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Results for “Lymphatic Abnormalities”

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PubMed2003

[Lymphocyte immunophenotyping and cytogenetics for more precise prognosis in patients with type B chronic lymphatic leukemia].

BACKGROUND: The B-chronic lymphocytic leukemia (B-CLL) has highly variable prognosis. Possibility of more relevant prognosis has a great impact for the beginning and mode of therapy. METHODS AND RESULTS: One hundred patients diagnosed as having B-CLL were included into the study. Beside usual examinations necessary to establish the diagnosis, cytogenetic examination for the detection of deletion 13q14, 17p13, 11q23 and trisomy 12 and immunophenotyping was done. The mean age of the patients was 58.2 years, there were 62 men and 38 women. 91 evaluated patients were divided into two groups--those with the steady disease (55 pts) and those with progressive disease (36 pts). No relation between the number of cytogenetic abnormalities and the Rai stage of the disease was found. We identified a relation between the bone marrow infiltration pattern (diffuse) and the number of cytogenetic abnormalities and the del 13q14 (p.05). Using the immunophenotyping of the lymphocytes we found a relation between the expression of CD38 and CD11c and the disease progression (p < 0.05). Neither of the method (FISH and immunophenotyping) revealed differences between results from bone marrow samples and those from peripheral blood. CONCLUSIONS: Though the cytogenetic (FISH) and immunophenotype evaluation at the time of diagnosis did not improve the ability to define better the clinical course of the B-CLL, we suggest to use both methods routinely as an important tool to identify patients who would develop the progressive disease.

Adult↗

PubMed1995

Multiple sclerosis: immune mechanism and update on current therapies.

Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS) afflicting approximately 250,000 individuals in the United States. This inflammatory disease has variable clinical manifestations, ranging from a relapsing-remitting course to a chronic progressive disease. Approximately one third of MS patients have chronic progressive disease often leading to severe impairment of mobility, paralysis, poor vision, and disturbances of bladder and bowel function. Although the etiology and pathogenesis remain unknown, accumulating evidence supports the hypothesis that exposure to an as-yet-unidentified infectious agent(s) triggers an aberrant immune response against self nervous tissue in genetically susceptible individuals. The tenfold higher concordance rate for MS in monozygotic twins compared to dizygotic twins, the increased incidence of MS in women compared to men (2:1), and the familial and racial occurrence of MS provide strong evidence that genetic factors influence susceptibility to MS. The major predisposing genes in MS are the human leukocyte antigen (HLA) class II molecules, DR15 and DQw6, molecularly defined as HLA-DRB1, 1501-DQA1 0102-DQB1 0602. In certain ethnic groups, MS susceptibility is more strongly associated with other DR molecules. Environmental factors are also believed to play a role, as suggested by the unique worldwide prevalence, migration effects, and epidemiological studies. Increased serum and cerebrospinal fluid antibody titers to numerous viruses have been reported; however, there have been no confirmed studies detecting viral RNA or antigen in MS brain tissue. At the present time, no known treatment can significantly alter the progression of MS. Based on the postulate that MS is an autoimmune disease associated with abnormalities in immunoregulation, a number of different immunosuppressive and immunomodulating agents have been tested as therapeutic modalities. In this article, we review the circumstantial evidence suggesting that immune system abnormalities are associated with the disease process, and provide an update on current therapies used in MS.

Animals↗

PubMed1988

Histopathological characteristics of the kidney in recombinant inbred mice established from MRL/lpr x AKR crossing. Dissociation of severity of lupus nephritis from the degree of lymphadenopathy.

Recombinant inbred (RI) lines were established from (MRL/lpr x AKR) crosses in order to analyze the role of the lpr gene and the participation of background genes in the lymphoproliferation and the development of lupus glomerulonephritis (LGN). In this study, six lines were used to compare with MRL/lpr and AKR mice. Lymphadenopathy was present in four lines (A-22, A-31 b, A-31 e and C-12) but absent in the other two (A-21 and C-21). The degree of lymphoproliferation varied between individuals of the RI lines showing lymphadenopathy. On gross examinations, the most marked lymph node enlargement was seen in the A-31 b line, which resembled MRL/lpr mice in this respect; lymphadenopathy was least prominent in the C-12 line and intermediate degrees occurred in the A-22 and A-31 e lines. Like MRL/lpr mice, deaths in the RI lines were due to LGN; however, in the lines with lymphadenopathy, 50% mortalities occurred a few weeks later than in MRL/lpr mice. The kidneys were examined histologically for proliferative, exudative, extracapillary and membranous changes in the glomeruli. The glomerular lesions in the A-22, A-31 b and A-31 e lines closely resembled those in MRL/lpr mice, but in the C12 line in which lymph node enlargement was least apparent, the histological abnormalities were significantly more severe. Of the lines without lymphadenopathy, histopathological examination showed obvious renal abnormalities in the A-21 line but none in the C-21 line or in AKR mice. From these findings it appears that there are autosomal genes which affect the expression of the lpr gene and thus modify the development of LGN and lymphoproliferation.

Animals↗

PubMed1997

Aggressive endometrial cancer in a young patient.

An unusually aggressive case of endometrial cancer in a 30 year old woman is presented. The patient experienced abnormal uterine bleeding, at times requiring blood transfusions, for almost half a year before the diagnosis was revealed. For obvious reasons there is a reluctancy to perform invasive examinations in young women. The diagnostic options are discussed.

Adenocarcinoma↗

PubMed1980

Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature.

Two patients with plasma cell dyscrasias, manifested by osteosclerotic bone lesions and small amounts of M protein, and a complicating multi-system disorder are described. Their features of severe sensory-motor polyneuropathy, organomegaly, endocrine dysfunction, anasarca, elevated CSF protein, and skin hyperpigmentation are similar to a clinical syndrome reported primarily in Japanese men. Two previously unrecognized findings--hyperprolactinemia and an unusual radiographic abnormality of fluffy, spiculated bony proliferation--may facilitate recognition of the syndrome. The relationship of these various manifestations to the plasma cell dyscrasia is unknown, but a number of possibilities are discussed.

Adult↗

PubMed1987

Pulmonary lymphangitic spread of carcinoma: appearance on CT scans.

Chest computed tomography (CT), including high-resolution CT with thin (1.5-mm) sections was used to evaluate proved (pathologically or clinically) lymphangitic spread (LS) of tumor in 12 patients. These appearances were compared with thin-section scans obtained in 11 healthy subjects. Thin-section CT demonstrated findings consistent with thickening of the normal lung interstitium. In all patients, thin sections showed an increase in the number of peripheral lines (1-2 cm in length) that were diffuse in generalized disease and localized in focal disease. Normal peripheral arcades were not increased in number, but the limbs forming the arcades were thickened in all patients. A diffuse increase in linear and curvilinear structures (reticular pattern) was seen toward the center of the lung. Polygonal structures 1-2 cm in diameter were seen in seven patients with LS but not in healthy subjects. Fissures were thickened in nine patients. Selected 1.5-mm-thick CT sections are recommended through abnormal areas (seen at CT or on chest radiographs) or if these are normal at three levels (midapex, hilus, and 3 cm above the diaphragm) when scanning patients with tumors known to cause LS.

Adult↗

PubMed2005

Xerostomia following radiotherapy of the head and neck affects vocal function.

PURPOSE: To investigate whether xerostomia induced by wide-field radiotherapy (RT) of the head and neck affects vocal function. PATIENTS AND METHODS: We conducted a retrospective cohort study comparing 20 patients with early glottic cancer treated by limited RT of the larynx to 20 patients receiving wide-field RT of the primary tumor site and the lymphatic system of the entire head and neck, including the salivary glands. Salivary and vocal functions, as well as responses to questionnaires on xerostomia and quality of life were compared between groups. Twenty healthy volunteers matched for age, sex, and smoking status were included as controls. RESULTS: The wide-field RT patients showed high xerostomia-related symptom scores and significantly lower values of whole salivary flow rate compared to the limited RT and healthy patients (P < .001). Subjective vocal dysfunction and stroboscopic abnormality were observed in the wide-field RT group (P < .05), but acoustic or aerodynamic profiles showed no significant difference among groups (P > .05). Subjective and objective salivary gland hypofunction was significantly correlated to vocal dysfunction. CONCLUSION: Our results suggest that xerostomia following extensive RT of the head and neck can affect vocal function. In the treatment of head and neck malignancies, efforts to prevent post-RT xerostomia would be anticipated to contribute to the preservation of vocal function.

Aged↗

PubMed1992

Microsurgical lymph node dissection for metastatic asymptomatic C-cell carcinoma.

In persistent, clinically inapparent medullary thyroid carcinoma, microsurgical dissection of all lymph node compartments of the neck was performed. Between August 1988 and September 1991, 28 cases (mean age 43.3 years) were treated with 38 surgical interventions. Twenty patients had the sporadic form and eight patients the familial form. Unilateral neck dissection resulted in normalization of serum calcitonin (CT) levels even after pentagastrin stimulation in two patients whereas 16 patients exhibited abnormal CT stimulation tests. Eight of ten patients who had bilateral neck dissections had positive pentagastrin test results after surgery. The main postoperative complications included loss of local cutaneous sensation, generally temporary, and unilateral recurrent laryngeal nerve paralysis.

Adolescent↗

PubMed2006

Relationship between E-cadherin, matrix metalloproteinase-7 gene expression and clinicopathological features in gastric carcinoma.

We investigated whether the abnormal expression of E-cadherin (ECD), in conjunction with the overexpression of matrix metalloproteinase-7 (MMP-7), is correlated with clinicopathological parameters such as metastasis and the prognosis for human gastric carcinoma. Using RT-PCR, we examined the expression of ECD and MMP-7 mRNA in 42 gastric carcinoma tissues and in the surrounding non-neoplastic mucosa. The macroscopic and histopathological tumor findings and the survival rates were obtained from the patient records. The level of ECD mRNA expression was lower in most of the neoplasms as compared to the corresponding non-neoplastic tissues (50 vs. 80.9%, respectively). The abnormal expression of ECD mRNA was significantly correlated to the microscopic classification and lymph node metastases (p<0.05), and its stage (p<0.05). The level of MMP-7 mRNA expression was higher in most neoplasms compared to the corresponding non-neoplastic tissues (66.6 vs. 50%, respectively). The overexpression of MMP-7 mRNA was significantly correlated with the microscopic classification, lymph node metastases (p<0.05), and its stage (p<0.01). However, a correlation between the abnormal expression of ECD and the overexpression of MMP-7 was not obtained. The survival rate of the patients with an ECD mRNA expression was longer than that of the patients without this expression, but this finding was not statistically significant (p=0.2162). The survival rates of patients with MMP-7 mRNA expression was significantly shorter than that of the patients without MMP-7 mRNA expression (p=0.0025). Overexpression of MMP-7 may be considered as a useful marker for determining metastasis and the prognosis of human gastric carcinoma rather than the abnormal expression of E-cadherin.

Adult↗

PubMedDate not supplied

[Anatomo-clinical features of Castleman tumour. Report of a case with a membrano-proliferative glomerulonephritis (author's transl)].

A case of retro-peritoneal lymphoid angio-follicular hyperplasia or Castleman's pseudo-tumour with normochromic hormocytic anemia and membrano-proliferative glomerulonephritis is reported in a 53 year-old man. Since the first description by Castleman in 1954, 259 other cases have been published. The anatomo-clinical study of our observation and of 198 cases collected in the literature bears out 3 types of clinical forms, according to the location of the pseudo-tumour: mediastinal, superficial and abdominal. The first ones generally asymptomatic are the most frequent whereas the last ones, less common, are often associated with biological abnormalities. In spite of these differences such neoformations present the same morphologic features. They are round or ovoid and well delimited by a capsule. Under this capsule a lymphoid vascular tissue surrounds follicules with central capillaries or arterioles. When plasma cells infiltrate the stroma, perturbations such as anemia or hypoalbunemia appear. Besides a nephrotic syndrome has been discovered in our observation and in that of Humpherys. In all the cases the removal of the pseudo-tumour induces the retrogression of the biological abnormalities and of the nephrotic syndrome. Neither a recidive nor another location of the disease are noticed later on. This evolution is probably the fact of a benign hyperplatic process which grows after an antigenic stimulation.

Abdomen↗

PubMed1984

Absence of lymphocyte ecto-5'-nucleotidase in infants with reticuloendotheliosis and eosinophilia (Omenn's syndrome).

Lymphocytes from three infants with reticuloendotheliosis and eosinophilia ( Omenn 's syndrome) and immunodeficiency were assayed for 5'-nucleotidase activity. B and T lymphocytes from all three patients were totally deficient in ecto-5'-nucleotidase activity, but had normal levels of cytoplasmic 5'-nucleotidase. In contrast, cultured lymphocytes expressed normal ectoplasmic and cytoplasmic activities, suggesting that the lymphocyte-restricted enzyme deficiency was not likely a primary genetic defect. The deficiency of lymphocyte ecto-5'-nucleotidase was not associated with any abnormality of deoxynucleoside metabolism. The absence of lymphocyte ecto-5'-nucleotidase may be a characteristic feature of this syndrome and may help to distinguish this disease from others with similar manifestations.

5'-Nucleotidase↗

PubMed1994

The role of intrarectal ultrasound (IRUS) in staging of rectal cancer and detection of extrarectal pathology.

Intrarectal Ultrasound (IRUS) is rapidly becoming an effective tool in the staging of rectal cancer. Twenty-nine consecutive patients with adenocarcinoma of the rectum underwent both CT scanning and IRUS in the preoperative assessment of rectal cancer in an effort to correlate IRUS staging with surgical pathology, correlate tumor staging comparing IRUS with CT scan, and determine incidence of extrarectal pathology by IRUS. Patients were reviewed as to IRUS stage, results of CT scan, TNM stage of extirpated tumor, incidence of genitourinary pathology, and sonographic result of preoperative radiotherapy (RT). The mean age of all patients was 69 years; there were 25 males and four females. Twenty-four patients underwent proctectomy with either low pelvic anastomosis or end stoma; five underwent local surgical therapy. Thirteen patients received preoperative RT. CT scan correlated poorly with IRUS staging of tumors penetrating the muscularis propria. IRUS overstaged 40 per cent, understaged 5 per cent, and correctly staged 55 per cent of patients when compared with pathological specimens. Eleven of the 25 males (44 per cent) had abnormal prostates by IRUS. Five (20%) had further urologic intervention, resulting in two prostatic cancers found. Our data suggests that CT scan staging correlated poorly with IRUS staging. CT poorly determines depth of rectal tumor wall invasion. IRUS correlated well with pathology and understaged 5 per cent of patients before surgery. Genitourinary abnormalities were detected in a significant number of patients. IRUS is an effective modality for preoperative staging of rectal cancer.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenocarcinoma↗

PubMed1998

Morphologic diversity in malignant melanoma: the potential use of microdissection and the polymerase chain reaction for diagnosis.

Malignant melanoma (MM) can mimic soft tissue (ST) and epithelial neoplasms. An immunoperoxidase (IP) panel and a morphologic comparison of the primary are used in diagnosis, which can be difficult when the morphologic and IP profiles of a metastatic lesion simulate those of an ST neoplasm. Through the comparison of known genetic abnormalities in primary and metastatic neoplasms, a definitive diagnosis can be suggested on the basis of the finding of identical allelic losses through the use of microdissection (MD) and the polymerase chain reaction (PCR). Genetic alterations involving the p16 gene on chromosome 9p21 have been observed in MM. We present the case of a 56-year-old man with known MM in whom multiple metastatic lesions to the skin and an adrenal gland developed during a 5-year period. A fine-needle aspiration (FNA) of a new ST buttock lesion was performed; the specimen had cytologic features different from those of the primary neoplasm and simulated a possible primary ST neoplasm. We attempted to make a definitive diagnosis of MM in the FNA of the ST buttock lesion through a genetic comparison with the primary neoplasm as well as with the other metastatic sites. Direct-visualization MD was performed on histologic glass slides of the primary and adjacent tissue (normal control), and the metastatic lesions, along with malignant cell clusters from the buttock lesion FNA. DNA was extracted and PCR amplified with primers D9S171 and IFNA for the p16 locus at the 9p21-22 region. Loss of heterozygosity for the D9S171 marker at the p16 gene locus was identified in all of the neoplastic tissue tested. Normal skin elements did not show deletion. The combination of MD and PCR are powerful tools that can be used for the comparison of genetic abnormalities in primary and metastatic neoplasms with unusual morphologic features to help support a diagnosis with a noncontributory IP.

Adrenal Gland Neoplasms↗

PubMed1997

Cyclin D1 expression in mantle cell lymphoma is accompanied by downregulation of cyclin D3 and is not related to the proliferative activity.

The cell cycle regulatory protein cyclin D1 is essential for G1-S phase transition in several epithelial and mesenchymal tissues but is apparently not essential in normal mature B cells. An overexpression of cyclin D1 is induced by the chromosomal translocation t(11;14)(q13;q32), which characterizes non-Hodgkin's lymphomas (NHLs) of mantle cell type. We studied 26 cases of mantle cell lymphoma (MCL) for the expression of cyclins D1 and D3. A total of 23 lymphomas showed a nuclear staining for cyclin D1, whereas reactive B cells of residual germinal centers were constantly negative. When compared with cyclin D3, an inverse staining pattern emerged. Whereas the B cells of residual germinal centers reacted strongly positive for cyclin D3, there was low or missing expression of cyclin D3 in MCL cells. In other B-cell lymphomas (n = 55), including chronic lymphocytic leukemia, low-grade lymphomas of mucosa-associated lymphatic tissue, follicular lymphomas, and diffuse large B-cell lymphomas, no cyclin D1 expression could be detected and 89% of these cases displayed cyclin D3 positivity. Lymphoma cell lines harboring the t(11;14) showed cyclin D1 protein but no or very low levels of cyclin D3; three other B-cell lines, a T-cell line, and peripheral blood lymphocytes strongly expressed cyclin D3 and reacted negatively for cyclin D1. We conclude that the chromosomal translocation t(11;14) leads to an abnormal protein expression of cyclin D1 in the tumor cells of MCL and induces a consecutive downregulation of cyclin D3. In contrast to other B-NHLs, cyclin D1 and D3 expression in MCL is not related to the growth fraction.

Cell Division↗

PubMed1975

Radionuclide photoscanning. Usefulness in preoperative evaluation of melanoma patients.

The results of radionuclide scans (liver, bone, and brain scans and whole-body scans using bleomycin tagged with radioactive indium [111In]) performed on 100 melanoma patients during their initial evaluation were reviewed to determined whether or not they effected a change in clinical staging or therapeutic decision. Patients were classified as stage I (primary disease) or stage II (regional disease) on the basis of regional lymph node dissections. Only one patient (stage II) of 73 stage I and II patients had an abnormal finding on the initial radionuclide scan. Fifteen of 26 patients thought to be stage III (disseminated disease) on clinical grounds had abnormal findings on at least one scan. A therapeutic change as a result of scanning occurred in the stage II patient after abnormal bone and whole-body scans. As an initial routine screening test for stage I and II melanoma patients, radionuclide scanning was unproductive and rarely influenced therapeutic decisions.

Adult↗

PubMed1975

The platelet in leukemic reticuloendotheliosis. Functional and morphological evidence of a qualitative disorder.

Platelets were studied in a group of 10 patients with typical clinical course, morphological findings, and specific histochemical criteria for leukemic reticuloendotheliosis. In 8 of these, marked qualitative abnormalities were found. These included lack of aggregation following epinephrine stimulation (6 patients), and decreased platelet factor 3 availability following ADP stimulation (4 patients). In addition, platelets in 4 of the 10 patients were studied by electron microscopy. All had granular abnormality, and 1 case showed the presence of rough-surfaced endoplasmic reticulum. The functional and ultrastructural abnormalities of platelets reported here may be responsible for the clinically important bleeding episodes which were not attributable to thrombocytopenia in 2 of our patients. The findings also provide a clue to the basic nature of this histogenetically controversial malignancy.

Adenosine Diphosphate↗

PubMed1984

Neck web and congenital heart defects: a pathogenic association in 45 X-O Turner syndrome?

This study explores the association between neck web and congenital heart disease in patients with Turner syndrome. Of 193 cases with documented 45 X-O karyotype, 106 (55%) had a web neck and 87 (45%) had a normal neck. The incidence of congenital heart disease was significantly different between these two groups, occurring in 30% of those with web neck and 9% of those with normal neck (x2 = 12.82, P less than .0005). The difference was most striking in coarctation of the aorta for which the prevalence was 25% with web neck and 3% with normal neck (X2 = 17.65, P less than .0001). The association between web neck and congenital heart disease suggests a pathogenic relationship exists between the two. The following hypothesis is proposed to explain the association. Increased lymphatic pressure associated with jugular lymphatic sac obstruction distends the thoracic ducts, which compress the ascending aorta altering intracardiac blood flow. Redirection of intracardiac blood flow produces coarctation of the aorta and other defects in the spectrum of left heart obstruction. This proposed mechanism is an example of a teratogenic event remote from the heart, which alters cardiovascular morphology.

Abnormalities, Multiple↗

PubMed2003

Sinus histiocytosis with massive lymphadenopathy: complicated by autoimmune hemolytic anemia.

Sinus histiocytosis with massive lymphadenopathy is a rare histiocytic disorder with very few case reports in Indian literature. Immunological abnormalities have been documented in few cases. We report one such case of a child presenting with generalized lymphadenopathy and complicated by autoimmune hemolytic anemia, suggestive of an associated immune dysfunction.

Anemia, Hemolytic↗