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Biomedical subjects

M Tulliez

Publications and source records attributed to M Tulliez.

At least 109 records · Page 6Linked to original sources
PubMed1981

Beta-thalassemia and sickle cell disease in culture of early erythroid precursors: hemoglobin synthesis and ultrastructural study.

Hemoglobin synthesis was studied in culture of early erythroid precursors (BFU-E) from the blood of nine patients exhibiting sickle cell anemia and of 14 with various types of beta-thalassemia. The results concerning gamma gene expression and plating efficiency in heterozygotes for sickle cell anemia were similar to those of normal adults (gamma/alpha = 0.05; 65 BFU-E colonies/10(6) plated cells) while, in contrast, homozygotes for sickle cell disease exhibited average values higher than the controls (gamma/alpha = 0.18; 80 BFU-E colonies/10(6) plated cells). However, the results were very heterogeneous from one subject to another. In heterozygotes for beta-thalassemia, gamma gene expression and plating efficiency were both slightly higher than those for normal individuals (gamma/alpha = 0.095; 129 BFU-E colonies/10(6) plated cells). In patients homozygous for beta-thalassemia, a marked increase in plating efficiency and gamma-chain synthesis was constantly observed (gamma/alpha = 0.41; 221 BFU-E colonies/10(6) plated cells). The high proportion of gamma chain synthesis was not related to a positive selection of F cells, since the gamma/alpha ratio remained constant during the in vitro erythroid maturation. Furthermore, a major increase in free alpha chain proteolysis can be ruled out, since the beta/alpha ratio was of the same order of magnitude in culture and in freshly drawn cells. Thus, the increased Hb F synthesis in vitro was the consequence of a true stimulation of gamma gene expression, which permitted partial correction of the globin chain imbalance. Ultrastructural studies in two homozygotes for beta-thalassemia showed a marked decrease in the abnormalities of the erythroblasts derived from erythroid precursors in vitro in comparison to those from fresh bone marrow samples. In particular, Heinz bodies were much less numerous and a high frequency of mature erythroblasts and reticulocytes was observed in culture. These results support the view that, in sickle cell anemia and beta-thalassemia, a high potential for gamma gene expression exists and can be expressed in culture.

Anemia, Sickle Cell↗
PubMed1981

[A new plasma exchange technique : coagulation studies (author's transl)].

Coagulation studies were performed during and after 60 continuous plasma exchanges in 15 patients with neurologic disorders. One or several plasmapheresis were performed on each patient using a new filtration material "Plasmaflo" from Asahi Medical, Tokyo. During each exchange involving massive heparin infusion, two total plasma volumes were removed and replaced isovolumetrically by a material devoid of clotting factors (albumin and Plasmion). The variation of the platelet count was moderate (mean reduction of 19%). An important and rapid fall of all coagulation factors was observed, and during a single exchange procedure the extent of the fall was comparable for all coagulation factors. The mean reduction during plasmapheresis was 70%. However, a difference in the rapidity of normalisation was observed between the various proteins (F.V. : 24 h, A.T.III : 72 h). A particular property was noted concerning factor VIII. In all cases, a normal or high level of VIII : C as well as of VIII R : Ag was reached 3 hours after exchange. In some cases an increase could be observed even during plasmapheresis. No hemorrhagic diathesis was noted despite massive heparin administration to the patients and a striking decrease of blood procoagulant factors. On the other hand, one patient developed femoral vein thrombosis following the 8th exchange.

Blood Coagulation Factors↗
PubMed1980

Lymphoblastic lymphoma/leukemia with convoluted nuclei: the question of its relation to the T-cell lineage studied in 13 patients.

This work is devoted to the analysis of the nature of lymphoblastic lymphoma/leukemia with convoluted nuclei which were initially described by Barcos and Lukes. Ultrastructural, cytochemical, and immunologic features of tumor cells were investigated in patients chosen according to known morphologic criteria. Through results of the E rosette test, the patients were divided into two groups (E+ and E-). In the E+ group, the predominant features were sex (only men), the mediastinal localization, and the focal positivity of the acid phosphatase reaction. Cytotoxicity tests with rabbit antihuman T-lymphocyte anti-serum confirmed the results of the E rosette test in the 3 patients of the E+ group who were tested and were also positive in 2 patients from the E- group (1 of these 2 patients had the characteristics found in the E+ group and can thus be related to this group; the other patient had none of these characteristics). This raises the question of a leukemia arising from a less differentiated T-cell but this interpretation is limited by the specificity of the anti-T-serum. Ultrastructural study defines more precisely the convoluted aspect but does not at present allow a distinction between the two groups.

Adolescent↗
PubMed1980

Acute non myeloid leukaemia in adults. Prognostic factors in 92 patients.

An analysis of prognostic factors was performed on a series of 92 adult patients with acute non-myeloid leukaemia (ANML). Four factors were shown to be correlated with a poor short-term prognosis: (1) Neuropathy involving a single cranial nerve (numbness of the chin) as a presenting feature. (2) A low percentage blast cell infiltration of bone marrow (less than 55%). (3) Cytological L2 type or L3 type of cells according to FAB classification proposals. (4) Increased age. Two factors were shown to be correlated with a poor long-term prognosis: 1) A high level of circulating blast cells; 2) A high tumour burden, as in childhood ANML. These two long-term prognostic factors were interrelated. In addition, our results suggested that myelofibrosis could be a poor long-term prognosis factor as well.

Acute Disease↗
PubMed1980

Angiofollicular and plasmacytic polyadenopathy: a pseudotumourous syndrome with dysimmunity.

Two cases are reported of an apparently distinct type of immune disorder. Beginning with mild anaemia and widespread massive lymphadenopathy, the disease progressed to a fatal autoimmune type haemolytic anaemia. Serum investigation showed polyclonal hypergamma-globulinaemia and some autoantibodies. Repeat lymph node biopsies in each case showed hyperplasia within B lymphocyte territory (follicular hyperplasia and polyclonal plasmacytosis with IgG predominance) and atrophy of T dependent areas. Dilatation of lymph sinuses, vascular proliferation, and sclerosis were striking features. This appears to be a new entity, and reasons are given for separating this disease from other pseudotumourous lymph node disorders associated with dysimmunity.

Aged↗
PubMed1980

[Visceral Kaposi sarcoma with angioimmunoblastic lymphadenopathy and latent sicca syndrome (author's transl)].

A case clinically and morphologically typical angio-immunoblastic lymphadenopathy in a 64-year old man is reported. A sicca syndrome was discovered with histological localization in the labial accessory salivary glands. Death occurred after a course of a few months. The autopsy showed an association of angio-immunoblastic lymphadenopathy lesions of the bone marrow and the lymph node and of typical multivisceral localizations of Kaposi's sarcoma (lymph node, liver, thyroïdd gland) without skin involvement. This association of two diseases characterized by severe dysimmunity with B lymphocytes hyperplasia is discussed, and compared to the others diseases which could be associated with Kaposi's sarcoma.

Female↗
PubMed1979

Humoral and cellular immunity to intrinsic factor in myasthenia gravis.

Myasthenia gravis (MG) is an autoimmune disease often associated with other autoimmune disorders. A case history of MG with a coexisting atypical megaloblastic anaemia with vitamin B12 deficiency and anti Intrinsic Factor (IF) antibodies, led to a study of humoral and cellular immunity to IF in 81 MG patients. Within this series, 3 other patients had a disturbed humoral and cellular immunity to IF. These 3 patients presented no other features of pernicious anaemia. The possible origins and significance of the anti IF antibodies in MG patients are discussed.

Adolescent↗
PubMed1979

Three types of Auer bodies in acute leukemia. Visualization of their protein by negative contrast after peroxidase cytochemistry.

Ten cases of acute granulocytic leukemia with blast cells containing Auer bodies (ABs) have been studied by electron microscopy after cytochemical demonstration of myeloperoxidase. The cytochemical dense reaction product has been used as a dark field to visualize unreactive protein of ABs which may then be easily identified by its negative contrast. This method has allowed us to identify three types of ABs which differ in their substructure. In type I, (five patients with promyelocytic leukemia), our study confirmed that all of the ABs consisted of a hexagonal arrangement of hollow tubes. Cells from four cases of acute myeloblastic leukemia displayed type II ABs, in which a unique pattern of protein associated in a regular linear arrangement with well defined periodicity was identified. Type III appeared characteristic of a subclass of acute myeloblastic leukemia in which large inclusions with Chediak-Higashi-like granules containing numerous micro-ABs were seen. The configuration, size, and organization of the protein in the crystal were distinct from those seen in the two other types of ABs. These features suggest that the nature of the protein in ABs may be heterogeneous.

Cytoplasmic Granules↗
PubMed1979

Pseudo-Chediak-Higashi anomaly in a case of acute myeloid leukemia: electron microscopic studies.

The formation and fine structure of giant granules in neutrophil promyelocytes of a patient with a variant of acute myelogenous leukemia were investigated by electron microscopy. The patient presented with large lymph nodes and disseminated intravascular coagulation (DIC). By light microscopy, numerous giant granules, resembling those of Chediak-Higashi syndrome (CHS), were present, but Auer bodies could not be found. By electron microscopy, these giant granules were seen to be formed by fusion of azurophilic granules, as in CHS; however, they were different from the large granules of CHS, since they contained numerous microcrystalline structures like those of Auer bodies. However, the crystalline cores of these granules exhibited a periodicity different from that of Auer bodies of acute promyelocytic leukemia. This clinical and hematologic syndrome (giant granules, enlarged lymph nodes, and DIC may represent a variant of acute promyelocytic leukemia.

Adult↗
PubMed1979

[Myasthenia and pernicious anemia or Biermer's (author's transl)].

The association of myasthenia and Biermer's anemia is very rarely reported. In a series of 138 cases of myasthenia, this association was found in only one patient, in whom the anemia developed 19 years after the discovery of a calcified thymoma and 13 years after the appearance of the first signs of myasthenia. This led the authors to conduct a prospective study for the presence of intrinsic antifactor antibodies. A total of 81 patients (20 men and 61 women) with myasthenia were studied. The myasthenia had appeared after 35 years of age in 40 patients and 19 had a thymoma. The results of the study for the antibodies was positive in 3 women, as was the test of inhibition of leucocyte migration, but none of them had anemia, vitamin B12 malabsorption, achlorhydria, or gastric atrophy. The discovery of these immunological disorders raises the problem of their significance ; two hypotheses can be discussed : pre-Biermer state or immunological disturbance without pathogenetic significance. The problem can probably only be resolved by studying these antibody levels in a very much larger number of patients with myasthenia.

Adult↗
PubMed1978

The blast crisis of chronic granulocytic leukaemia: megakaryoblastic nature of cells as revealed by the presence of platelet-peroxidase--a cytochemical ultrastructural study.

The origin of cells in the blast crisis of some cases of chronic granulocytic leukaemia (CGL) remains controversial. Difficulties arise from the lack of cytochemical characteristics of differentiation. This report concerns the nature of cells in the blast crisis of a case of CGL in which blast cells exhibited an undifferentiated or lymphoid appearance by light and electron microscopy. The majority (90%) of such cells contained a peroxidase in the endoplasmic reticulum distinct from myeloperoxidase. In addition, some micromegakaryocytes could be recognized among the peroxidase reactive cells, by the presence of typical granules and demarcation membranes. Since this peroxidase exhibited identical characteristics to that of normal megakaryocytic precursors, these blast cells could be identified as megakaryoblasts. These data emphasize the possible megakaryoblastic nature of cells occurring in other cases of CGL blast crisis.

Adult↗
PubMed1978

Beta thalassemia with reticulocytopenia: clinical, biochemical, and ultrastructural studies.

An unusual form of beta thalassemia is described in two children of unrelated families. Its main features are a severe anaemia with a low reticulocyte count associated with an erythroblastic hyperplasia; these characteristics indicate a completely ineffective erythropoiesis. The results of the study of haemoglobin synthesis performed on the bone marrow in vitro showed a greater imbalance in chain synthesis than that typically found in Cooley's anaemia. Ultrastructural studies revealed, in erythroblasts, all the features observed in Cooley's anaemia, although these features were more widely encountered. In addition, two peculiar findings were noted: (1) many inclusion bodies, which were partially or totally surrounded by smooth membranes, and which may indicate an autophagic phenomenon; and (2) a spongy appearance of the chromatin in rare erythroblasts of one of the two patients which resembled that found in congenital dyserythropoietic anaemia type I. These findings suggest that the great imbalance in the synthesis of the haemoglobin chain is responsible for the presence of an increased number of inclusion bodies and results in the death of nearly all the late erythroblasts. Furthermore, the present results point out the need for further study of the synthesis of the globin chains in atypical congenital dyserythropoietic anaemias.

Blood Cell Count↗