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J Pris

Publications and source records attributed to J Pris.

At least 73 records · Page 4Linked to original sources

Polymorphonuclear functions in Hodgkin's disease patients at diagnosis, in remission, and in relapse.

Five tests investigating different aspects of the nonspecific defense mechanisms including capillary tube random migration, particle ingestion activity, quantitative and histochemical nitroblue tetrazolium dye reduction by polymorphonuclear neutrophils, and serum lysozyme concentrations were performed in 46 patients with Hodgkin's disease. The anomalies observed in the active stage of the disease consisted of a decreased random migration, a high level of serum lysozyme, and an increased nitroblue tetrazolium reduction by resting phagocytes associated with a decrease in nitroblue tetrazolium reduction by stimulated phagocytes. The particle ingestion activity was normal. The serum lysozyme assay was the only test observed to normalize in the group of patients in remission. Its determination, therefore, offers an additional means of evaluating disease activity.

Adolescent↗

Richter's syndrome. Evidence for the clonal origin of the two proliferations.

A case of Richter's syndrome was investigated by several technics: light and electron microscopy, surface markers, immunohistological studies and immunoelectron microscopy. On light microscopy lymph node proliferation was composed of large lymphoid cells, some exhibiting Reed-Sternberg like features. On electron microscopy many intermediary cells, from small lymphocytes to immunoblasts and plasma cells, were noted. The circulating small lymphocytes were characterized as B cells (SIg +; MRBC+). The lymph node cells shared the same SIg phenotype (IgMK) but the percentage of MRBC was slightly lower. Immunohistological studies showed that lymph node cells were stained exclusively by anti micron and anti kappa antisera. These results are indicative of the B clonal origin of the two cell proliferations. Immunoelectron microscopy showed three staining patterns: 1) cells presenting only surface staining, 2) cells with diffuse staining presumably related to ribosomal fixation, 3) cells with endoplasmic reticulum staining. The significance of these three patterns is discussed with regard to B lymphocyte differentiation.

Aged↗

Platelets in myeloproliferative disorders. III: Glycoprotein profile in relation to platelet function and platelet density.

Membrane glycoproteins (GP) are implicated in platelet functions. In myeloproliferative diseases (MD), some of these functions are known to be perturbed. 16 patients with various MD were investigated for platelet functions (retention to glass beads, epinephrine- and ristocetin-induced aggregation), platelet density distribution and PAS-staining glycoprotein profile on SDS-polyacrylamide-gel-electrophoresis. An abnormal GP pattern (moderate reduction of GP (Ib + Is) and GP IIb with corresponding increase in GP IIIb) was demonstrated but no relation to platelet dysfunction or density distribution was observed. No differences between the various types of MD were noticed although the group of polycythaemia vera was the less perturbed for platelet function, platelet density and also GP profile.

Blood Platelets↗

Phagocytosis in myeloproliferative disorders.

The phagocytic function was investigated by means of four tests (capillary tube random migration, phagocytosis of yeast particles, quantitative nitroblue tetrazolium dye reduction and whole-blood bactericidal activity for Staphylococcus aureus) in 57 patients who had myeloproliferative disorders: 24 had chronic granulocytic leukemia, 22 had polycythemia vera, six had myelofibrosis, and five had essential thrombocythemia. This study confirms the previously reported functional anomalies of phagocytosis in all the myeloproliferative disorders and shows that, despite these anomalies, the increased number of phagocytes allows an efficient whole-blood bactericidal activity, essential for the nonspecific host defence mechanisms.

Blood Bactericidal Activity↗

Near haploid cell line in lymphoid blast crisis of Ph1-positive chronic myeloid leukemia.

This report describes a case of lymphoid blast crisis of a chronic myelocytic leukemia with the occurrence of a double chromosomal population carrying a Philadelphia chromosome. Fifty-five % of the cells have 28 chromosomes, and 36% show the exact duplicate of the near haploid chromosome complement. The similarities between this near haploid cell line and those previously reported, as well as the presence of such clones in acute lymphoblastic leukemia, are discussed. In the leukemic lymphoblasts, the association of the Philadelphia chromosomes with a near haploid karyotype described so far in acute lymphoblastic leukemia provides further support for the concept of a pluripotent Philadelphia chromosome-positive stem cell common to both lymphoid and myeloid lines.

Chromosome Aberrations↗

Platelets in myeloproliferative disorders. II. Serotonin uptake and storage: correlations with mepacrine labelled dense bodies and with platelet density.

Platelet serotonin (5-HT) uptake and storage in the presence and absence of reserpine were studied simultaneously with platelet volume, density and dense bodies content (mepacrine test) in 33 patients affected with myeloproliferative disorders (MD): 12 chronic myeloid leukaemia (CML), 9 polycythaemia vera (PV), 6 essential thrombocythaemia (ET) and 9 agnogenic myeloid metaplasia (AMM). Observations were (1) a dramatic reduction of the initial velocity (Vi) uptake and of the granular pool of 5-HT; (2) a slight reduction of the number of platelet dense bodies which, in many cases, were less fluorescent than in controls; (3) an increase of the percentage of light platelets while platelet volume was mostly normal; (4) a significant correlation between the number of dense bodies per platelet volume unit and either the percentage of light platelets (r = 0.76) or the size of the granular pool of 5-HT (r = 0.81). These results support evidence of a quantitative and qualitative acquired storage pool syndrome in these patients. In addition, the Vi studies demonstrate that the serotonin uptake across the plasmatic membrane is abnormal.

Blood Platelets↗

Platelets in myeloproliferative disorders. I. A comparative evaluation with certain platelet function tests.

Certain platelet functions were evaluated in 24 patients with secondary polycythaemia (SP) and in a large number of patients suffering from myeloproliferative disorders (MD'S): 89 patients with chronic myeloid leukaemia (CML) at different stages of development, 58 with polycythaemia vera (PV), 23 with essential thrombocythaemia (ET), and 25 with agnogenic myeloid metaplasia (AMM). Bleeding time, epinephrine-induced platelet aggregation and adhesiveness agreed with those generally reported in the literature; they are independent of thrombocytosis, the haemoglobin level and the leucocyte count. Macrothrombocytosis, evaluated by an electronic method, was only found in CML, mainly during acute blast crisis. An increased percentage of light platelets was a constant feature in all groups except in the SP and in 20% of the PV. The most severe abnormalities were observed in AMM and CML in the acute stage; in the chronic phase of CML there is no correlation between the severity of platelet abnormalities and the survival of the patients.

Blood Platelets↗

Leukoerythroblastosis and cancer frequency, prognosis, and physiopathologic significance.

This investigation was carried out on 100 bone marrow biopsies with metastases and 56 autopsies on patients with evidence of cancer. Leukoerythroblastosis was found in 44% of the patients with bone marrow mestastases and was more frequent in prostatic and gastric carcinoma. Moreover, the postmortem study of patients who died with cancer showed that leukoerythroblastosis was always the sign of bone marrow metastasis. A significant correlation was found between these blood changes and bone marrow fibrosis around the metastasis. Furthermore, leukoerythroblastosis seems caused by hepatosplenic extra medullary hematopoiesis.

Anemia, Myelophthisic↗

Platelet volume, density and 5 HT organelles (mepacrine test) in acute leukaemia.

Qualitative platelet parameters (volume, 5 hydroxy-tryptamine (5 HT) organelles studied by the mepacrine test, and density) were evaluated in 31 patients with acute leukaemia: 11 myelomonocytic (AML), 8 lymphoblastic (ALL), 12 granulocytic (AGL). Macrothrombocytosis was observed in most of the cases of AML, was rare in AGL and was never found in ALL. The 5 HT organelles/volume ratio was normal in AGL and ALL but was significantly decreased in AML. In contrast, platelet density distribution was always abnormal whatever the platelet volume and 5 HT organelle concentration. Thus, using simple new methods, convenient even in cases of thrombocytopenia, we demonstrate that qualitative platelet abnormalities are a constant feature in acue leukaemia and that they are more severe in AML.

Blood Cell Count↗

[Dysimmunologic and pseudolymphomatous adenopathies. I. Immunoblastic and plasmocytic lymphadenopathies].

The authors report 9 cases with pseudo-lymphomatous lesions associated with dysimmunitary features. They discuss the correlations between these cases and similar entities, for instance angio-immunoblastic lymphadenopathy (LAID). It seems that all these anatomoclinical syndromes could be referred to as dysimmunitary and pseudolymphomatous adenopathies (ADPL). The first type--ADPL type I--rich in immunoblasts and plasmocytes is defined. LAID is the most common form. The term "dysimmunitary" reflects not only the biological disturbances which accompany these lesions, but also their possible physiopathological mechanism, type I ADPL being apparently due to problem chiefly affecting humoral immunity.

Adolescent↗

[Dysimmunologic and pseudolymphomatous adenopathies. II. Lymphadenopathies rich in epithelial cells].

Besides the dysimmunitary and pseudo-lymphomatous adenopathies rich in immunoblasts and plasmocytes (ADPL type I) five cases showed similar clinical and biological data but with frequently otorhinolaryngologic location. The lesions are characterized by important structural changes and abundant epithelioid cells are comparable to Lukes' type III immunoblastic lymphadenopathies and to Lennert's lymphoepithelioid lymphomas. They must be distinguished from Hodgkin's granulomas which are rich in epithelioid cells. They are perhaps due to a disturbancy bearing mainly on the cellular immunity.

Bone Marrow↗