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Lymphangioma involving the mandible: immunohistochemical expressions for the lymphatic proliferation.

We report a case of lymphangioma involving oral mucosa and mandible of an elderly female. The surgical and radiological examinations indicated that the lymphangioma was mainly distributed in the labial mucosa tissue, but had gradually extended into the periosteum and intrabony space of mandible. Immunohistochemical staining was also performed using antiseras of alpha-smooth muscle actin (alpha-SMA), von Willebrand factor (vWF), angiogenin, vascular endothelial growth factor (VEGF), and proliferating cell nuclear antigen (PCNA) to elucidate the pathogenetic implications of the intraosseous lymphangioma. The present case of lymphangioma showed strong immunohistochemical reactivity of angiogenin and vWF, while it showed weak reactions of VEGF and PCNA. The immunostaining of alpha-SMA disclosed an abnormally thinned and discontinuous smooth muscle layer in the lymphatics. Both the X-rays and histological examination showed that the lymphangioma lesion was gradually extending into the adjacent osteoporotic marrow space of mandible. Therefore, we believe that the present case of intraosseous lymphangioma, which showed the harmatomatous growth of the lymphatics into the marrow space of mandible, is closely related to osteoporotic changes of old age.

Actins↗

[Cancer of the prostate: role of surgery in the evaluation of lymph node extension].

None of the non surgical methods for investigations of the lymph node extension of prostate carcinoma is entirely satisfactory. However, the risk of lymphatic extension is correctly appreciated when the initial tumor is well documented by rectal examination and endo-rectal ultrasonography. Histology shows an increasing interest. IVP, CT scan are of little interest for micrometastases while the results of lymphography are improved by cytoaspiration of abnormal models. Lymphadenectomy gives the most accurate appreciation of the lymphatic extension. The reliability of frozen sections interpretation is now very satisfactory. When limited to the triangle: external iliac artery, internal iliac artery and obturator fossa, the morbidity is reduced. In case of negative lymph nodes, radical prostatectomy gives to the patients a reasonable chance of complete cure.

Biopsy, Needle↗

The effect of tonsillectomy on impedance audiometry.

In an experimental group of 22 patients undergoing elective tonsillectomies and having normal preoperative middle ear function, there is a significant development of abnormal tympanogram types, loss of crossed acoustic reflexes, and decrease in middle ear compliance on the first postoperative day when compared to a control group. Postoperative recovery of middle ear function begins at one week and is usually completed by one month. The loss of acoustic reflexes occurs in those patients with extreme changes in compliance and abnormal tympanogram types. The authors postulate that these changes in middle ear function in the immediate posttonsillectomy period might be due to the surgical disturbance of the peritubal lymphatic drainage, causing engorgement of the tympanic and tubular mucosa; and to abnormal swallowing with impaired coordination of the nasopharyngeal and tubal musculature. These data are helpful in investigating the tonsillar effect on middle ear function and disease.

Acoustic Impedance Tests↗

Radiographic demonstration of intercostal lymphatics and lymph nodes.

The anatomy of the posterior intercostal lymphatics and lymph nodes is reviewed. These lymph nodes are occasionally visualized by bipedal conventional lymphography. Opacified and/or enlarged posterior intercostal lymph nodes may also be identified with computed tomography of the chest. Familiarity with the lymphatic drainage patterns of the intercostal spaces and recognition of abnormal intercostal lymph nodes may provide additional information regarding disease status in patients with inflammatory or malignant disease of the thorax.

Female↗

Lymphatic regeneration following microvascular limb replantation: a qualitative and quantitative animal study.

Lymphatic regeneration following unilateral hindlimb autotransplantation was studied in 14 Lewis rats using Technetium-99 radiolabeled sulfur colloid (TC-99) lymphoscintigraphy and compared to the lymphatic pattern exhibited in four unoperated control rats. Control animals demonstrate a reproducible flow of lymph along the deep lymphatic system from the foot, draining into the ipsilateral inguinal nodes, and then up to the para-aortic nodes. Following replantation, lymphatic outflow from the replanted limb begins to occur within 3 to 6 days, reaching normal lymphatic clearance within 12 days. However, the pattern of lymphatic flow is ill-defined, relying on multiple small channels of the superficial lymphatic system. In contrast to controls, at 3 hr post TC-99 injection, lymphatic drainage in replanted rats is via the inguinal and axillary nodal regions bilaterally. This abnormal pattern persisted in the replanted animals for the duration of this study, 160 days. It is evident that lymphatic regeneration in this animal model is associated with a rapid return to normal levels of lymphatic clearance via collateral channels within the subcutaneous tissue. The rapidity with which lymphatic drainage is restored, and its localization within the subcutaneous tissue, can explain why replanted tissues and microvascular free flaps seldom develop lymphedema. In addition, the rat hindlimb replantation model may prove useful for studies of the general mechanisms and specific factors responsible for lymphangiogenesis.

Animals↗

Characteristics of lymphatic endothelial cells in physiological and pathological conditions.

Impairment of lymphatic structure and function, e.g., inadequate endothelial permeability and intercellular openings, abnormal lymphangiogenesis and overexpression for immunoreactive agents, will result in tumor metastasis, autoimmune response alteration and accumulation of interstitial fluid and proteins. Recently, several novel molecules have been identified that allow a more precise distinction between lymphatic and blood vascular endothelium. The differences in expression of endothelial markers on the lymphatic vessel strongly suggest the possibility that there will be important divergence in the differentiating and regenerating responses in lymphatic behavior to various pathological processes. Undoubtfully, molecular techniques would also lead to the definition of unique markers found on lymphatic endothelial cells (LECs) in lymphatic-associated diseases which are mostly involved in lymphangiogenesis. This review is mainly concentrated on the characteristics of LECs in diabetes, wound healing, lymphedema and tumor, especially in the experimental models that have offered insight into the LEC role in these diseases affecting the lymphatic system. Increased knowledge of the molecular signaling pathways driving lymphatic development and lymphangiogenesis should boost the impact of therapeutics on the diseases. Although the field about the mechanisms that control the formation and lineage-specific differentiation and function of lymphatic vessels has experienced rapid progress in the past few years, an understanding of the basis of the differences and their implications in the pathological conditions will require much more investigation.

Animals↗

Lymphadenopathy, elevated serum IgE levels, autoimmunity, and mast cell accumulation in flaky skin mutant mice.

The autosomal recessive mutation "flaky skin" (fsn) causes pleiotropic abnormalities in the immune and hematopoietic systems accompanied by pathologic changes in the skin. Homozygotes (fsn/fsn) showed increased size and histological alterations in the spleen and lymph nodes. Abnormalities in lymphoid architecture of the spleen in fsn/fsn mice were accompanied by marked increases in total numbers of B cells, macrophages, and immature erythroid cells. Splenic B cells displayed elevated MHC class II expression. Serum IgE levels were greater than 100 microg/ml by 10 weeks of age, representing > 7000-fold increase compared with normal littermates. This increased IgE level was associated with elevated IL-4 production by spleen cells and with increased amounts of serum IL-4. Serum IgM, IgG1, and IgG2b levels were also increased in fsn/fsn mice while IgG3 was decreased. Autoimmunity in fsn/fsn mice was evidenced by glomerulonephritis accompanied by immune complex deposition in the kidneys, increased serum blood urea nitrogen levels, and the presence of circulating anti-double-stranded DNA autoantibodies. Pathological changes in the skin of fsn/fsn mice were characterized by epidermal hyperplasia and mixed dermal inflammation. Increased numbers of mast cells were also observed in the dermis of the truncal skin as well as in the epithelial stomach. These marked immunological abnormalities suggest that the fsn locus encodes a major immunoregulatory molecule important in multiple immune and hematopoietic functions.

Abnormalities, Multiple↗

[Role of radioisotopic lymphoscintigraphy in postphlebitic syndrome of the legs].

Post-phlebitis syndrome of the lower limbs is a set of symptoms that follow a state of phlebostasis and phlebolymphostasis with consequent oedema and tissue distress. We submitted 36 patients with post-phlebitis syndrome of the lower limbs to radioisotopic lymphoscintigraphy for the purposes of evaluating the vicariant function of the lymphatic system and the possible role of the lymphatic system in the genesis of dermo-epidermal abnormalities. The average age of the patients was 53.2 years (range: 43-69 years; M:F ratio: 0.50). The control group consisted of 6 healthy subjects. The average duration of the post-phlebitis syndrome in the patients studied was 8.5 years. We excluded from the study patients in whom a central cause was identified as being responsible for the pathogenisis of the oedema of the lower limbs. In addition, patients with obliterant arteriopathy were also excluded. Venous pathology was evaluated first clinically and then investigated by continuous-wave Doppler. As a first step, venous pressure was measured by Doppler phlebomanometry in clino- and orthostatism. All patients underwent radioisotopic lymphoscintigraphy with microcolloids using the Rijke technique. Radioactivity was monitored by means of a computerized gamma-camera. We classified the pathological findings of radioisotopic lymphoscintigraphy as follows: 1) delayed transit; 2) obstacles; 3) star-shaped superficial collateral lymphatic circulations; 4) lymphocoele or cutaneous lakes. A significant difference was detected (p < 0.05) between the pressure values in the post- phlebitis lower limbs and the pressure values in normal subjects. Combining the results of our measurements we recorded delayed transit in 5 patients (69.5%). This latter group included the cases with the severest forms of post-phlebitis syndrome. Therefore, better knowledge of the pathophysiology of the lymphatic system would appear to be useful in order to understand the origin and evolution of oedema of the lower limbs of patients with post-phlebitis syndrome. To this end, radioisotopic lymphoscintigraphy may be useful as a first-level examination in order to evaluate the capacity and efficiency of the lymphatic system.

Adult↗

Micro-magnetic resonance lymphangiography in mice using a novel dendrimer-based magnetic resonance imaging contrast agent.

Major advances in cancer biology and immunology have been gained using mouse models. However, very few methods are currently available to visualize the deep lymphatic system. A new micro-magnetic resonance lymphangiography (MRL) method in mice, which uses dendrimer-based magnetic resonance imaging contrast agents, was developed. Micro-MRL imaging clearly visualized most of the mouse lymphatic system, including both lymphatics and lymph nodes. This method could detect and distinguish among dilation of lymphatic vessels in a lymphangitis model, proliferative or neoplastic lymph node swellings in a lymphoproliferative model, and inflammatory lymph node swellings in an infection/inflammation model. Changes in the lymphoid system of transgenic mice overexpressing interleukin-15 could be visualized. Abnormal enlarged lymph nodes identified by micro-MRL were selectively removed and analyzed to demonstrate their cell type, receptor expression, and clonality in individual mice. We conclude that the enhanced resolution of this noninvasive micro-MRL can detect and classify lymphatic and lymph node abnormalities in mice, which should have wide applicability to the study of immunology and cancer in both experimental animals and clinical medicine.

Animals↗

Lymphatic and venous function in lipoedema.

Lipoedema is a common but infrequently recognized condition causing bilateral enlargement of the legs in women. Although generally considered to be the result of an abnormal deposition of subcutaneous fat with associated oedema, the precise mechanisms responsible for oedema formation have yet to be fully established. In order to evaluate the possible role of lymphatic or venous dysfunction in the pathogenesis of lipoedema, 10 patients were investigated by photoplethysmography (venous function) and quantitative lymphoscintigraphy (lymphatic function). The results were compared with those from patients with primary lymphoedema and those from healthy volunteers. The results demonstrated minor abnormalities of venous function in only two patients. One patient had moderately impaired lymphatic function in both legs and seven patients had a marginal degree of impairment in one or both legs. However, in none of these cases did the impairment attain the low levels seen in true lymphoedema. Lipoedema appears to be a distinct clinical entity best classified as a lipodystrophy rather than a direct consequence of any primary venous or lymphatic insufficiency.

Adipose Tissue↗

Gorham disease: correlation of MR findings with histopathologic changes.

Gorham disease is a rare disorder of unknown etiology characterized by bone destruction and abnormal proliferation of thin-walled vascular channels including lymphatic capillaries. Starting monocentrically in a single bone, the angiomatous masses in this disease extend to adjacent bones and soft tissues without respecting articular barriers. Herein we report a case of Gorham disease with its MR and histopathologic appearance.

Bone Marrow↗

Lymphedema management.

Lymphedema, defined as the abnormal accumulation of protein rich fluid dysfunction of the lymphatic system, is a common sequela of cancer therapy. The incidence is highest among patients who have undergone resection and irradiation of a lymph node bed. Recently, increased attention has been focused on the modification of anticancer therapies in an effort to minimize lymphatic compromise. Sentinel lymph node biopsy is an example of a surgical procedure developed to preserve lymphatic function. Concurrent with the development of less invasive treatments, the field of lymphedema management has evolved rapidly over the past decade. Combined manual therapy, often referred to as complex decongestive physiotherapy (CDP), has emerged as the standard of care. CDP combines compression bandaging, manual lymphatic drainage (a specialized massage technique), exercise, and skin care with extensive patient education. Case series collectively describing a mean 65% volume reduction in over 10,000 patients attest its efficacy. Pneumatic compression pumps were historically widely used to control lymphedema. Their use as an isolated treatment modality is now rare. Reliance on pumps diminished with the recognition that they may exacerbate truncal and genital lymphedema, as well as injure peripheral lymphatics when applied at high pressures. Many noncompressive approaches, particularly the use of benzopyrone medications and liposuction, continue to be used abroad.

Bandages↗

E receptor expression of T lymphocytes in T lymphoproliferative diseases.

The lymphoid component of skin infiltrates from 6 cases of mycosis fungoides and 2 cases of T cell chronic lymphatic leukaemia consisted almost exclusively of T lymphocytes with abnormally high E receptor expression. Such T cells were also found to dominate the skin infiltrates from 10 patients with severe atopic dermatitis. Blood T lymphocyte counts from all types of patients were within the normal range, but even the blood T lymphocytes of the patients had a strong E receptor expression. The bone marrow in atopic dermatitis and in mycosis fungoides showed normal T cell counts with normal E receptor expression. Supported by basic lymphocyte-kinetic considerations it is likely that the skin in T lymphoproliferative diseases is characterized by an abnormal ability to home and/or to trap T lymphocytes with strong E receptor expression.

Adult↗

Bone marrow karyotypes in 94 children with acute leukemia.

During the last 10 years, we have cytogenetically analyzed at diagnosis bone marrow cells from a total of 94 children with acute leukemia. Of the 78 children with acute lymphatic leukemia (ALL), 53 (68%) had clonal acquired chromosome abnormalities; in the group with acute nonlymphatic leukemia (ANLL), the corresponding proportion was 13 out of 16 (81%). Among the cytogenetically abnormal ALL patients, the most numerous subset was the hyperdiploid cases with stemlines containing 51 or more chromosomes (26 of 53 abnormal cases; 49%). This is a clearly higher proportion than has been reported in large series from other centers. Deletions of 6q were present in 8 cases and rearrangements of 12p in 5. Of the 7 T-cell ALLs, 3 had translocations of the distal part of 7q, i.e., of the region where the beta T-cell receptor is encoded. Only 2 of 26 (8%) patients with leukemic stemlines with more than 50 chromosomes have relapsed; the remainder are still in first remission (mean observation time 42 months). This may be contrasted with 6 of 25 (24%) relapses among the cytogenetically normal (observation time 41 months), and 8 of 27 (30%) relapses among ALL patients with aberrations but with less than 51 chromosomes (observation time 26 months). Our results support the conclusion that the finding of a markedly hyperdiploid leukemia karyotype is indicative of good prognosis in ALL.

Adolescent↗

In chyloptysis, SP-A affects the clearance of serum lipoproteins entering the airways.

Serum lipoproteins may enter the airways and appear in sputum (chyloptysis) when the lymphatic circulation is impaired by inflammation, neoplasia, or an abnormal proliferation of smooth muscle cells. While analyzing the bronchoalveolar lavage fluid of a patient with chyloptysis, we noticed that surfactant could not be separated from contaminating serum lipoproteins and speculated that lipoproteins might interact with surfactant components. To clarify this point we immobilized surfactant protein (SP) A on microtiter wells and incubated it with 125I-labeled very low density lipoproteins (VLDLs), low-density lipoproteins, and high-density lipoproteins. We found that SP-A binds lipoproteins. Studying in greater detail the interaction of SP-A with VLDLs, we found that the binding is time and concentration dependent; is inhibited by unlabeled lipoproteins, phospholipids, and antibodies to SP-A; is increased by Ca2+; and is unaffected by methyl alpha-D-mannopyranoside. Whole surfactant is a potent inhibitor of binding. Furthermore, we found that SP-A increases the degradation of VLDLs by alveolar macrophages and favors the association of VLDLs with alveolar surfactant. We conclude that SP-A influences the disposal of serum lipoproteins entering the airways and speculate that binding to alveolar surfactant might represent an important step in the interaction between exogenous substances and the lung.

Bronchoalveolar Lavage Fluid↗

[Clinicopathologic analysis of pulmonary lymphangioleiomyomatosis].

OBJECTIVE: To investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis (PLAM). METHODS: By means of HE and immunohistochemistry (SP method) studies, the clinical and pathological features of 5 PLAM cases were analyzed and the related literature reviewed. RESULTS: PLAM was a rare lung disease of unknown etiology and was restricted to females who were generally pre-menopausal. Pathological features showed abnormal smooth muscle cells (LAM cells) line the airways, lymphatics and blood vesssels leading to airflow obstruction and replacement of the lung parenchyma by cysts. LAM cells were positive for HMB45. Clinically the disease was categorized by dyspnoea, haemoptysis, recurrent pneumothoraces and chylous effusions. CONCLUSIONS: PLAM should be considered when recurrent pneumothorax, haemoptysis and dyspnoea occur in females. Pathologic examination of lung tissue biopsy is required for confirmation of PLAM diagnosis.

Actins↗

Gorham disease: an intraoperative case study.

Gorham disease is a rare chronic disorder that is characterized by the abnormal proliferation of thin-walled capillaries and small lymphatic vessels that results in the massive osteolysis of adjacent bone. Clinical manifestations are determined by the area of involvement, which may include the chest and ribs. The case presented involves a 47-year-old man with Gorham disease complicated by unilateral chylothorax who was treated with thoracic duct ligation. The anesthetic implications associated with Gorham disease are discussed, and nonsurgical primary and adjunctive treatments for chylothorax are summarized.

Anesthesia, Inhalation↗