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[Biermer's anemia with chronic lymphoid leukemia and hemolysis with autoantibodies].

The authors report a case of chronic lymphatic leukemia occurring four years after the onset of pernicious anemia. Multiple immunological abnormalities were detected. Hemolytic anemia with auto-antibodies and anti-thyroglobulin antibodies. Anti-intrinsic factor antibodies of type 1 were found but disappeared later. This case, the 9 th in the world literature, emphasizes the links between pernicious anemia, an auto-immune disease, and malignant disorders of the lymphatic and immune systems.

Aged↗

Achondrogenesis type II (Langer-Saldino) in association with jugular lymphatic obstruction sequence.

The prenatal diagnosis of achondrogenesis in association with cystic hygroma is described. Ultrasound findings of severe short-limbed dwarfism, decreased vertebral ossification, and normal ossification of the calvarium were all consistent with achondrogenesis type II. Although the unusual finding of associated cystic hygroma raised the suspicion of a concurrent chromosome abnormality, the karyotype of both fetal lymphocytes and fetal fibroblasts was normal. Autopsy confirmed dilated lymphatic channels in the basal endothelial layer of the skin, cystic hygroma, and coarctation of the aorta. Although previously unreported, we suggest that the features of this case of achondrogenesis indicate an association with lymphatic stasis and jugular lymphatic obstruction sequence in this syndrome.

Adult↗

Lymphatics in the alimentary tract of children in health and disease: study on mucosal biopsies using the monoclonal antibody d2-40.

Primary intestinal lymphangiectasia and intestinal lymphatic hypoplasia are 2 causes of protein-losing enteropathy in children and share many common clinical features. For the diagnosis of lymphatic hypoplasia on endoscopic biopsies of the intestine, i.e., based on a negative finding in a small specimen, a very sensitive and specific method for identifying lymphatics is essential. In the present study, lymphatic vessels were labelled using D2-40 immunostaining in mucosal biopsy specimens of the alimentary tract of children in whom no histologic abnormality was noted and of those who had different relatively common pediatric conditions, including inflammatory and neoplastic diseases. Using this method, lymphatic vessels were well visualized even in young infants and not destroyed by diseases. The presence of the muscularis mucosae in specimens was important for adequate assessment. In the duodenum and esophagus, lymphatics were observed in every single section; in the stomach, ileum, and colon, they were less regular and several sections were sometimes required. The extreme sensitivity of this method for demonstrating lymphatic vessels in the duodenum makes it ideal for the histologic diagnosis of intestinal lymphatic hypoplasia. In 4 patients who were considered to have this diagnosis based on clinical features, full-thickness intestinal biopsies and electron microscopy, D2-40 immunostaining confirmed the absence or marked paucity of lymphatics.

Antibodies, Monoclonal↗

Lymph vessel defects in patients with ovarian dysgenesis.

Lymphangiography of the lower extremities was performed in 21 patients with ovarian dysgenesis. Seventeen subjects turned out to have hypoplastic superficial lymph vessels of the lower limbs. Aplasia of the lymphatics of the legs occurred in four instances. Hypoplastic lymph pathways were also observed in the pelvis and in the retroperitoneal space. Although the impairment of the lymphatic channels occurred in all cases of ovarian dysgenesis, it appears that it is more severe in patients with streak gonads. There was no correlation between the lymphatic defect and the karyotype. Patients with and without lymphedema had similar lymphatic defects. Our findings suggest that hypoplasia of the superficial lymph vessels of the lower limbs is a common abnormality in patients with ovarian dysgenesis irrespective of their chromosomal complements. The hypothesis that lymphedema in ovarian dysgenesis is mainly attributable to hypoplasia of the lymphatic channels is supported by this study. However, it appears that other factor(s) may also play a role in the pathogenesis of the lymphedema, since, despite lymphatic hypoplasia, lymphedema does not develop in all instances.

Adolescent↗

Linking hematopoiesis to endochondral skeletogenesis through analysis of mice transgenic for collagen X.

Each skeletal element where marrow develops is first defined by a hypertrophic cartilage blueprint. Through programmed tissue substitution, the cartilaginous skeletal model is replaced by trabecular bone and marrow, with accompanying longitudinal tissue growth. During this process of endochondral ossification, hypertrophic cartilage expresses a unique matrix molecule, collagen X. Previously we reported that transgenic mice with dominant interference collagen X mutations develop variable skeleto-hematopoietic abnormalities, manifested as growth plate compressions, diminished trabecular bone, and reduced lymphatic organs (Nature 1993, 365:56). Here, histology and flow cytometry reveal marrow hypoplasia and impaired hematopoiesis in all collagen X transgenic mice. A subset of mice with perinatal lethality manifested the most severe skeletal defects and a reduction of marrow hematopoiesis, highlighted by a lymphocyte decrease. Thymic reduction is accompanied by a paucity of cortical immature T cells, consistent with the marrow's inability to replenish maturing cortical lymphocytes. Diminished spleens exhibit indistinct lymphatic nodules and red pulp depletion; the latter correlates with erythrocyte-filled vascular sinusoids in marrows. All mice display reduced B cells in marrows and spleens, and elevated splenic T cells. These hematopoietic defects underscore an unforeseen link between hypertrophic cartilage, endochondral ossification, and establishment of the marrow microenvironment required for blood cell differentiation.

Animals↗

The role of radiology in the assessment of bladder cancer.

Bipedal lymphography and intravenous urography were carried out in 44 patients in an attempt to define the role of these procedures in the assessment of the patient with carcinoma of the bladder. Evidence of lymphatic involvement, even though unsupported by histological proof and a major abnormality on urography, conferred a worse prognosis than if these examinations were thought to be normal. Implications for therapy are considered.

Aged↗

Vulval lymphangiectasia.

Three cases of lymphangiectasia of the vulva are reported. One case followed Wertheim's hysterectomy for carcinoma of the cervix; two other cases had Crohn's disease with perineal involvement. Lymphangiectasia is a secondary phenomenon resulting from obstruction of previously normal lymphatics. This is in contrast with lymphangioma which is an anatomical abnormality. A review of the literature suggests that the vulval skin may be particularly susceptible to the formation of lymphangiectasia, and our patients' experiences suggest that they are easily mis-diagnosed.

Adult↗

Pediatric AIDS: a longitudinal comparative MRI and CT brain imaging study.

Fourteen consecutive children with acquired immune deficiency syndrome (AIDS) (age range, 4 months to 11 years; median 4 years) were studied prospectively comparing nonenhanced cranial magnetic resonance imaging (MRI) and computed tomographic (CT) scans. MRI and CT were performed twice: at time of entry into protocol and again at 1 year. In addition, sequential neurologic (every 2 months) and neuropsychological examinations (every 6 months) were performed. At entry, 12 children had abnormal neurologic examinations; of these, 10 had developmental delay; two children were normal by developmental history and neurologic examination. Five children performed in the normal range on a standardized neuropsychological test, whereas nine children showed significant delays in verbal or motor/perceptual development. Following 1 year of study, four children had normal and six had abnormal neurologic examinations (six stable and four improved). Neuropsychological examinations were normal in five children and abnormal in five (seven stable, one improved, and two deteriorated). At entry, the following neuroradiographic abnormalities were seen: brain parenchymal volume loss (eight, MR = CT), cervical lymphatic enlargement (four, MR = CT), striatal-thalamic calcification (one, CT > MR), delayed myelination (one, MR > CT), and focal white-matter lesions (one, MR > CT). At 1 year the following neuroradiographic changes were seen: brain parenchymal volume loss (10, MR = CT; two improved, eight stable); cervical lymphatic enlargement (one, MR = CT; three improved, one stable), striatal-thalamic calcification (one, CT > MR; one new), and focal white-matter lesions (one, MR > CT; one stable).(ABSTRACT TRUNCATED AT 250 WORDS)

AIDS Dementia Complex↗

Forearm blood flow measured by venous occlusion plethysmography in healthy subjects and in women with postmastectomy oedema.

A chronic swelling of the arm may develop following damage to axillary lymph vessels during breast cancer treatment. Certain unusual pathophysiological features of postmastectomy oedema indicate that factors additional to lymphatic damage are important. One factor postulated previously is a haemodynamic abnormality. Forearm blood flow was therefore measured by venous occlusion plethysmography using both a conventional mercury strain gauge and a newer optical volumeter (Perometer). The latter was initially assessed in healthy subjects. When forearm blood flow was measured using the two methods simultaneously, the strain gauge and Perometer did not differ significantly for healthy or oedematous arms. Dominant and non-dominant arm blood flow in healthy subjects was similar but with greater variation between sides with the Perometer. Using the strain gauge, blood flow in the oedematous arm (2.51 +/- 1.33 ml/100 ml per min) was significantly lower than in the opposite unaffected arm (3.77 +/- 2.29 ml/100 ml per min; p = 0.026). The Perometer results showed a similar but nonsignificant trend (p = 0.105). Taking into account the greater limb volume on the affected side, total forearm blood flow was essentially the same in each arm (unaffected: 40.72 +/- 28.70 micromilligrams/min; oedematous: 41.78 +/- 22.11 ml/min). The reduced blood flow per unit volume of arm was therefore likely to be due to a simple 'dilution' effect of the increase in volume. The results indicate that it is unlikely that raised blood flow contributes to oedema formation, contrary to the haemodynamic concept.

Adult↗

The secondary pulmonary lobule: normal and abnormal CT appearances.

The secondary pulmonary lobule is a unit of lung supplied by three to five terminal bronchioles and contained by fibrous septa. High-resolution CT is able to show features of the secondary lobule, including interlobular septa, terminal bronchioles, and pulmonary arteries within a bronchiolovascular bundle. Because interstitial diseases have been shown to affect different components of the secondary lobule, high-resolution CT was used to compare the appearance of the normal secondary lobule with the abnormal secondary lobule in three patients with interstitial diseases primarily affecting lymphatic channels: lymphangitic carcinomatosis, sarcoidosis, and lymphangioleiomyomatosis. In lymphangitic carcinomatosis, the bronchiolovascular bundles and interlobular septa were thicker than those seen in a normal subject. In sarcoidosis, the interlobular septa and bronchiolovascular bundles were also thicker than normal. However, fibrosis caused distortion of the normal polygonal shape of the secondary lobule, not seen in either the normal subject or the patient with lymphangitic carcinomatosis. Lymphangioleiomyomatosis was characterized by multiple cysts within secondary lobules, often obliterating the normal polygonal appearance.

Adult↗

Discordant patterns of chromosome changes and myeloblast proliferation during the terminal phase of chronic myeloid leukemia.

A patient with Ph1 positive chronic myeloid leukemia (CML) developed blastic transformation which by morphologic criteria appeared to be localized to the lymphatic system. Chromosome analysis at this time, however, revealed new chromosomal abnormalities in addition to the existing Ph1 in all tissues studied (lymph node, blood, and bone marrow) consisting primarily of extra chromosome numbers 19 and 9 and a second Ph1. Therapy resulted in clinical remission with significant decrease in the aneuploid cell lines. However, these reappeared with recurrence of the blast crisis. Colony formation in semisolid culture of blood and marrow cells at the time of initial blast crisis yielded growth patterns characteristic of CML. On recurrence of the blast crisis after therapy, growth patterns were characteristic of CML in blast crisis or acute myeloblastic leukemia even though blood and marrow still showed relatively low levels of myeloblasts and promyelocytes. Possible explanations are discussed for the disparity in distribution between morphologic and chromosomal abnormalities in this patient.

Adult↗

The first-reported case of diffuse purulent peritonitis in a patient with retroperitoneal Hodgkin disease (etiopathogenetic hypotheses).

We report a case of purulent diffuse peritonitis in a patient who was affected by Hodgkin lymphoma, with no evidence of other abdominal diseases. This is a 54 y. old. white male who was admitted to our department with a history of asthenia, recurrent fever, dysphagia and abdominal pain. In the plain abdominal radiology pneumoperitoneum was evident. Duodenal perforation suspicion was confirmed by anamnesis and plain radiology which showed the presence of intra abdominal air. Emergency exploratory laparotomy showed a purulent diffuse peritonitis, which relapsed after multiple surgical toilettes and peritoneal lavage. A postoperative abdominal CT scan and histology of a biopsy taken during the second surgical operation showed a retroperitoneal Hodgkin lymphoma, which went to remission after chemotherapy. Considering the two simultaneous clinical manifestations (retroperitoneal Hodgkin lymphoma and peritonitis), we made two pathogenetic hypotheses: a) The retroperitoneal disease produced lymphatic stagnation and peritoneal transudation, which then was infected; b) The abnormal lymph nodes were infected and the abdominal cavity was contaminated from retroperitoneum from blood/lymphatic stream or by contiguity.

Hodgkin Disease↗

Platelet function in chronic leukemias.

Bleeding time, clot retraction, platelet factor 3 availability and platelet aggregation in response to ADP, epinephrine, collagen and ristocetin were studied in 12 cases of chronic leukemia which included eight of chronic myeloid leukemia, two of chronic lymphatic leukemia and two of CLL related disorders. One or more abnormalities in platelet function were detected in all the cases. Among the cases of CML, bleeding time was prolonged in one, clot retraction was impaired in one and PF3 availability was decreased in one case. Defects in platelet aggregation were variable. Among the cases of CLL and CLL related disorders, bleeding time was prolonged in two, clot retraction was impaired in one and PF3 availability was decreased in three cases. Platelet aggregation responses were significantly impaired in all the cases.

Blood Platelets↗

[Ureteritis].

We relate our experience about ureteritis, especially non specific ureteritis. The traumatic, radiation ureteritis will be discussed in others chapters. Most cases of ureteritis are infective, and may be due to any of the organism normally found in urinary tract infections, particularly Escherichia Coli, staphylococci, streptococci, enterococci, proteus and pyocyaneus. It is really primary, but it usually ascending from an associated cystitis, descending from pyelonephritis, or due to direct spread from and adjacent inflammatory lesion such as appendicitis or salpingitis. The infection may also reach the ureter by lymphatic spread, particularly from the prostate and seminal vesicles. Any associated abnormalities of the ureter, such as stricture, megaloureter, ureterocele, and so on, will naturally predispose to infective ureteritis. As ureteritis is rarely primary, the first step in treatment must be toward the elucidation and cure of any underlying lesion. Thus calculi, cystitis, pyelitis, and so on, will need appropriate therapy, and this in itself will considerably improve or cure the ureteritis, and specially in the more acute cases. In the chronic cases with stricture formation, dilation or even excision of the stenosed portion may be required. For the treatment of the strictures we want emphasize the role of the ureteral stenting thinking its use is necessary to preserve the renal function.

Bacterial Infections↗

[Involvement of the lymphatic system in primary non-lymphogenic edema of the leg. Studies with 2-compartment lymphoscintigraphy].

Two-compartment lymphoscintigraphy was developed to examine the sub- and epifascial lymphatics of the leg. Digital images were evaluated visually and semiquantitatively by calculating the uptake of activity within the lymph nodes. The data from patient groups with four different types of leg edema were compared with those of the control group to prove the involvement of the lymphatics in the non-lymphatic edema. The cyclic idiopathic edema demonstrated an accelerated transport of the lymph consistent with a high volume insufficiency. In phlebedema the high volume insufficiency was epifascially so distinct, that it could be detected scintigraphically. In post thrombotic syndrome the transport of the lymph was reduced dramatically corresponding to a safety valve insufficiency. Epifascially however, an accelerated lymph flow was observed due to compensatory mechanisms. The lipedema did not show any scintigraphic abnormalities. These results show that two-compartment lymphoscintigraphy can detect alterations in lymphatic function secondary to non-lymphogenic leg edema. The lymphatic function is changed according to the underlying pathophysiology which may be facilitate the differential diagnosis of such a leg edema.

Adult↗

Immunohistochemical study of carcinoembryonic antigen in patients with colorectal cancer. Correlation with plasma carcinoembryonic antigen levels.

Using peroxidase-antiperoxidase (PAP) method, carcinoembryonic antigen (CEA) was demonstrated in conventionally processed colorectal cancer tissues. A new immunohistochemical grading for colorectal cancers based on the mode of the localization was made in an attempt to clarify the factors responsible for elevation of plasma CEA levels in colorectal cancer patients. Most of the patients with well differentiated adenocarcinoma, in which CEA was densely distributed along the apical surface but only rarely present along the basolateral surfaces of the carcinoma cells, had very low levels of plasma CEA, whereas all patients showing CEA distribution in the stroma as well as over the entire surfaces of the cancer cells and their cytoplasm showed high plasma CEA levels. In addition, there was a good correlation between the grading and presence of the blood vessel and lymphatic invasions. Thus, the appearance of CEA in the surrounding stroma, due to abnormal distribution of CEA on the basolateral plasma membrane of cancer cells, may play a significant role in the elevation of plasma CEA levels in colorectal cancer patients.

Adenocarcinoma↗

Pathogenesis of the lethal multiple pterygium syndrome.

We present autopsy studies in 4 unrelated fetuses with the lethal multiple pterygium syndrome (LMPS) with special emphasis on the neuromuscular system. The data suggest that LMPS combines the manifestations of a jugular lymphatic obstruction sequence with those of an early severe fetal akinesia sequence. The jugular lymphatic obstruction sequence with resultant edema and cystic hygroma colli causes fetal lethality usually in the second trimester of pregnancy. Generalized amyoplasia appears to be an important mechanism in the pathogenesis of fetal akinesia as part of LMPS and is not associated with dysgenesis or degeneration of the central nervous system (CNS) but is apparently the result of an early fetal muscular "dystrophy." We propose a genetically determined insult affecting the early embryonic development of both lymph vessels and muscles as the basic defect in LMPS. Placental structure, studied in all 4 cases, demonstrated that triploidy-like placental lesions are specific to LMPS. The present findings suggest that LMPS may be a less heterogeneous entity than previously proposed.

Abnormalities, Multiple↗