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PubMed1983

[Systemic reactive histiocytosis with hemophagocytosis and hemostasis disorders associated with septic granulomatosis].

Authors describe a case of hemophagocytic systemic histiocytosis observed in a child suffering from chronic granulomatous disease, associated with septicemia due to Salmonella typhi murium; outcome was favorable. This type of histiocytic proliferation and activation usually induced by an infectious agent and combined with a congenital or acquired immunological disorder leads to severe clinical and hematological consequences which may contribute to a misleading diagnosis of malignant hematological disease. Moreover, systemic histiocytosis is constantly responsible for early blood clotting abnormalities concerning simultaneously coagulation and fibrinolysis; these changes have to be carefully examined before choosing either heparin or substitutive coagulant fractions.

Blood Coagulation Disorders↗
PubMed1984

[An autopsy case of malignant histiocytosis showing a phenotype of S100 +lysozyme].

A 37-year-old Japanese man was admitted to our hospital because of high fever. The peripheral blood showed pancytopenia with a few erythrophagocytic histiocytes. A bone marrow aspirate revealed 12% immature histiocytes and 7% leukoerythrophagocytic histiocytes. A postmortem examination showed an infiltrative proliferation of immature histiocytes and erythrophagocytic histiocytes in the sinusoid of the liver, the urinary bladder, etc. These findings were consistent with malignant histiocytosis. In addition, these abnormal histiocytes showed a phenotype of S 100 protein +lysozyme.- In agreement with Watanabe's report, our findings suggest that malignant histiocytosis is the neoplasm of T-zone histiocytes.

Adult↗
PubMed1996

[The role of P53 gene in the development of oral squamous cell carcinoma: study of P53 gene mutation by silver staining polymerase chain reaction-single strand conformation polymorphism].

It is widely accepted that the inactivation of P53 tumor suppressor gene is related to the tumorigenesis of many cancers. The study on P53 tumor suppressor gene has become a hotspot in molecular oncology. But until now, the reports on the research of the role of P53 gene in the tumorigenesis and prognosis of oral squamous cell carcinoma are scarce. So, two studies have been carried out. (1) A silver staining method of polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP) analysis was established by detecting the mobility shifts of the DNA bands of the products of PCR of mutant P53 gene plasmid and the Sup F mutants which had been proved to have point mutation by DNA sequencing in shuttle vector plasmid pS 189 mutagenesised by N-methyl-N-nito-N-nitosoguanidine (MNNG). For the method is non-radiation with high sensitive, it has applicable value. (2) By silver staining PCR-SSCP system, tissues from normal oral mucoas membrane to metastatic oral squamous cell carcinoma (OSCC) were analyzed and the results showed that tissues from 53% oral precancerous lesions and 65% OSCC had abnormal SSCP bands and the abnormal frequency of metastatic cancers was higher than that of nonmetastatic ones. These suggest that P53 gene mutation plays some role on the process of initiation, development and metastasis of OSCC.

Adult↗
PubMed1975

A clinical evaluation of indium-111 bleomycin as a tumor-imaging agent.

In an evaluation of indium-111-bleomycin as a tumor-imaging agent, 357 whole-body tumor scans were performed in 293 patients. Of 246 studies performed in patients with a variety of active solid tumors, 218 (89%) were true-positive studies and 28 (11%) were false-negative. Of 69 scans in patients thought to be free of tumor after therapy, 32 (46%) were false-positive studies and 37 (54%) were true-negative. The true-positive rates by major tumor type were: adenocarcinoma of gastrointestinal tract origin (95%), lymphoma (88%), melanoma (87%), sarcomas (82%), lung (77%), breast (77%), childhood tumors (71%), gynecologic tumors (70%), and genitourinary tumors (68%). Soft tissue and lymphatic sites of tumor, both above and below the diaphragm, were easily visualized, whereas hepatic and bone marrow sites of involvement were less easily discerned. False-positive uptake with 111In-bleomycin was noted in lungs (6%), gut (3%), mediastinum (2%), normal breast tissue (0.8%), and in occasional inflammatory lesions. In 19 patients with multiple myeloma or leukemia, a pattern of diminished bone marrow uptake associated with abnormal accumulation of 111In-bleomycin in extramedullary sites of involvement was the rule. In another 23 patients in whom scans were performed because an occult tumor was suspected, scanning did not lead to specific diagnosis of tumor in a single instance. We conclude that 111In-bleomycin is a safe, effective, and useful new tumor-imaging agent in the initial staging and followup of patients with a variety of solid tumors. Significant advantages of this agent over other currently available radiopharmaceuticals include: A) a broader spectrum of tumors taking up the radio-pharmaceutical, and B) generally better delineation of abdominal and pelvic disease due to lack of interference from gut uptake.

Adenocarcinoma↗
PubMed2000

Ultrasound-guided core needle biopsy for breast cancer: preliminary report.

BACKGROUND: Ultrasound-guided automated percutaneous core needle biopsy (US-CNB) for breast tumors has been introduced into clinical practice, but it has not yet been used routinely. We evaluated its usefulness, especially in terms of histological accuracy. METHODS: Thirty-one consecutive patients underwent mammography followed by breast biopsy with the automated core needle biopsy device. RESULTS: Mammography was highly suggestive of malignancy or suspicious abnormalities in 17 cases whose histological findings from US-CNB specimens were invasive ductal carcinoma without exception. The other 14 cases with benign or probably benign mammography findings showed no malignancy histologically in the US-CNB specimens. In cases of malignancy, the accuracy rates of histological findings for the specimens obtained by US-CNB were 94.1% in histological type, 100% in direct infiltration, 82.4% in lymphatic infiltration, 82.4% in venous infiltration, 94.1% in histological grading and 82.4% in intraductal spread. CONCLUSION: US-CNB was useful for making reliable preoperative histopathological diagnosis and may substitute fine needle aspiration biopsy and surgical biopsy.

Biopsy↗
PubMedDate not supplied

Human neurolymphomatosis in a patient with chronic lymphatic leukemia.

A 62-year-old woman with chronic lymphatic leukemia (CLL) (RAI stage IV) with multiple organ involvement and diabetes mellitus, three months prior to death presented with a symmetrical sensory neuropathy of the upper extremities with little motor impairment and, two months later, sensory atactic neuropathy of the lower limbs. No cranial nerve or CNS impairment was noted. Clinical diagnosis was predominantly sensory neuropathy, but nerve conduction velocities were normal on upper limbs and moderately abnormal on lower limbs, the latter attributing to long lasting diabetes mellitus. The women died from acute subarachnoid hemorrhage. Autopsy revealed CLL of B-cell type with generalized organ involvement and acute craniospinal subarachnoid hemorrhage from ruptured cerebral aneurysm. There was selective neoplastic infiltration of the dorsal root ganglia and peripheral nerves, particularly the median nerve. Although selective infiltration of peripheral nerves by B-cell lymphoma cells was not associated with myelo-axonal degeneration, the relationship of this case to human neurolymphomatosis is discussed.

Axons↗
PubMedDate not supplied

Identification of new Fas mutations in a patient with autoimmune lymphoproliferative syndrome (ALPS) and eosinophilia.

Autoimmune lymphoproliferative syndrome (ALPS) is a rare, newly recognized, chronic lymphoproliferative disorder in children and is characterized by lymphadenopathy, splenomegaly, pancytopenia, autoimmune phenomena and expansion of double-negative (DN) T lymphocytes (TCR alpha beta+, CD4-, CD8-). Defective lymphocyte apoptosis caused by mutations of the Fas (CD95) gene has been linked in the pathogenesis of ALPS, as binding of Fas-ligand to Fas can trigger apoptosis. Of the ALPS cases reported to date, point mutations, frameshifts and silent mutations in Fas all have been identified. We report two new point mutations in Fas in a child with ALPS and eosinophilia; studies on other family members established the pattern of inheritance for these mutations. Flow cytometric analysis of blood and tissues (spleen, lymph node, bone marrow) revealed abnormally expanded populations of DN T lymphocytes. Furthermore, activated lymphocytes and IFN gamma-activated eosinophils were resistant to Fas-mediated apoptosis. Eosinophil resistance to Fas-mediated apoptosis has not been previously described in ALPS. Sequencing of Fas revealed two separate mutations not previously reported. One mutation, a C to T change at base 836, was a silent mutation inherited from the mother, while the second mutation, a C to A change at base 916, caused a non-conservative amino acid substitution in the death domain of Fas, changing a threonine to a lysine. This mutation is associated with a predicted change in the structure of a part of the death domain from a beta-pleated sheet to an alpha-helix. We speculate that the mutation in the death domain prevents the interaction of Fas with intracellular mediators of apoptosis and is responsible for the autoimmune manifestations of ALPS and the abnormal lymphocytosis and eosinophilia in this patient.

Adult↗
PubMed2003

A case of primary tularemic pneumonia presenting with necrotizing mediastinal and hilar lymph nodes.

Tularemia is an unusual disease caused by the gram-negative coccobacillus Francisella tularensis. The clinical features of the disease depend on the route of inoculation. Ulceroglandular and typhoidal forms have been recognized as occurring in tularemia, however primary or secondary pneumonic infections have also been reported. Symptoms, laboratory markers and radiological features are non-specific in tularemic pneumonia. Diagnosis is made on clinical grounds and evidence of elevated agglutinating antibodies to F. tularensis (> 1:128). We report a case of primary tularemic pneumonia presenting with pulmonary infiltrates and necrotizing mediastinal and hilar lymph nodes in an otherwise healthy subject from a non-endemic area. Diagnosis of tularemia was obtained serologically, and antibiotic therapy with doxycycline and streptomycin resolved symptoms and radiological abnormalities. We suggest that tularemia should be considered in the differential diagnosis of pneumonia with mediastinal and/or hilar lymphadenopathy.

Aged↗
PubMed2001

Incidental malignancy in internal thoracic artery lymph nodes.

The incidental finding of malignant internal thoracic lymph nodes while mobilizing the internal thoracic artery (ITA) for coronary bypass grafting has not to our knowledge been previously reported. The cases of 3 male patients who underwent surgery between January 1990 and January 1993 and in whom malignant lymph nodes were found in the ITA pedicle are reviewed. One individual was found to have metastatic carcinoma of the breast, whereas the other 2 were discovered to have previously undiagnosed lymphomas. After undergoing further relevant investigation and treatment, all 3 patients remain free of recurrent disease 6.8 to 9.8 years after their original cardiac surgery. Primary or metastatic malignancy may be encountered in the course of ITA mobilization for grafting. Abnormally enlarged internal thoracic lymph nodes should be sent for pathologic examination.

Aged↗
PubMed2004

Accuracy of EUS criteria and primary tumor site for identification of mediastinal lymph node metastasis from non-small-cell lung cancer.

BACKGROUND: EUS with FNA is useful for staging non-small-cell lung cancer. However, benign mediastinal adenopathy is common. The aims of this study were to identify clinical factors, especially primary tumor location, and EUS lymph nodal characteristics predictive of aortopulmonary window and subcarinal lymph node metastases of non-small-cell lung cancer. METHODS: Patients with known or suspected non-small-cell lung cancer underwent EUS staging at which EUS-FNA was performed for all identified mediastinal lymph nodes. Clinical characteristics, primary tumor data, EUS findings, and histopathology were reviewed. Exact tests were performed for both aortopulmonary window and subcarinal lymph nodes to identify factors predictive of malignant cytology. RESULTS: Ninety-two patients with non-small-cell lung cancer were included. Fifty-one had aortopulmonary window, and 73 had subcarinal lymph nodes on EUS. The EUS with FNA specimens were interpreted as suspicious or diagnostic for malignancy for 9 aortopulmonary window and 9 subcarinal lymph nodes. When comparing benign vs. malignant EUS with FNA findings for aortopulmonary window and subcarinal lymph nodes, only lymph node size of 1 cm or greater and sharp lymph nodal edges were associated with malignancy in lymph nodes at both sites, whereas primary tumor site, lymph node shape, and echogenicity were associated with malignant subcarinal nodes. When 4 classic lymph nodal features of malignancy were evaluated, the presence of 3 or more typical features had positive and negative predictive values of, respectively, 41% and 96%. CONCLUSIONS: Although tumor location and EUS lymph nodal characteristics are associated with malignant involvement of lymph nodes, the accuracy of these predictors does not obviate the need for cytologic evaluation. EUS with FNA should be performed for all lymph nodes when an abnormal finding will alter management.

Carcinoma, Non-Small-Cell Lung↗
PubMed2000

Grey-scale sonography in assessment of cervical lymphadenopathy: review of sonographic appearances and features that may help a beginner.

Ultrasound examinations of the neck in 218 patients with confirmed cervical lymphadenopathy were reviewed. Lymph nodes were assessed for their size, shape, internal architecture, echogenicity, nodal border, posterior enhancement, and ancillary features (adjacent soft tissues oedema, and matting). The hilus is a linear, echogenic, non-shadowing structure containing nodal vessels, and is continuous with fat around the node. Coagulation necrosis is an ill-defined, rounded, non-shadowing echogenic area within a node. It is less echogenic than the hilus and is not continuous with the fat around the node. Calcification is a highly echogenic focus within the node, which may be dense or punctate echogenic foci. It is not continuous with the fat around the node. Dense intranodal calcification usually produces shadowing. However, fine punctate calcification may not have posterior shadowing though, if the transducer frequency is increased, it may show thin lines. Cystic necrosis is focal, often ill-defined echolucent area within the node. Echogenicity of lymph nodes is usually compared with the adjacent muscles, and is classified as hypoechogenicity, isoechogenicity, and hyperechogenicity. The nodal border is assessed for its sharpness. Posterior enhancement is when the structures posterior to the node look more echogenic than neighbouring areas. Oedema of soft tissues is an ill-defined, hypoechoic area around the node with loss of adjacent fascial planes. Nodes are considered matted when they are clumped or adherent to each other with no normal intervening soft tissue between them. Ultrasound features that help only in identifying abnormal nodes include size, shape, echogenic hilus, hypoechogenicity or isoechogenicity, echogeneity, coagulation necrosis, and a sharp nodal border. Ultrasound features that help to identify abnormal nodes as well as giving clues to the primary lesion include hyperechogenicity, intranodal calcification, intranodal cystic necrosis, ragged nodal border, posterior enhancement, adjacent soft tissue oedema, and matting.

Biopsy, Needle↗
PubMed1986

Defective regulation of Epstein-Barr virus infection in patients with acquired immunodeficiency syndrome (AIDS) or AIDS-related disorders.

Patients with the acquired immunodeficiency syndrome (AIDS) acquire undifferentiated B-cell lymphomas that are similar to African Burkitt's lymphoma and contain Epstein-Barr virus (EBV). Using an in vitro assay system that measures a complex of cellular responses to EBV-infected lymphocytes, we found that B cells from 7 patients with AIDS and from 10 patients with AIDS-related disorders produced abnormally low numbers of immunoglobulin-secreting cells (P less than 0.001 as compared with normal controls) and that T-cell suppression, which was greater than 80 percent in EBV-seropositive normal controls, was absent. Instead, the patients' T cells markedly increased immunoglobulin production induced by EBV. In further studies, we determined that the mean frequency of circulating EBV-infected B cells capable of spontaneous outgrowth in vitro was 13 per 10(6) B cells in 7 patients with AIDS and 21 per 10(6) B cells in 10 patients with AIDS-related disorders--figures that were significantly higher than the mean in normal controls (P less than 0.001). Thus, patients with AIDS or AIDS-related disorders may be predisposed to the development of EBV-containing lymphomas, because they have a profound defect of T-cell immunity to EBV and abnormally high numbers of EBV-infected B cells in the circulation.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

MR and MRCP in the evaluation of primary sclerosing cholangitis: current applications and imaging findings.

Primary sclerosing cholangitis is a progressive cholestatic disease of unknown etiology leading to cirrhosis and liver failure. Several imaging modalities have been used to study this disease, including ultrasonography, computed tomography and hepatobiliary scintigraphy, but accurate diagnosis was found to be best made with endoscopic retrograde cholangiopancreatography or direct cholangiography. However, these 2 methods are invasive and may produce serious complications. Magnetic resonance cholangiopancreatography is a noninvasive imaging technique that has become very useful for diagnosing primary sclerosing cholangitis. Contrast enhanced magnetic resonance imaging provides pertinent information of extraductal abnormalities in addition to biliary ductal changes.

Adult↗
PubMed1982

The throat culture in Kawasaki disease.

Recently, Kawasaki disease has been attracting more attention as a new acquired heart disease in pediatrics, but the etiology of this disease has still not been established. In this study, the authors performed throat cultures not only on the patients themselves but also on their parents in order to investigate its etiology. No abnormal and specific bacterium was found in the present study. However, there have been many reports which may suggest bacterial or viral involvement at the onset of this disease. Therefore, it may be necessary to continue bacteriological analysis more systematically.

Bacteria↗
PubMed1975

[Sézary's syndrome. Case report].

A patient with Sézary syndrome is presented. The symptoms persisted for 5 years when a sudden involvement of internal organs led to the death of the patient. Much evidence indicates that the Sézary syndrome is a special form of mycosis fungoides with erythroderma and a constant release of atypical cells into the blood. The nature of the Sézary cell has not yet been clearly elucidated. Cytogenetic and immunological findings identified the Sézary cell as an abnormal T-lymphocyte whereas cytochemical data support the concept of the presence of different atypical cell populations with lymphocytic respectively monocytic character.

Blood Cells↗
PubMed2005

Sickle cell anemia patient with sarcoidosis-associated inguinal lymph node and lung infiltration.

Sarcoidosis is a chronic, systemic inflammatory disease of unknown etiology, characterized by noncaseating granulomatous infiltration of any organ. Sickle cell anemia (SCA) is the homozygoid form of sickle cell disease (SCD), which includes a group of genetic disorders characterized by production of an abnormal hemoglobin S (HbS). There are a few case reports with coexistence of sarcoidosis and SCA. We reported a 47-year-old female with SCA and sarcoidosis.

Anemia, Sickle Cell↗
PubMed2005

[Expressions of Axin and beta-catenin in non-small cell lung cancer].

OBJECTIVE: To investigate the protein expression of Axin and beta-catenin, the exon 3 mutation status of beta-catenin and their clinicopathological correlations in non-small cell lung cancer (NSCLC). METHODS: A total of 100 NSCLC samples and their corresponding normal lung tissues were obtained from the patients undergoing surgery in the First Affiliated Hospital of China Medical University between 2001 and 2003. Protein expressions of Axin and beta-catenin were detected by immunohistochemistry. DNA sequence alterations of exon 3 of beta-catenin were investigated by polymerase chain reaction (PCR) and direct sequencing. RESULTS: A reduced membranous expression rate of beta-catenin was observed in 80.0% of the cases (80/100) along with a nuclear expression rate of 26.0% (26/100). There was a significant difference in beta-catenin expression between well and poorly differentiated NSCLCs. Well to moderately differentiated NSCLCs showed a reduced expression rate of 70.0% (35/50), in contrast to 90.0% (45/ 50) in poorly differentiated tumors (P = 0.012). Reduced beta-catenin expression rate was 87.3% (48/55) in cases with lymph node metastasis, in contrast to 71.1% (32/45) in cases without lymph node metastasis (P = 0.044). The positive expression rate of Axin was 48.0% (48/100). Well to moderately differentiated NSCLCs demonstrated a 60.0% positive expression rate of Axin (30/50), much higher than poorly differentiated tumors [36.0% (18/50), P = 0.016]. The positive expression rate of Axin in beta-catenin nuclear expressed NSCLCs was 15.4% (4/26), much lower than cases without beta-catenin nuclear expression [59.5% (44/74), P < 0.001]. Axin nuclear expression was found in two cases in this study, suggesting that it may function as a nuclear-cytoplasmic shuttling protein. PCR and direct sequencing failed to reveal any exon 3 mutation of beta-catenin gene. CONCLUSIONS: The reduced membranous expression of beta-catenin is associated with poorly differentiated and lymph node positive NSCLCs. The expression of Axin is inversely correlated with the degree of tumor differentiation and nuclear expression of beta-catenin. The exon 3 mutations do not contribute to the abnormal protein expression of beta-catenin in NSCLCs.

Adult↗
PubMed1991

[CT diagnosis in primary lung cancer (a correlative study of roentgenogram CT, surgical and pathological findings)].

A correlative study of roentgenogram, CT, surgical and pathological findings was investigated on 40 cases with primary lung cancer to evaluate differences between x-ray and CT of the chest in imaging pathological changes of lung cancer. The study revealed that CT was more sensitive than roentgenogram in showing lobulation, speculate and internal structure of SPN or masses, hilar and mediastinal adenopathy, invasion to neighbouring tissues and soft tissues of chest wall and abnormal pattern of the bronchus in cross section. Abnormal findings in CT was 67.4% higher than that in roentgenogram, CT provided more information in diagnosis of primary lung cancer. Some limitations of CT in diagnosis of primary lung cancer were also discussed.

Adenocarcinoma↗