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PubMed1980

Hypogammaglobulinaemia associated with abnormalities of both B and T lymphocytes in patients with chronic lymphatic leukaemia.

The underlying basis for hypogammaglobulinaemia in patients with chronic lymphatic leukaemia (CLL) was investigated by measurement if immunoglobulin produced in vitro in cultures of pokeweek mitogen-stimulated B and T lymphocytes. B and T cells were separated by sheep red blood cell rosette techniques and, by culture of these cells from CLL patients in various combinations with B or T cells from normal subjects, it was possible to measure independently the function of B lymphocytes and the helper or suppressor function of T lymphocytes. By these methods it was found that the B lymphocytes of six of eight patients failed to produce immunoglobulins in vitro. B lymphocytes from two patients appeared to produce immunoglobulins in vitro. T lymphocytes from five of the eight patients had low or undetectable helper T cell function and in six patients their T lymphocytes had excessive suppressor activity in comparison to T lymphocyte populations from normal subjects. Whether the primary abnormality in the CLL T cell populations was a deficiency of helper T cells or excess of suppressor T cells was uncertain from these studies. These results suggest that immunoglobulin production by B lymphocytes from most patients with CLL was abnormal but also that T cells from CLL patients may be abnormal in respect to their role in immunoglobulin production at an early stage of the disease. These findings may assist in understanding the pathogenesis of this disease and lead to new approaches in treatment.

Agammaglobulinemia↗
PubMed1988

Pulmonary pseudofibrosis and pseudomass lesions.

Abnormal size and/or number of the pulmonary and bronchial vessels and lymphatics may mimic pulmonary parenchymal lesions (pseudofibrosis). Abnormalities of the central pulmonary artery, pulmonary vein, thoracic aorta, and brachiocephalic branches may mimic intrathoracic mass lesions (pseudomass lesions). Radiologists should interpret these radiographic findings in the light of other accompanying radiographic findings and the clinical presentation.

Bronchial Arteries↗
PubMed2002

Therapeutic lymphangiogenesis with human recombinant VEGF-C.

Chronic regional impairments of the lymphatic circulation often lead to striking architectural abnormalities in the lymphedematous tissues. Lymphedema is a common, disabling disease that currently lacks a cure. Vascular endothelial growth factors C and D mediate lymphangiogenesis through the VEGFR-3 receptor on lymphatic endothelia. The purpose of this study was to investigate the therapeutic potential for lymphangiogenesis with VEGF-C. We developed a rabbit ear model to simulate human chronic postsurgical lymphatic insufficiency. Successful, sustained surgical ablation of the ear lymphatics was confirmed by water displacement volumetry. After complete healing, the experimental animals (n=8) received a single, s.c. 100 microg dose of VEGF-C in the operated ear; controls (n=8) received normal saline. Radionuclide lymphoscintigraphy was performed to quantitate lymphatic function. Immunohistochemistry (IHC) was performed 7-8 days following treatment. After VEGF-C, there was a quantifiable amelioration of lymphatic function. IHC confirmed a significant increase in lymphatic vascularity, along with reversal of the intense tissue hypercellularity of untreated lymphedema. This study confirms the capacity of a single dose of VEGF-C to induce therapeutic lymphangiogenesis in acquired lymphedema. In addition to improving lymphatic function and vascularity, VEGF-C can apparently reverse the abnormalities in tissue architecture that accompany chronic lymphatic insufficiency.

Animals↗
PubMed1999

Lymphatic vessel hypoplasia in fetuses with Turner syndrome.

Turner syndrome is associated with subcutaneous accumulation of fluid in the neck region that can be visualized sonographically from 10-14 weeks of gestation as massively increased nuchal translucency thickness. Possible mechanisms for this increased translucency include dilatation of the jugular lymphatic sacs because of developmental delay in the connection with the venous system, or a primary abnormal dilatation or proliferation of the lymphatic channels interfering with a normal flow between the lymphatic and venous systems. The aim of this study was to investigate the distribution of lymphatic vessels in nuchal skin tissue from fetuses with Turner syndrome compared with fetuses carrying trisomies 21, 18 and 13 and chromosomally normal controls. The distribution of vessels was examined by immunohistochemistry using a monoclonal antibody, PTN63, against 5' nucleotidase and an anti-laminin antibody. In normal control fetuses (n = 6) and those with trisomies 21 (n = 3), 18 (n = 2) and 13 (n = 2), PTN63-positive and laminin-positive vessels were evenly distributed throughout the dermis and subcutis. In Turner syndrome (n = 3), there was a chain of large vessels that stained with both PTN63 and laminin at the border between dermis and subcutis, but there was scarcity of vessels in the upper dermis and the subcutis. Using PTN63 alone, there were no positive vessels in the upper dermis. We conclude that in Turner syndrome lymphatic vessels in the upper dermis are hypoplastic.

Chromosomes, Human, Pair 13↗
PubMed2004

Lymphatic malformation of the sphenoid sinus in a pediatric patient.

UNLABELLED: Lymphatic malformation is a benign vascular lesion resulting from lymphatic tissue being isolated from the remainder of the lymphatic system. They are present at birth and up to 90% are diagnosed by 2 years of age. More aggressive lesions are usually diagnosed earlier, with low-grade lesions presenting later with fewer complications. These lesions are hamartomas and not true neoplasms. The term hamartoma is used to describe an abnormally large mass of histologically normal tissue in a normal location. The lymphatic malformation is composed of lymph-filled channels lined with a single layer of flat endothelial cells on a basement membrane. They present with either generalized edema and poorly defined borders (microcystic) or a localized area of multilocular cysts (macrocystic). The term lymphatic malformation has replaced many other outdated terms, such as lymphangioma, cystic hygroma, lymphangioma circumscriptum, and lymphangiomatosis. In this study, we present a case report of a pediatric lymphatic malformation of the sphenoid sinus. To our knowledge, this lesion has not been described in the pediatric population and has been described only once in an adult in the German literature. EBM RATING: C.

Child↗
PubMed1990

AIDS, Kaposi sarcoma, and the lymphatic system: update and reflections.

Based on ongoing basic and clinical investigations, further evidence is presented that the AIDS-Kaposi sarcoma (AIDS-KS) complex involves a progressive disturbance of the blood-lymph circulatory loop of fluid, macromolecules, and migrating cells. We first surveyed the spectrum of vascular abnormalities including Kaposi sarcoma (KS) found in the AIDS/ARC population, then non-invasively imaged lymphatic system abnormalities in AIDS-KS by whole body lymphangioscintigraphy, and finally examined the biologic behavior of AIDS-KS cells in long-term tissue culture. These observations are viewed in terms of the lymphatic and blood vascular route of entry and transport of free and cell-associated virus and other opportunistic pathogens as well as poorly understood host endothelial-immune system interactions.

AIDS-Related Complex↗
PubMed1983

Lymphoscintigraphy--techniques and applications in the management of breast carcinoma.

Radiocolloid lymphoscintigraphy is a versatile, simple technique which has been utilized extensively in the assessment of lymphatic function and structure in anatomic regions accessible to neither clinical examination nor radiocontrast lymphography, as well as in circumstances where radiocontrast lymphography is not feasible. The most important requirement for an effective study are: (a) Determination of the appropriate injection site for reproducibly visualizing the majority of the lymphatics under study and, (b) use of an agent with the optimum properties of a preformed small, uniform, well-dispersed particle colloid. Multiple applications of lymphoscintigraphy in the patient with breast carcinoma have demonstrated its value in distinguishing abnormalities of the internal mammary lymphatics shown to have significant prognostic implications, in providing accurate localization of the parasternal lymphatics for radiation therapy and its potential for determining the status of axillary lymphatics in lieu of surgical intervention. The following review is based on an experience of over 6500 studies in 5550 patients with breast carcinoma.

Antimony↗
PubMed2004

Microangiopathy of split-skin grafts in venous ulcers.

BACKGROUND: In patients with chronic venous insufficiency, microangiopathy of blood and lymph capillaries caused by venous hypertension plays a major role in the development of venous ulceration. Conservative treatment of venous leg ulcers often fails, and split-skin grafting is sometimes performed. OBJECTIVE: To evaluate the microcirculation and especially the regeneration and function of lymphatic vessels in skin grafts in patients with chronic venous insufficiency. METHODS: The microcirculation of 15 split-skin grafts was studied by fluorescence microlymphography and measurement of transcutaneous oxygen tension (tcpO2) in 13 patients. RESULTS: The mean age of the skin grafts was 70.9 months. In only two skin grafts were a few intact lymph meshes found. In all other cases, only fragments of lymphatic capillaries have been detected. In seven skin grafts, cutaneous backflow of dye through insufficient deeper lymph channels was observed. The maximal spread of the dye in the lymphatic network was increased in the skin grafts (17.6 +/- 22.9 mm). The mean value of tcpO2 was only 27.1 +/- 18.1 mm Hg. CONCLUSIONS: In split-skin grafts of patients with venous ulcers, severe microcirculatory changes are present and characterized by hypoxia and abnormal regeneration and function of lymphatic vessels.

Aged↗
PubMed2005

Abdominal confluence of lymph trunks: detectability and morphology on heavily T2-weighted images.

OBJECTIVE: Our aims were to evaluate the detectability, configuration, location, and dimensions of the cisterna chyli on heavily T2-weighted images obtained with a single-shot fast spin-echo technique and to determine whether the disorders that have the potential to affect the abdominal lymphatic drainage could change the cisternal dimensions. MATERIALS AND METHODS: Thin-collimated axial and coronal images that were originally obtained for MR cholangiopancreatography in 125 patients were reviewed by three observers individually for the presence of abdominal lymphatic confluence. The configuration, location, and dimensions of hyperintense ductal or saccular structures immediately anterior to the vertebral bodies below the diaphragma were recorded. The differences between the mean values of the diameters of the cisterna chyli obtained in the control group and in groups in which lymphatic drainage was expected to be abnormally high were compared using a one-way analysis of variance test. RESULTS: Abdominal confluence of the lymphatics was shown in 96% of patients. The most common configuration of the cisterna chyli was tubular (42.5%). It was located at the level of L1-2 in 33% of cases and at the midline in 70%. Mean longitudinal, anteroposterior, and transverse diameters of the duct were 33.45 +/- 1.74 (SD) mm, 5.23 +/- 0.13 mm, and 5.23 +/- 0.15 mm, respectively. No significant difference was found in the mean values of antero-posterior, transverse, and longitudinal diameters of the cisterna chyli in the control group and in the groups expected to have an increased flow into the cisterna chyli. CONCLUSION: Abdominal confluence of lymphatics seems to be present on most of the heavily T2-weighted images. Its morphologic details and extensions can be visualized on images reconstructed with a maximum-intensity-projection algorithm. Any disorder does not necessarily lead to dilatation of these lymphatic structures.

Abdomen↗
PubMed1999

Measurement of lymphatic function with technetium-99m-labelled polyclonal immunoglobulin.

A reliable method for measuring lymph flow in physiological units would be valuable, especially in conditions in which it is uncertain whether lymph flow is increased or decreased. The requirements of a radiopharmaceutical for such measurement include stable radionuclide labelling and rapid access to lymphatic vessels following tissue injection but no access to blood vessels. A soluble macromolecule is likely to come closest to meeting these requirements. Technetium-99m-labelled human polyclonal immunoglobulin (HIG) was therefore investigated firstly in comparison with 99mTc-labelled human serum albumin (HSA) in patients undergoing routine lymphoscintigraphy and secondly with respect to injection site in a group of volunteers with post-mastectomy oedema (PMO). Subcutaneous injection of 99mTc-HIG into the web space of a distal extremity gave images in which lymphatic vessels were more clearly defined compared with images obtained after injection of 99mTc-HSA. Lymph nodes were also more clearly defined, suggesting specific retention of HIG, possibly through Fc-mediated binding. Peripheral blood sampling showed a delayed arrival in blood of radioactivity after 99mTc-HIG compared with 99mTc-HSA, although ultimately, the blood recovery of 99mTc-HIG was significantly higher (P < 0.05) than that of 99mTc-HSA. Clearance rates of radioactivity from the injection site were not significantly different, however, between the two agents. In patients with PMO, web space injection of 99mTc-HIG gave excellent images of normal lymphatic vessels, of lymph nodes and of abnormal lymph drainage such as dermal backflow in swollen arms. In contrast, neither lymphatic vessels nor lymph nodes were visualised after injection into the skin of the dorsum of the distal forearm. Although there was no difference in clearance rates from the injection sites between normal and swollen arms with either agent in PMO, clearance was significantly faster following injection into the web space (0.11% per minute for normal and swollen arms combined) than into the forearm (0.053% per minute; P < 0.05). These results suggest that (a) 99mTc-HIG is a potentially useful agent for measuring lymph flow and lymph node function; but (b) injection into the dorsum of the forearm is not a useful method of administration for these measurements; and (c) clearance rates from the injection site do not support the notion that PMO is the result of decreased lymph flow. Further studies are warranted to evaluate 99mTc-HIG as an agent for assessment of lymphatic function, especially with respect to measurement of lymph flow and possibly also for the evaluation of lymph node Fc-mediated immunocompetence.

Female↗
PubMed2000

[Development of a case of Mycobacterium avium complex disease from right pleural effusion].

Moist pleurisy in patients with Mycobacterium avium Complex (MAC) is rarer than tuberculosis. We encountered an extremely rare case of MAC disease in a 75-year-old man who initially had only right pleural effusion. Gaffky VII was detected in the pleural effusion, and Mycobacterium avium was identified by culture and PCR. Although administration of antitubercular agents (RFP, INH, EB, and SM) + CAM and thoracic lavage were repeated, the Gaffky persisted strongly. Accordingly, pulmonary decortication and filling of the cavity with an omental flap were performed as surgical treatments. However, fistulas were formed between the remaining empyema cavity and the surgical wounds. Fenestration was also carried out. Postoperatively, centriacinar abnormalities appeared on computed tomography (CT). It has been reported that MAC disease begins with centriacinar abnormalities and the incidence of the lymphatic developmental pattern was low. Tuberculosis (the idiopathic pleuritis type) is considered to be caused this pattern from the primary infection focus. Therefore, the onset of unilateral effusion is extremely rare in patient with MAC disease, suggesting that the lymphatic developmental pattern occurs less frequently in patients with MAC disease. Furthermore, in this case, we speculated that centriacinar abnormalities were the MAC infection foci and could be detected by CT due to surgical invasion.

Aged↗
PubMed1991

[Lymphoscintigraphy with 99mTc-DTPA-HSA: detection of metastases to iliopelvic lymph nodes].

The purpose of this study was to evaluate 99mTc-DTPA-HSA as an iliopelvic lymphoscintigraphic agent in 5 normal volunteers and 10 patients with metastases of malignant tumors (cancer, 9; and malignant lymphoma, 1) to the iliopelvic lymph nodes. The subjects underwent intradermal injection of 185 MBq of 99mTc-DTPA-HSA into digital web spaces of the feet. Massage was applied at the injection sites for 30 sec; the subjects then walked around for 2 min. Whole-body scintigrams were obtained 5 min after injection. The whole-body scanning speed was 20 cm/min. The tracer transport was prompt. Within 15 min after injection, the tracer reached the termination of the thoracic duct in all normal volunteers. Normal whole-body images of excellent quality delineated the lymph nodes and channels almost without background radioactivity. The images of 9 patients with metastases of cancer showed clearly the following abnormal patterns: a) obstruction of lymphatic system (5/9, 55.6%); b) absence of visualization of the thoracic duct (44.4%); c) decreased uptake in lymph nodes (88.9%); d) visualization of collateral circulation (44.4%); e) tracer extravasation into more proximal soft tissue (22.2%). The image in the patient with malignant lymphoma showed increased uptake in the enlarged lymph nodes in addition to the all abnormal findings mentioned above. We concluded that 99mTc-DTPA-HSA is an excellent radiopharmaceutical for iliopelvic lymphoscintigraphy.

Adult↗
PubMed1977

A quantitative measurement of peritoneal drainage in malignant ascites.

With a two-compartment model, a method is described for quantitative determination of peritoneal drainage rates in malignant ascites. Data on twenty-four patients are presented and comparison is made with the qualitative assessment of the integrity of diaphragmatic and mediastinal lymphatics on the basis of lymphoscintigrams. It is concluded that flow rates less than 50 ml/hour are usually associated with abnormalities of the diaphragmatic and mediastinal lymphatics, indicating that tumor permeation of these structures is a significant contributing factor to persistent, intractable ascites in patients with malignant diseases.

Ascites↗
PubMed1993

Noninvasive evaluation of the lymphatic system with lymphoscintigraphy: a prospective, semiquantitative analysis in 386 extremities.

PURPOSE: Lymphoscintigraphy has emerged as the diagnostic test of choice in patients with suspected lymphedema. To assess the lymphatic circulation of 386 extremities in 188 patients, we prospectively recorded a semiquantitative index of lymphatic transport in addition to visual evaluation of lymphoscintigraphy image patterns. METHODS: Sixty-one male and 127 female patients were studied (mean age 48 years, range 13 to 87 years). Twenty had upper extremity swelling, and 168 had lower extremity swelling. The disease was bilateral in 60 patients. Lymphoscintigraphy was performed by injecting a mean of 503 microCi of technetium 99m-antimony trisulfide colloid subcutaneously into the second interdigital space of the extremity. Time for transport to regional lymph nodes, appearance of lymph vessels and nodes and distribution pattern were scored. These scores were compiled into a modified Kleinhans transport index (TI). To assess the venous circulation, 155 patients underwent evaluation of the venous system by impedance plethysmography, ultrasonography, or contrast venography. RESULTS: The mean TI (+/- SEM) in 79 asymptomatic extremities was 2.6 +/- 0.5, with 66 (83.5%) demonstrating normal lymphoscintigraphy pattern (TI < 5). Patients with clinical diagnosis of lymphedema (n = 124) had a mean TI of 23.8 +/- 1.5; 81.5% of these were greater than 5. Fifty-six patients (30%) had primary and 68 (36%) had secondary lymphedema. (TI of 26 +/- 3.5 and 22.1 +/- 1.9, respectively, p = NS). Patients without any lymphatic transport (TI of 45) were more likely to have cellulitis in their history (p < 0.05). Contrast lymphangiography in six patients correlated with lymphoscintigraphy. Sixty-four patients (34%) had swelling without lymphedema (venous edema, cardiac edema, lipedema, etc.; TI of 1.9 +/- 0.4, p < 0.001). Of the 41 patients with abnormal venous studies, 18 (44%) had an elevated TI. CONCLUSIONS: Semiquantitative evaluation of the lymphatic transport with lymphoscintigraphy reliably depicts abnormalities in the lymphatic circulation. Lymphoscintigraphy excluded lymphedema as a cause of leg swelling in one third of our patients.

Adolescent↗
PubMed1977

A lymphographic study of chyluria.

Ninety patients with chyluria were studied by bilateral pedal lymphography. The retroperitoneal lymphatic vessels were dilated, tortuous and increased in number in 61% of cases while the appearance of the lymph nodes was abnormal in 67% of cases. Renal lymphatic reflux, either bilateral or unilateral, was seen in 93% of patients. A correlation (P greater than 0.007) was observed between the site of origin of the cisterna chyli and side of unilateral renal lymphatic reflux. There was no evidence of thoracic duct obstruction although the thoracic duct was more than 3 mm in calibre in seven cases. Lymphography was also performed on 22 patients who had undergone stripping of the lymphatics along the renal pedicle to determine if surgical disconnection was complete. The pathogenesis of chyluria is briefly discussed.

Adult↗
PubMed1984

Membrane markers, karyotypic abnormalities, ultrastructure and functional properties of lymphocytes in a case of 'D-cell' chronic lymphatic leukemia.

D cells are lymphocytes bearing both receptors for the third complement component and the ability to form spontaneous rosettes with SRBC. We report the case of a patient with a D-cell chronic lymphatic leukemia who presented a long evolution without treatment and whose leukemic cell characteristics have been extensively studied. Cytogenetic analysis showed numerous karyotypic abnormalities among leukemic cells; all metaphases were hypodiploid and arranged in four different clones; seven marker chromosomes were present. The cells were found to bear human T-cell specific antigen, the T helper/inducer phenotype, HLA-A and HLA-B determinants, but no HLA-DR antigens. They displayed a high proliferative response to PHA and Con A, no response to PWM stimulation, and possibly the capacity of allogeneic stimulation in the mixed lymphocyte culture system. Assays for cell-mediated cytotoxicity in the CML system, and for K and NK activities were negative.

Antigens, Surface↗
PubMed1984

Lymphatic hypoplasia without lymphoedema.

A prospective study of lymph vessel abnormalities in the lower limbs of 744 adult patients without lymphoedema revealed seven patients with lymphatic hypoplasia, a prevalence rate of 0.94% (95% limits of confidence 0.24-1.64%). The prevalence was higher in males (1.1%) than in females (0.5%) but the difference is not statistically significant. This is much higher than any available estimate of the prevalence of primary lymphoedema of the lower limbs and suggests that lymphatic hypoplasia is merely one factor in the aetiology of clinical lymphoedema.

Adolescent↗