[Cardiac arrhythmias and superior vena cava syndrome revealing a Richter's syndrome]. / Troubles du rythme cardiaque et syndrome cave supérieur révélateurs d'un syndrome de Richter.

Richter's syndrome is the aggressive transformation of chronic lymphocytic leukemia in a diffuse large cell lymphoma. The locations and the clinical manifestations are varied. We report the case of a Richter's syndrome revealed by cardiac arrhythmias and superior vena cava syndrome in a patient of 78 years followed during 2 years for chronic lymphocytic leukemia.

The differential expression of Galectin-1 and Galectin-3 in normal lymphoid tissue and non-Hodgkin's and Hodgkin's lymphomas.

The WHO classification of lymphomas was established on the basis of clinical, morphological, immunohistochemical and genetic criteria. However, each entity displays its own spectrum of clinical aggressiveness. Treatment success varies widely and is not predictable. Since galectins are involved in oncogenesis and the physiology of immune cells, we investigated whether galectin-1 and galectin-3 immunohistochemical expression could differ in 25 normal lymphoid tissues, 42 non-Hodgkins and 14 Hodgkins lymphomas. Immunohistochemical galectin expression was submitted to semi-quantitative and quantitative (computer-assisted microscopy) evaluations. This study is completed by an analysis (by means of quantitative RT-PCR) of galectin-3 mRNA expression in 3 normal lymph nodes, 3 follicular lymphomas (FLs) and 3 diffuse large B-cell lymphomas (DLBCLs). The data show that in normal lymphoid tissue, lymphocytes do not express galectin-1 and rarely express galectin-3. In contrast, galectin-3 was expressed in 8 of the 16 DLBCL cases and in 1 of the 8 FL cases. Furthermore, galectin-3 mRNA was expressed 3 times more in the DLBCLs than in the FLs. While the blood vessel walls of the lymphomas expressed galectin-1, the vessel walls of normal lymphoid tissues did not. This expression of galectin-1 in blood vessel walls was correlated with vascular density. The present study thus shows that DLBCL can be distinguished from normal lymphoid tissue and other lymphomas on the basis of galectin-3 expression.

Heterotopic gastric mucosa in the upper esophagus: a prospective study of 33 cases and review of literature.

BACKGROUND AND STUDY AIMS: The prevalence of endoscopically diagnosed heterotopic gastric mucosa in the upper esophagus (HGMUE) has been reported in a few studies, and varies from 0.1 to 10%. Clinical relevance and possible association with other pathological conditions remain a matter of debate. A prospective study was carried out to determine the prevalence of HGMUE, the influence on it of age and sex, and to study the macroscopic and microscopic aspects of the lesion, its clinical relevance and possible association with other pathological conditions. PATIENTS AND METHODS: A total of 674 new patients with upper digestive complaints or alteration of their state of health underwent upper gastrointestinal endoscopy, with special attention paid to the proximal esophagus when withdrawing the gastroscope. They had been carefully questioned, especially regarding possible complaints, which could have drawn attention to the upper esophagus. RESULTS: Heterotopic columnar epithelium in the proximal esophagus was found in 4.9 % of patients. No difference was observed according to age or sex. A mild to moderate chronic inflammatory infiltration of the heterotopic patch was observed in most cases, not related to the presence in the lesion of Helicobacter pylori, which was found in only one case. Pathological conditions of the gastroesophageal junction, especially esophagitis, were slightly more frequent in patients with HGMUE. Mild complaints, possibly related to the presence of the lesion, were observed in three out of the 33 cases. CONCLUSIONS: On the basis of our prospective study we consider that heterotopic columnar epithelium in the proximal esophagus is a rather common, generally asymptomatic, benign congenital anomaly. Malignant transformation of heterotopic gastric mucosa in the upper esophagus and other severe complications are rare. The need for surveillance should be reserved for the rare cases with metaplasia or dysplasia in the heterotopic columnar mucosa.

Anaplastic large cell lymphoma of true histiocytic origin in an infant: unusual clinical, hematological, and cytogenetic features.

We report here the case of a 7-month-old boy who developed anaplastic large cell lymphoma of true histiocytic origin or malignant histiocytosis, with fever, bone and bone marrow infiltration. Usual clinical features were absent. The neoplastic nature of the disease was supported by the presence of clonal chromosomal abnormalities [t(6;8)(p23;p21),der(8)del(8)(q11aq13), der(22) t(11;22) (q13;13)]. Neither B nor T lineage could be demonstrated here. Morphology, ultrastructural analysis, surface antigens expression, and cytogenetics were more specific for the monocyte-macrophage lineage.

Progressive paraparesis due to thoracic extramedullary hematopoiesis in myelofibrosis. Case report.

A patient with myelofibrosis who developed a progressive paraparesis caused by spinal cord compression due to thoracic extramedullary hematopoietic tissue is reported. He recovered well after local radiotherapy alone.

Leukemia and lymphoma immunophenotyping in cell smears with immunogold-silver staining.

The potential of the immunogold-silver staining (IGSS) technique for immunophenotyping leukemia and lymphoma cells in cell smears was examined. Peripheral blood, bone marrow aspirates, lymph node biopsy specimens, fine-needle aspirates, and biologic fluids of 83 patients with acute or chronic leukemias, non-Hodgkin's lymphomas, or Hodgkin's disease were labeled. Cell smears, cytocentrifuge preparations, or imprints were fixed, incubated with the reagents, and counterstained with May-Grünwald-Giemsa. Stable immunostaining and good morphologic characteristics allowed accurate cell identification and rapid enumeration of the positive cells. The immunophenotypes obtained with the use of 35 monoclonal antibodies with different specificities were similar to those determined by flow cytometry or immunohistochemical studies on the same samples. This IGSS method was especially useful for the examination of poor samples or complex cell suspensions with rare malignant cells. It could be an alternative to the immunoenzyme methods that generally are used for this purpose.

Southern blot analysis in a case of Richter's syndrome. Evidence for a postrearrangement heavy chain gene deletion associated with the altered phenotype.

Richter's syndrome (RS) can be defined as the emergence of an aggressive lymphoma in patients suffering from chronic lymphocytic leukemia (CLL). The authors performed immunophenotypic and Southern blot analysis of the peripheral blood and tissue specimen of a patient with RS. Using immunoperoxidase and immunogold-silver staining techniques and a panel of monoclonal antibodies, the authors found that the large cells characteristic of RS showed an altered immunophenotype as compared with the CLL cells and did not express mu heavy chain. Southern blot analysis revealed identical kappa light chain rearrangements in both tumoral cell populations consistent with a common clonal origin. Using the JH probe and several restriction enzymes, the authors also found evidence for a postrearrangement deletion of the heavy chain mu gene. These findings suggest that in this case of RS, a deletion of the heavy chain mu gene resulted in loss of mu expression by the larger cells that were characteristic of RS and was associated with their altered phenotype.

Diagnostic and prognostic value of the MB2 monoclonal antibody in paraffin-embedded bone marrow sections of patients with multiple myeloma and monoclonal gammopathy of undetermined significance.

Routinely processed bone marrow biopsies of 59 patients with untreated multiple myeloma (MM) and of 41 patients with monoclonal gammopathies of undetermined significance (MGUS) were immunocytochemically studied with the MB2 monoclonal antibody. In 54 of 59 biopsies of patients with MM, most neoplastic plasma cells showed strong cytoplasmic positivity to MB2. In contrast, only three biopsies of patients with MGUS contained highly MB2-positive plasma cells, whereas the plasma cells in the remaining biopsies were either negative (18 of 41) or revealed a weak dot-like staining of the cytoplasm (20 of 41). Plasma cells in tonsillar tissue, gastric and duodenal mucosae, and bone marrow with reactive plasmacytosis were not stained with MB2. These findings suggest that MB2 helps to distinguish between MM and MGUS. Because the five MB2-negative patients with MM were all in stage III and had very short survival time, neoplastic plasma cell staining with MB2 could also have a prognostic significance.

Metastasizing neuroendocrine carcinoma of the larynx with calcitonin and somatostatin secretion and CEA production, resembling medullary thyroid carcinoma.

A 55-year-old man presented with a metastasizing moderately differentiated neuroendocrine carcinoma of the larynx (atypical carcinoid). Immunocytochemical demonstration of neuroendocrine markers (neuron-specific enolase and chromogranin-A) and presence of membrane-bound neurosecretory granules in the cells established the neuroendocrine nature of the tumour. In addition, the tumour was found to produce calcitonin, somatostatin and carcino-embryonic antigen (CEA). Calcitonin and somatostatin were also secreted. On the basis of this particular marker constellation the tumour closely resembles medullary thyroid carcinoma. Review of the recent literature on carcinoids of the larynx reveals immunoreactivity for calcitonin and CEA in a high percentage of cases.

Anaplastic small cell (oat cell) carcinoma of the tonsils: report of two cases.

Two cases of anaplastic small cell (oat cell) carcinoma of the tonsils are presented. In the first, cervical metastases preceded the manifestation of the primary tumour by 2 years. In case 2 the tonsillar carcinoma was accompanied by a bronchial tumour of the same histological type and by cervical and axillary metastases. Positive Grimelius stain, positive immunohistochemical staining for chromogranin A and neurone-specific enolase and the presence, in case 1, of membrane-bound granules indicate that these tumours display many similarities with neuroendocrine carcinomas even if they originate from pluripotential ductal cells of tonsillar minor salivary glands and not from Kulchitsky-like cells.

Endometrial morphology in stimulated in vitro fertilization cycles and after steroid replacement therapy in cases of primary ovarian failure.

Endometrial morphology and ultrastructure are studied in 17 spontaneous, 23 stimulated, and 18 artificial cycles in cases of primary ovarian failure. Normal light-microscopic aspect was found, but impaired development of nucleolar channel system and stronger intercellular junction have been observed by electron-microscopic studies in stimulated cycles with relative excess of luteal estrogen. Normal glandular maturation can be obtained in patients with premature menopause, given adequate steroid replacement, but an abnormally dense fibrocytic aspect of the stroma is characteristic of the first treatment cycles.

Detection of cell surface antigens in cryostat sections with immunogold-silver staining.

Immunogold-silver staining was used for the detection of lymphocyte cell surface antigens in cryostat sections of lymphoid tissues. The sections were incubated with monoclonal mouse antibodies and then with colloidal gold-labeled goat anti-mouse antibodies. They were then immersed in a physical developer, counterstained, and mounted. In light microscopy, the tissue architecture was well preserved, and a dark labeling was seen on the positive cells. Optimal labeling conditions were determined. The distribution of the lymphocyte subsets, as defined by a panel of monoclonal antibodies in tonsil and reactive lymph nodes, was similar to that found with a biotin-avidin-horseradish peroxidase method. The monoclonality of the neoplastic cells in lymph nodes of B-cell non-Hodgkin's lymphomas clearly could be demonstrated. The sensitivity of the technic was comparable with that of the biotin-avidin-horseradish peroxidase labeling method. In addition, immunogold-silver labeling was combined with acid phosphatase cytochemistry.

Unusual nonimmunoglobulin-containing inclusions in a case of follicular large cell lymphoma.

Intracytoplasmic inclusion bodies were found in a case of follicular large cell lymphoma. They did not react with anti-immunoglobulin antisera and showed no enzyme reactivity. On electron microscopy the inclusions consisted of loosely packed fibrillar material not surrounded by a membrane or by rough endoplasmic reticulum. They were found only in the large lymphomatous cells. Immunocytochemistry showed a reactivity of these cells with anti-HLA-Dr and the OKT10 monoclonal antibodies. The nature of the inclusions remains unknown. They differ significantly from those described in the literature in cases of B-cell lymphoproliferative disorders.