of oxidative stress. The study aim was to examine, immunohistochemically, the expression of MPO, using surgically resected aortic valve specimens from AS patients undergoing HD. METHODS: The study population consisted of 15 HD patients and 19 non-HD patients with severe AS undergoing aortic valve replacement. Frozen aortic valve samples obtained surgically from AS patients were stained immunohistochemically with antibodies against smooth muscle cells, neutrophils, macrophages, T lymphocytes, CD31, MPO and 4-hydroxy-2-nonenal (4-HNE). RESULTS: Quantitative analyses showed that the macrophage-positive area, and numbers of T lymphocytes, neutrophils, CD31-positive microvessels and MPO-positive cells in HD patients were significantly higher than in non-HD patients (macrophages, p < 0.0001; T lymphocytes, p < 0.0001; neutrophils, p < 0.0001; CD31, p < 0.0001; MPO, p < 0.0001). Moreover, the number of MPO-positive cells was positively correlated with CD31-positive microvessels and the 4-HNE-positive macrophage score (CD31, R = 0.73, p < 0.0001; 4-HNE, R = 0.49; p < 0.005). CONCLUSION: These findings suggest that MPO is highly expressed in the aortic valves of AS patients undergoing HD. Furthermore, MPO is positively associated with neovascularization and oxidative stress, which contribute to a rapid progression of AS in HD patients.

Resolution of acute photoreceptor damage as revealed by serial SD-OCT.

PURPOSE: To describe findings in spectral-domain optical coherence tomography (SD-OCT) for four patients with acute foveal photoreceptor damage. CASE REPORT: Four patients with acute foveal photoreceptor damage were evaluated by color photography and SD-OCT during follow-up. All four cases were Japanese patients with high myopia. Three were women aged 28, 29, and 46 years and one was a boy aged 17 years. The follow-up ranged from 2 weeks to 3 months. On presentation, four eyes had orange-yellow foveal granularity with visual acuities that varied from 1.2 to 0.5. Spectral-domain optical coherence tomography revealed a disrupted photoreceptor layer. All patients achieved spontaneous resolution without treatment within a few months. Of the four eyes, only one had recurrence of the disease. CONCLUSIONS: Lesions exhibiting orange-yellow foveal granularity characteristic of these four cases corresponded to hyperreflective localized photoreceptor lesions on SD-OCT. Spectral-domain optical coherence tomography was useful for detecting an acute disruption and a resolution of the photoreceptor layer in the fovea.

Erythrocyte-rich thrombus aspirated from patients with ST-elevation myocardial infarction: association with oxidative stress and its impact on myocardial reperfusion.

AIMS: Recent studies have demonstrated that erythrocytes are a potential component in atheromatous lesions and thrombus formation in patients with ST-elevation myocardial infarction (STEMI). The purpose of this study was to determine the associations of red blood cell (RBC) component of coronary thrombi with oxidative stress and myocardial reperfusion. METHODS AND RESULTS: Aspirated thrombi from 178 STEMI patients within 12 h of symptom onset were investigated immunohistochemically using antibodies against platelets, RBCs, fibrin, macrophages, and neutrophils [myeloperoxidase (MPO)]. The thrombi were divided into tertiles according to the percentage of glycophorin-A-positive area: low (glycophorin-A-positive area <33%; n = 60), intermediate (<54 to 33%; n = 59), and high group (≥54%; n = 59). We also measured plasma MPO levels on admission. In the thrombi, the number of MPO-positive cells in the high-RBC group was significantly greater than that in the low-RBC group (high, 927 ± 385; intermediate, 765 ± 406; low, 279 ± 220 cells/mm(2); P< 0.0001). Plasma MPO levels were significantly higher in the high-RBC group than that in the low-RBC group [low 43.1 (25.0-71.6); intermediate 71.0 (32.9-111.2); high 74.3 (31.1-126.4)ng/mL; P< 0.005]. Distal embolization occurred more frequently in the high-RBC group (P= 0.0009). Moreover, the signs of impaired myocardial reperfusion, as indicated by incomplete ST-segment resolution (STR) and lower myocardial blush grades (MBG), and progression of left ventricular remodelling at 6 months were frequently observed in the high-RBC group (high vs. low: STR, P= 0.056; MBG, P< 0.01; remodelling, P< 0.01). CONCLUSION: The present study demonstrated that erythrocyte-rich thrombi contain more inflammatory cells and reflect high thrombus burden, leading to impaired myocardial reperfusion in STEMI patients.

Relationship between oxidative stress and aortic valve stenosis in humans: an immunohistochemical study.

BACKGROUND: Oxidative stress contributes to plaque formation and the destabilization of coronary atherosclerotic lesions. It has been reported that disease processes and clinical risk factors of aortic valve stenosis (AS) are similar to those of atherosclerosis. In this study, we immunohistochemically examined the expression of 4-hydroxy-2-nonenal (4-HNE), an oxidative stress-related molecule, by using surgically resected aortic valve specimens from AS patients. METHODS: The study was conducted using aortic valve specimens, surgically obtained from 24 patients with severe AS undergoing aortic valve replacement. We immunohistochemically investigated frozen aortic valve samples with antibodies against smooth muscle cells, macrophages, CD31 and 4-HNE. RESULTS: Morphometric analysis showed that the percentage of the macrophage-positive area and the number of CD31-positive microvessels were significantly higher in AS patients than those in reference cases (macrophages, p < 0.005 and CD31, p < 0.0001). Furthermore, the 4-HNE-positive macrophage score was also significantly higher in AS patients than in reference cases (p < 0.005). CONCLUSIONS: 4-HNE was expressed in the stenotic aortic valves in patients with severe AS, suggesting a close relationship between oxidative stress and the progression of calcific AS.

Pseudo dilated cardiomyopathy: Dilated cardiomyopathy-like changes due to a combination of stuck mechanical mitral valve and coronary microvascular dysfunction-An autopsy case.

Thrombus formation in the microvessels and endocardium was suggestive of endothelial cell damage, myocardial ischemia, and a decreased coronary flow reserve. Sustained pulmonary hypertension due to thrombosis worsened the biventricular dysfunction.

Cytomegalovirus Oophoritis Mimicking Burkitt's Lymphoma Recurrence: A Case Report and Literature Review.

Cytomegalovirus (CMV) oophoritis is an extremely rare and fatal condition. We encountered a 63-year-old woman with CMV oophoritis who had been treated for Burkitt's lymphoma. Positron emission tomography/computed tomography performed after chemotherapy showed a high 18F-fluoro-2deoxy-D-glucose uptake in both ovaries, which required distinguishing relapse. CMV oophoritis was diagnosed on histology following bilateral salpingo-oophorectomy. Although the patient later developed recurrent episodes of CMV antigenemia, after which complications of CMV retinitis appeared, and she ultimately died of CMV meningitis, surgical resection with antiviral medication resolved her abdominal symptoms and cleared CMV antigenemia for several weeks. It is therefore worth considering surgical resection in combination with antiviral drugs as a treatment option.

Acute myocarditis secondary to Campylobacter jejuni enteritis: A case report.

Acute myocarditis is a rare complication of Campylobacter jejuni enteritis. Herein, we report the case of a 20-year-old man who presented with chest pain that developed three days after the onset of enteritis. Electrocardiogram, echocardiogram, and cardiac enzyme levels suggested myocarditis. Cardiac magnetic resonance imaging revealed a late gadolinium enhancement in the inferior wall. Degeneration and necrosis of myocardial cells and lymphocyte-dominant inflammatory cell infiltration were found in the tissue obtained by endomyocardial biopsy. Acute myocarditis associated with C. jejuni enteritis was confirmed by these findings and C. jejuni detected in the stool culture. The symptoms of enteritis and myocarditis remitted 10 days after the onset. The left ventricular ejection fraction was improved from 40 % to 57 %.In previous cases, endomyocardial biopsy has not been performed because of mild myocarditis. The lack of pathological reports makes the mechanism of myocarditis associated with C. jejuni enteritis unknown. We report a case of myocarditis associated with C. jejuni enteritis, which was diagnosed using cardiac magnetic resonance imaging and endomyocardial biopsy. Learning objective: Acute myocarditis is a rare but important complication of Campylobacter jejuni enteritis. Cardiac magnetic resonance imaging is useful for diagnosis. Most cases of myocarditis associated with C. jejuni enteritis were mild and remitted without specific treatment. In the present case, endomyocardial biopsy was performed and CD4-positive lymphocytes were predominantly detected in the myocardial tissue.

Urethral metastasis from esophageal cancer: symptoms of dysuria and cystoscopic diagnosis.

Urethral malignant tumors are rare and can lead to stenosis, causing dysuria. We report a case of urethral metastasis secondary to esophageal cancer. At the time of diagnosis, a patient with esophageal squamous cell carcinoma presented with voiding difficulties, feeble stream, terminal dribbling and incomplete voiding. The urethral tumor was diagnosed using cystoscopy, and biopsy was thereafter performed. Histopathology of the urethral tumor microscopically resembled to that of esophageal cancer. On immunohistochemistry, the urothelium markers uroplakin 2 and GATA3 were negative in the carcinomatous component; however, GATA3 was detected on the lesion's surface. This case demonstrated that esophageal cancer metastasized to the urethra. Medical oncologists should consider this diagnosis in patients with cancer presenting with dysuria.

Pembrolizumab-related cholangitis with multiple fatal liver abscesses after endoscopic biliary drainage: a case report and review of the literature.

A 60-year-old male with cStage IVB lung cancer was treated with pembrolizumab. However, after five courses of pembrolizumab, he developed pembrolizumab-related cholangitis. Imaging studies showed enlargement and diffuse wall thickening of the gallbladder and mild dilation of the bile ducts without any obvious obstruction. As the patient experienced severe abdominal pain, we suspected bile stasis and performed biliary drainage. However, his condition did not improve, and he developed multiple liver abscesses and died during immunosuppressive therapy. Our case suggests that in ir-cholangitis, the indication and method of endoscopic retrograde cholangiopancreatography should be carefully judged.

Intraoperative liquid-based cytology of rinsed tissue fragments from head and neck lesions.

BACKGROUND: Intraoperative diagnosis is an essential tool for the rapid diagnostic assessment of clinically critical head and neck lesions. Thus, we assumed that a combination of frozen section histological and cytological diagnoses may be preferable. Here, we investigated a relatively new method called liquid-based cytology of rinsed tissue fragments (LBC-RTF) and compared this method with intraoperative histological diagnosis. METHODS: We used 68 tissue biopsies (9 brains, 8 lymph nodes, 22 salivary glands, and 29 thyroid samples). Samples submitted for intraoperative consultation were divided into two-halves: one was used to prepare frozen sections, and the other was prepared for LBC-RTF by washing with PreservCyt. We then compared the final diagnosis obtained from permanent sections with the intraoperative histological diagnosis based on frozen sections and examination of LBC-RTF preparations. RESULTS: The accuracy of LBC-RTF was higher than that of intraoperative histological diagnosis alone, based on frozen sections of every organ (LBC-RTF: 91.2% vs intraoperative histological diagnosis: 80.9%). With LBC-RTF, artifacts that are commonly observed in frozen sections were not present. In addition, even with challenging cases from which it is impossible to prepare frozen sections, intraoperative diagnosis was possible using the LBC-RTF technique. CONCLUSION: Both histological and cytological intraoperative diagnoses were possible during a surgery if the LBC-RTF technique was used. Moreover, our findings suggest that LBC-RTF improved the diagnostic accuracy of traditional intraoperative diagnosis.

Localized lymphadenopathy with myelodysplastic syndrome associated with tuberculosis.

We report the case of a man who developed myelodysplastic syndrome (MDS) and refractory cytopenia of unilineage dysplasia, 5 months after aortic valve replacement surgery. He also developed fever of unknown origin. After bone marrow- and other laboratory examinations, he was diagnosed with tuberculosis.

Intensity of SLX predicts distance of tumor spread through alveolar spaces in stage I lung adenocarcinoma.

BACKGROUND: Tumor spread through alveolar spaces (STAS) is a recently described invasive pattern associated with the prognosis and recurrence of lung adenocarcinoma. This study was performed to determine whether the presence and distance of STAS can be predicted by the immunohistochemical intensity of SLX, a well-known cell adhesion protein. METHODS: In total, 245 patients with pathological stage I lung adenocarcinoma who underwent lobectomy with radical mediastinal lymph node dissection were identified from 1998 to 2012. Recurrence-free survival (RFS) was compared between patients stratified by STAS and the immunohistochemical intensity of SLX in the main tumor. Patients were divided into three groups based on the intensity of SLX staining: high (n = 108), moderate (n = 48), and low (n = 89). RESULTS: STAS was observed in 71 patients (29.0%). Patients with STAS had significantly poorer five-year RFS (67.1%) than those without STAS (84.8%). Although no relationship was observed between the existence of STAS and SLX intensity, the distance between STAS cells and the main tumor was significantly shorter in the moderate group (median 0.9 mm, range: 0.2-1.2 mm) than in the other two groups (median 1.2 mm, range: 0.4-5.0 mm). The five-year RFS rates in the high, moderate, and low groups were 80.0%, 96.0%, and 75.8%, respectively. Multivariate analysis revealed that pathological stage, lymphatic/vascular invasion, and SLX intensity were independent predictors of recurrence. CONCLUSION: SLX staining cannot predict the presence of STAS; however, it can predict the distance between STAS and the main tumor in stage I lung adenocarcinoma.