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PubMed1977

Polymyalgia rheumatica and chronic lymphatic leukaemia.

A patient with polymyalgia rheumatica (PMR) is reported in whom, at the time of diagnosis, the number of lymphocytes in peripheral blood and bone marrow was normal. Lymphocytic infiltration typical of PMR was detected in renal and muscle biopsy specimens, and a liver fine-needle aspirate contained an abnormal lymphocytic infiltration, probably leukaemic. The number of lymphocytes gradually increased in bone marrow and peripheral blood until, almost 5 years after PMR had been diagnosed, a typical picture of chronic lymphatic leukaemia (CLL) was observable. The significance of the reported combination is discussed. The possibility is pointed out that a CLL in progress must be suspected when persistent lymphocytosis occurs in a patient with typical PMR, even if initially the response to treatment with corticosteroids suggests that PMR is the sole disorder.

Aged↗
PubMed2004

Adjacent parenchymal abnormalities in peripheral bronchogenic carcinoma: correlation of thin-section CT with histology.

Our purpose is to correlate thin section CT of peripheral bronchogenic carcinomas with histologically detected lymphatic or vascular invasion. Retrospective 3-year database search revealed 186 surgical resections for primary bronchogenic carcinoma, of which 58 had available preoperative imaging performed at our institution. Cases with prior surgery, nonconfirmatory pathology, remote imaging, or central location were excluded, resulting in a study population of 42 patients, 25 men, 17 women, with a mean age of 69 years. Imaging with 1-3 mm collimation was performed within a mean of 32 days prior to surgery. Histologic diagnoses included adenocarcinoma (n = 24, 57%), squamous cell carcinoma (n = 13, 31%), large cell carcinoma (n = 4, 10%), and small cell carcinoma (n = 1, 2%), with a mean tumor size of 27 mm. Three radiologists blindly and independently recorded bronchovascular thickening, septal and nonseptal opacities, and the extent of each beyond tumor margins: 1) <5 mm, 2) 5-10 mm, and 3) >10 mm. Lymphangio-invasion was correlated with imaging findings, tumor size, and histology. Adjacent parenchymal abnormalities were recorded in 40 (95%) of 42 masses, with isolated nonseptal opacities representing the most frequent abnormality in 21 (50%), followed by bronchovascular thickening in 16 (38%), and septal opacities in 12 (29%). Lymphangio-invasion was present in 16 (38%) of cases. The frequency of lymphangio-invasion was highest (53%) in cases with 2 or more positive findings, and extension beyond 10mm from the tumor margin. This trend did not achieve statistical significance by ROC analysis. Lymphangio-invasion was positively correlated with tumor size, P =.03, but not histology.In conclusion, parenchymal abnormalities beyond tumor margins shown by CT may be due to lymphangio-invasion but imaging findings did not reliably distinguish cases with and without lymphangio-invasion.

Adult↗
PubMed2001

[Fluorescent in situ hybridization (FISH) in the diagnosis of acute childhood lymphatic leukemia (ALL)].

Classical cytogenetic analysis plays an important role in the diagnosis, classification, therapy monitoring and prognosis of patients with leukemia. Many recurrent cytogenetic abnormalities with major prognostic values have been described in childhood ALL. Hyperdiploidy and/or t(12;21) are associated with good prognosis, whereas t(9;22) and/or rearrangements of MLL gene correlate with poor outcome and therefore early detection of these abnormalities is very important. FISH can overcome some limitations of conventional cytogenetic and molecular-genetic analyses and due to high sensitivity specific chromosomal aberrations in mitoses and/or interphase nuclei can be detected. In the Center of Oncocytogenetics of the 3rd Medical Department for assessment of hyperdiploidy and structural rearrangements we use double-color FISH with centromeric and/or locus-specific probes and complex aberrations are ascertained by whole chromosome painting probes and multicolor FISH. Among 275 children with ALL examined during the last 8 years by different FISH methods we found seven patients with translocation t(9;22) and 14 patients with MLL rearrangements in bone marrow cells. Since 1988 we focus on detection of hyperdiploidy and/or t(12;21). High hyperdiploidy was found in 35 children, 10 of them had further complex rearrangements. Translocation t(12;21) was proved in 37 patients and complex rearrangements were found in 22 of them. FISH, cytogenetic and molecular-genetic analyses become obligatory for the first diagnostic examination as well as for monitoring of treatment effect in children with ALL.

Child↗
PubMed1981

Recurrent unilateral lung disease.

A 25-year-old patient, with multiple pulmonary metastases from osteogenic sarcoma who two years ago developed respiratory failure with unilateral left lung involvement and was successfully treated with independent lung ventilation, was readmitted to an ICU with recurrent unilateral lung disease. Conventional therapy failed to reverse the respiratory failure; independent ventilation temporarily improved the patient's oxygenation. At autopsy, different involvement of the two lungs by the tumor was evident so that blood flow of the right lung and lymphatic flow of the left lung were impaired. When respiratory failure unresponsive to conventional therapy develops, anatomical abnormalities should be considered.

Adult↗
PubMed1985

Internal mammary lymphoscintigraphy in the conservative management of breast carcinoma: an update and recommendations for a new TNM staging.

The results of internal mammary lymphoscintigraphy (IML) in 524 patients with breast carcinoma who underwent partial mastectomy, with or without axillary dissection, demonstrate the predictive value of an abnormal lymphoscintigram. There was a significant difference (P less than 0.0005) in actuarial survival between patients with normal and abnormal IML. In view of the increased mortality associated with metastases to both internal mammary and axillary lymphatics compared with involvement of either site alone, and the most favourable outcome in patients with involvement of neither site, it is proposed that the current TNM staging for breast carcinoma should henceforth include the status of internal mammary lymph nodes determined by radiocolloid lymphoscintigraphy. Further refinement of the N status into NA (axilla) and N1 (internal mammary nodes) is suggested. Stages I, II, and III should designate, respectively, patients with no evidence of involvement of either lymphatic site, patients with involvement of one or the other site and patients with involvement of both sites. This would constitute a more rational approach to the staging and management of the patient with breast carcinoma and would also provide a more accurate means of evaluating the true efficacy of current strategies in comparable groups of patients. Unrecognised internal mammary lymphatic metastases have probably confounded the results of past prospective trials.

Actuarial Analysis↗
PubMed1979

Normal growth despite abnormalities of growth hormone secretion in children treated for acute leukemia.

We have studied the relationship between abnormalities of the growth hormone-somatomedin axis and growth in 26 children previously treated for acute lymphatic leukemia. Each child had previously received cranial irradiation, was in complete clinical and hematologic remission, and off all drugs. The mean standing height SDS of the 26 children was significantly less than normal. There was no significant difference between the mean standing height SDS, height velocity SDS, somatomedin activities, and degree of bone age retardation between the 17 children who received the higher dose of cranial irradiation (Group 1) and the nine who had the lower dose of cranial irradiation (Group II). Furthermore, there was no significant reduction in mean height velocity SDS, somatomedin activity, or bone age in either group when compared to normal age-matched controls. The peak GH responses to both insulin hypoglycemia and an arginine test were significantly lowered in Groups I and II when compared to a control group of children. We conclude that only a minority of children, who previously received cranial irradiation for ALL were clinically GH deficient and, therefore, likely to benefit from GH therapy despite the finding that the majority of these children had reduced GH responses to pharmacologic stimuli.

Age Determination by Skeleton↗
PubMed2006

Cervicofacial vascular anomalies. II. Vascular malformations.

Vascular malformations are the second major category of vascular anomalies. In contrast to vascular tumors, they are present at birth and grow commensurately with the child. Although the molecular mechanisms underlying the formation of these lesions remain unclear, lesions are known to result from abnormal development and morphogenesis. Histologic examination of vascular malformations shows no evidence of cellular proliferation, but rather progressive dilation of abnormal channels. Vascular malformations are designated according to their predominant channel type; they may be capillary, venous, lymphatic, arterial, and combined malformations. Malformations with an arterial component are rheologically fast-flow, whereas capillary, lymphatic, and venous malformations are slow-flow in nature. The morbidity of vascular malformations varies greatly both within and among the clinical subgroups cited above. This article describes the clinical presentation, diagnosis, and management of vascular malformations. The more frequently encountered clinical presentations involving the head and neck are highlighted.

Arteriovenous Malformations↗
PubMed2006

Vascular endothelial growth factor-D is increased in serum of patients with lymphangioleiomyomatosis.

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by an abnormal proliferation of smooth muscle-like cells (LAM cells) in the lung and along the axial lymphatics. LAM demonstrates a heterogeneous clinical course, but there is no serum surrogate marker available for assessing the disease severity or predicting the disease progression. Since the authors have recently demonstrated the extensive LAM-associated lymphangiogenesis and its potential role in progression and metastasis of LAM cells, they hypothesized that serum levels of lymphangiogenic growth factors might be increased in LAM and become a surrogate marker for disease severity. METHODS AND RESULTS: VEGF-A, VEGF-C, and VEGF-D in serum of 44 patients with LAM were measured by enzyme-linked immunosorbant assay. Only VEGF-D was significantly increased in LAM patients as compared with age- and gender-matched healthy volunteers (n=24) (LAM vs. control, geometric mean 95% CI; 1069.3 pg/mL (809.4 approximately 1412.6) vs. 295.9 pg/mL (262.6 approximately 333.5), p<0.0001). Serum VEGF-D levels negatively correlated with variables of pulmonary function tests, FEV1/FVC (forced expiratory volume in one second/forced vital capacity) (r=-0.365, p<0.05) and %DLco/VA (the percentage of diffusing capacity for carbon monoxide/alveolar volume to the predicted value) (r=-0.560, p<0.001). As expected, the group who received hormone therapy showed more deteriorated pulmonary function with higher serum VEGF-D levels than the group who was just observed without hormone therapy. Immunohistochemical examination of lung specimens demonstrated the positive immunoreactivity of LAM cells for VEGF-D. CONCLUSION: Serum VEGF-D levels may be a valuable surrogate marker for evaluating the disease severity in LAM.

Adult↗
PubMed2004

Dehydrated hereditary stomatocytosis is associated with neonatal hepatitis.

Dehydrated hereditary stomatocytosis (DHSt) is an inherited haemolytic anaemia associated with increased red cell membrane permeability to Na(+) and K(+). It is increasingly recognized that a syndrome of self-limiting perinatal ascites can accompany the haemolysis. The cause of the perinatal ascites is unknown, and it has been argued that this could be due to cardiovascular, hepatic or lymphatic problems. We describe the case of a 16-year-old girl who presented neonatally with abnormal liver function tests and ascites. She was extensively investigated at that time. A liver biopsy showed hepatitis and fatty changes. Her ascites resolved within 6 months. At the age of 15 years, she developed an episode of acute haemolysis and was re-investigated. A diagnosis of DHSt was made. Pseudohyperkalaemia, due to ex vivo loss of K(+) from red cells, was present. This study confirms the previously noted association of DHSt, pseudohyperkalaemia and perinatal ascites, and suggests that the latter is of predominantly hepatic origin.

Anemia, Hemolytic, Congenital↗
PubMed1979

Widespread eczema vaccinatum acquired by contacts. A report of an autopsy case.

A 4-month-old male infant predisposed to allergic dermatitis acquired wide-spread eczema vaccinatum by contacts with a recently vaccinated sibling. He died of acute purulent peritonitis following a perforation of multiple duodenal ulcers. Fluorescence immunocytochemical and electron microscopic studies on the skin lesions revealed the presence of viral antigens and numerous virus particles compatible morphologically with those of the mature form from the same batch of smallpox vaccine given to the sibling. A large number of virus particles in the developmental form were also predominantly scattered in the cytoplasm of cells at the stratum malpighii of the epidermis as well as in neutrophils and macrophages in the skin lesions. The virus isolation from the skin lesions was done by using the HeLa cells and the human embryonic lung fibroblasts. No abnormal laboratory data were noted in immunoglobulins. On the basis of atrophy of the thymus and other lymphatic tissues and an appearance of large pyroninophilic cells in association with blastoid transformation, the authors discussed a possible participation of the disturbance of cellular immunity secondary to the virus infection in the development of the disease.

Antigens, Viral↗
PubMed1990

[A clinical evaluation of lymphography in bladder tumor].

A study was conducted in a total of 51 patients with urinary bladder tumor who had received surgical operation associated with removal of regional lymph nodes after lymphography. Abnormal findings were observed in lymphogram of 22 patients. The other 29 patients showed no such abnormality. After the operation pathological metastasis was confirmed in 7 patients. Lymphograms of these patients ith positive lymphatic metastasis revealed filling defects in 85.7% and collaterals in 57.4%. There as correlation between the occurrences of these two findings based on quantification analysis III of multivariate analysis. Lymphography before operation showed 87.5% of sensitivity, 65.1% of specificity and accuracy of 68.6%, not having higher accuracy compared to other studies. These results suggest that lymphography, which may have clinical significance, is not a decisive method in exploration of bladder tumor. Comprehensive analysis of diagnostic imaging should be performed, including CT after lymphography.

Female↗
PubMed1978

[Sézary's syndrome (author's transl)].

The syndrome described in 1938 by Sézary and Bouvrain is characterized by a possibly hyperpigmented erythroderma with oedema and infiltration of the skin, palmo-plantar keratoderma, diffuse alopecia and large lymphadenopathies. The cutaneous histopathology frequently shows a dermal mononuclear infiltrate with, sometimes, epidermal microabcesses. But none of these signs is actually specific for the Sézary syndrom, the only criteria of which is the presence of circulating Sézary cells with their folded, cerebriform nucleus demonstrated by electron microscopy. The Sezary cell is to date clearly identified as a T lymphocyte but membrane markers and Tcell fonction studies could elicite abnormal and poor reactive T cell. In order to assert the Sézary Syndrome it is stated by the authors that the erythroderma must be associated with more than 10% of Sézary cells in peripheral blood. This feature is needed to differentiate the Sézary syndrome from the erythrodermic form of mycosis fondoïdes in which the abnormal T cell proliferation is mainly located in the skin. The relationship with the T cell chronic lymphatic leukemia and the main treatments of the Sezary syndrome are discussed.

Dermatitis, Exfoliative↗
PubMed2000

Cell biology of lymphatic metastasis. The potential role of c-erbB oncogene signalling.

Lymphatic metastases are an important indicator of the malignancy of epithelial cancers. Empirical clinical observations associating specific genetic abnormalities with tumour progression, allied with basic laboratory investigations, are providing not only improved prognostic and diagnostic opportunities, but also a detailed understanding of the molecular machinery of metastasis. One such association--between the c-erbB oncogene family and metastasis--has proved particularly instructive. Functional links between over-expression (and occasionally mutational activation) of c-erbB-1 (EGFR) and c-erbB-2 and specific phenotypes of metastatic cells have been elucidated. Activated c-erbB oncogenes potentiate tumour cell adhesion to endothelial cells and upregulate VEGF, potentially facilitating angiogenesis and vascular invasion. In addition, cells over-expressing these oncogenes frequently show aberrant cell-cell and cell-matrix interactions, mediated by changes in integrin and cadherin function. Thirdly, both EGFR and c-erbB-2 signalling can significantly upregulate specific matrix metalloproteinases, key enzymes involved in angiogenesis and invasion. Finally, c-erbB receptors linked to the actin cytoskeleton and highly expressed on invadopodia, are thought to assist cell migration. Taken together, these observations suggest that such receptors can act as "master switches" in metastasis, whose activation co-ordinately controls events normally utilised in development, now subverted by the metastatic cell. As such, they represent ideal targets for therapeutic intervention.

Cell Adhesion↗
PubMed1997

Hypoplastic thymus and T-cell reduction in EECUT syndrome.

We report on a patient with EEC/EECUT syndrome and concomitant hypoplasia of the thymus and reduction of T cells in secondary lymphatic organs. The patient was born prematurely at 35 weeks of gestational age and exhibited ectodermal dysplasia, ectrodactyly, cleft palate and urinary tract abnormalities. On the left side, a large ureterocele was present. On the right side, an atretic ureter was found. Both conditions had led to intrauterine hydronephrosis, renal dysplasia, oligohydramnios, pulmonary hypoplasia, and death of the child. Ureteral malformations are thought to be of epithelial origin. Autopsy showed only small rudiments of thymic tissue containing single epithelial cells, but were completely devoid of Hassall corpuscules. Again, this clearly points to an ectodermal defect. Although there was severe reduction of T cells in secondary lymphatic organs, the thymic defect would not have necessarily led to immunological deficiency; perhaps this is the reason that an epithelial defect in the thymus of patients with EEC syndrome has not yet been reported. With regard to an updating of the diagnosis of the EEC/EECUT syndrome, an "EEC/EECUT plus" syndrome is suggested.

Abnormalities, Multiple↗
PubMed1995

Cervicofacial lymphatic malformation: clinical course, surgical intervention, and pathogenesis of skeletal hypertrophy.

This is a retrospective review of the clinical course and long-term soft-tissue/skeletal problems in 17 patients with large cervicofacial lymphatic malformations. Morbidity included infection (71 percent), airway compromise requiring tracheostomy (65 percent), poor dental health with aggressive caries (53 percent), abnormal articulatory patterns (47 percent), and episodic bleeding (35 percent). All patients underwent soft-tissue excision (mean four procedures per patient). Damage to facial nerve (76 percent) and hypoglossal nerve (24 percent) were common postoperative sequelae. Contour resection did not alter the progression of skeletal hypertrophy. Overgrowth most commonly occurred in the mandibular body, manifesting as anterior open bite deformity and class III occlusion (65 percent). Early mandibular body ostectomy was done in four children with grotesque hypertrophy. Jaw osteotomy was required in 71 percent of the patients to improve the maxillary/mandibular relationship. Histologic examination revealed intraosseous lymphatic malformation in areas of skeletal overgrowth in two-thirds of surgical specimens. The complexity of managing cervicofacial lymphatic malformation underscores the need for an interdisciplinary program in every major referral center.

Adolescent↗
PubMed1989

[Sonographic detection of hygroma colli in the fetus].

Foetal cystic hygroma is a congenital malformation of the lymphatic systems. Ten cases of foetal cystic hygroma were diagnosed prenatally during the second trimester, in a period of two years, at Ultrasound Unit of the Department of Obstetrics and Gynaecology of the University of Mainz, West Germany. Six foetuses had Turner's syndrome, one had a 47 X + 21 karyotype, two foetuses were cytogenetically normal and in one case tue chromosome cultures failed. Sonographic findings are demonstrated and compared to autopsy results. In addition, clinical management of foetuses with cystic hygroma are discussed.

Abnormalities, Multiple↗