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Biomedical subjects

M Tulliez

Publications and source records attributed to M Tulliez.

At least 91 records · Page 5Linked to original sources
PubMed1987

Effects of granulocyte-macrophage colony-stimulating factor and erythropoietin on leukemic erythroid colony formation in human early erythroblastic leukemias.

Erythroid colonies from five patients with an early erythroblastic leukemia were obtained in "serum-free" cultures in the presence or absence of recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF) and homogeneous native erythropoietin (Epo). Erythroid colonies with abnormal morphology and karyotype could be grown in different culture conditions. Their erythroid nature was ascertained by the presence of carbonic anhydrase I and glycophorin A. Leukemic erythroid progenitors strongly differed from normal progenitors in that spontaneous colonies were always obtained, sometimes with an extremely high plating efficiency (up to 5.7%). Colonies were found to be autonomous from exogenous hematopoietic growth factors because they were still obtained with a high plating efficiency at an average of one cell per culture in the absence of any added growth factor. No evidence for an autocrine secretion of Epo or GM-CSF emerged because Epo or GM-CSF could not be detected by biologic or radioimmunologic assays from the culture supernatant or cellular extracts of the leukemic cells and that Epo or GM-CSF antibodies did not block autonomous growth. In all cases, however, hematopoietic growth factors increased the plating efficiency of the abnormal erythroid progenitors. In the two "de novo" leukemias, leukemic erythroid progenitors responded primarily to Epo, whereas in the three other patients' (chronic myeloid leukemia) blast crisis they responded maximally to GM-CSF plus Epo. Recombinant erythroid-potentiating activity had no effect in any of these cases. These results suggest that the leukemic erythroid clonogenic cells arise from expansion of erythroid progenitors at different levels of differentiation (ie, CFU-E or BFU-E, depending upon the disease) and that autonomous growth is not related to a secretion of Epo or GM-CSF.

Cell Cycle↗
PubMed1987

[Sinus histiocytosis with extensive adenopathy in a Mali child].

Destombes, Rosai and Dorfman's sinus histiocytosis (HS) is a rare disease. Many cases have been reported in Africans, particularly in children. Case reported here is the second malian clinical report. Its particularities are difficulties for the histological diagnosis with confusion with Hodgkin's disease, a temporary decrease of adenopathies after vincristine therapy, an eosinophilic infiltrate of lymph nodes and a long evolution during at least 4 years. The pathological physiology of HS remains uncertain. The benign natural course of the disease does not justify employment of chemotherapy.

Diagnosis, Differential↗
PubMed1987

Expression of blood group A antigen during erythroid differentiation in A1 and A2 subjects.

The expression of blood group A antigen on marrow and blood cells from A1 and A2 subjects was investigated by the binding of Helix pomatia and Dolichos biflorus lectins using immunofluorescence. These two lectins stained BFU-E-derived colonies from A subjects in the early days of culture before the expression of glycophorin. The erythroid origin of these cells was ascertained by the coexpression of two other very early erythroid markers. In bone marrow, the ultrastructural immunogold method revealed that the entire erythroid lineage including proerythroblasts was labeled by HPA, whereas no staining was observed on granulomonocytic cells including myeloblasts. Platelets from A subjects were HPA-labeled and so were platelets from an O subject preincubated in A plasma. Megakaryocytes obtained in CFU-MK-derived colonies were weakly and heterogeneously labeled by the HPA lectin. Cultures from A1 and A2 subjects were the reflection of the genetic differences only when investigations were performed on mature erythroblasts. In contrast, the great majority of immature erythroblasts both from A2 and A1 subjects were equally labeled by both lectins; during further erythroid maturation, binding of both lectins markedly diminished only on A2 erythroblasts. When marrow erythroblasts were investigated at electron microscopic level, heterogeneity of labeling among all stages of maturation was clearly observed in A2 subjects, with staining stronger on immature than on mature erythroblasts. Therefore, the genetic differences between A1 and A2 subjects are revealed during terminal erythroid differentiation.

ABO Blood-Group System↗
PubMed1986

[Hashimoto's thyroiditis with intra-thyroid production of a monoclonal antithyroglobulin autoantibody].

A 46-year old man had an Hashimoto's thyroiditis. The disease was particular by the voluminous goiter, associated with pressure symptoms and by the presence of a monoclonal Ig.G Kappa. Because of a possibly associated lymphoma of the thyroid, a total thyroidectomy was performed. Post-operative course showed a rapid and total disappearance of monoclonal Ig.G and antithyroglobulin autoantibodies. There was no pathological feature of thyroid malignancy. We could show that the monoclonal Ig.G bore an antithyroglobulin activity and that this monoclonal antithyroglobulin autoantibody was produced with in the thyroid. This case demonstrate that a monoclonal proliferation can arise from autoreactive B lymphocytes infiltrating the thyroid during Hashimoto's thyroiditis. Such a mechanism could explain, in some cases, the well-known association between lymphomas of the thyroid and Hashimoto's thyroiditis.

Antibodies, Monoclonal↗
PubMed1986

[Lymphomas and AIDS].

We report 21 cases of lymphomas associated with AIDS. Sixteen cases are Non Hodgkin's lymphomas of high grade malignancy. Immunoblastic B cell lymphomas are frequent: 11 cases/16, especially with extra nodal localisations. Three cases are Burkitt's lymphoma; 2 cases are large non cleaved cell lymphoma. Five cases of Hodgkin's disease are associated with AIDS related complex syndrome (ARC) showing the interest of lymph node biopsy in such patients. We analyzed 6 cases with lymphoid bone marrow infiltration and discuss the relationship between prelymphomatous states and low grade malignant lymphomas in AIDS or ARC patients.

Acquired Immunodeficiency Syndrome↗
PubMed1985

Immunoblastic lymphoma involving the bone marrow in a patient with alpha chain disease. Clinical and immunoelectron microscopic study.

A patient is reported who had disseminated immunoblastic proliferation that emerged during the course of alpha chain disease. This proliferation was characterized by overt marrow invasion together with osseous and neurologic manifestations. On immunoelectron microscopic study, the malignant immunoblasts displayed varying degrees of cytoplasmic maturation and constituted a morphologic spectrum of alpha-chain-synthesizing cells, ranging from immature blasts without endoplasmic reticulum development to relatively mature plasmablasts; alpha chain was not expressed at the surface of these cells. The general features of the overt malignant stage of alpha chain disease are reviewed in reference to this unusual case. The implications of the cellular findings are discussed with regard to the maturation stage of malignant immunoblasts.

Adult↗
PubMed1984

Two new cases of heterozygosity for hemoglobin Knossos alpha 2 beta 2 27 Ala----Ser detected in the French West Indies and Algeria.

Hb Knossos alpha 2 beta 2 27 Ala----Ser was first described in a Greek family as a silent beta(+) thalassemia variant. Reexamination of 5,000 isoelectric focusing patterns of patients with microcytosis allowed the presumptive identification of two additional propositi. The first originated in the French West Indies (Martinique) and the second in Algeria. A branch of the family of the second propositus was also investigated. Identification of Hb Knossos was made easily in the first family since one member was a double heterozygote for Hb S and Hb Knossos. In the second family HPLC elution of the peptide fragments obtained by tryptic digestion of the aminoethylated beta chain allowed the isolation and characterization of an abnormal beta T3 peak with expected beta 27 Ala----Ser substitution. The Hb Knossos heterozygote from Martinique, besides an elevated alpha/beta globin chain ratio, had an elevated Hb A2 concentration in contrast to the Greek and Algerian families in which it was normal. This difference in phenotypes may be explained by the occurrence in the Mediterranean cases of a delta gene abnormality, presumably delta(0) thalassemia, in position cis to the abnormal beta-globin gene.

Adolescent↗
PubMed1984

[Histopathologic aspects of bone marrow in angioimmunoblastic lymphadenopathy].

42 bone marrow biopsies (BMB) were done in 34 patients with angio-immunoblastic lymphadenopathy (AIL), 3 of them associated with Gougerot-Sjögren syndrome. Involvement of bone marrow was discovered in 36 BMB (85%) and in 27 patients (79%). The lesions appeared as nodules destroying the hematopoietic tissue. Each nodule was constituted by a variable amount of lymphocytes, plasma cells, immunoblasts and histiocytes. Epithelioid cells were associated in 14 BMB and in 11 patients. Collagenous myelofibrosis was seen in and around nodules. The bone marrow lesions were uninodular in 5 BMB (11.9%), multinodular in 17 patients and 20 BMB (47.6%) or massive in 10 patients and 11 BMB (26.1%). Vascular lesions were disclosed in only 17 BMB with multinodular or massive involvement. The hematopoietic bone marrow outside the nodular lesions showed hyperplasia in 12 BMB and was normal in all other cases. In the 7 BMB without AIL localization the hematopoiesis was 6 times normal and hyperplasic in one case. No case of malignant lymphoma was disclosed. AIL bone marrow involvement should not be confused with non Hodgkin's ML or Hodgkin's disease. Correlation with median survival and bone marrow involvement was shown: 12 months in cases with massive involvement, 16 months in uni or multinodular lesions, 56 months in bone marrow free of disease. So BMB is useful for the diagnosis of AIL and the presence or absence of bone marrow involvement is of prognostic value.

Bone Marrow↗
PubMed1984

[Sideroblastic anemia, lead poisoning and wine from Bordeaux].

The detection of progressive sideroblastic anaemia over a period of weeks in a 58 year old woman led to the diagnosis of chronic lead poisoning confirmed by serum lead levels of 2 mg/l. The alimentary origin of the lead poisoning was proved by the finding of high lead levels in the wine which the patient drank excessively. An inquiry at the wine merchant's showed that the local wine was not responsible but that the foreign wine used to fortify the blend had been contaminated. Chronic lead poisoning in alcoholics which has already been widely reported and the demonstration of abnormally high lead levels in wine, rise the problem of regular statutory surveillance of lead levels in alcoholic beverages.

Alcoholism↗
PubMed1983

Platelet peroxidase deficiency in a case of myelodysplastic syndrome with myelofibrosis.

Morphological and functional abnormalities of the megakaryocytic series have been well described in myelodysplastic syndromes. Platelet peroxidase has always been demonstrated in abnormal megakaryocytes and early megakaryoblasts in such syndromes. We have studied a case of myelodysplastic syndrome with marked morphological abnormalities of megakaryocytes in which ultrastructural studies showed the coexistence of platelet peroxidase positive and platelet peroxidase negative megakaryocytes. This enzymatic deficiency was confirmed by the ultrastructural study of circulating platelets. This case appears to be the first report of a partial platelet peroxidase deficiency. It adds to the enzymatic abnormalities in myelodysplastic syndrome already described for the red cells and the granulocytic cells.

Aged↗
PubMed1983

[Thrombotic thrombopenic purpura associated with Hodgkin's disease].

We report the second case of Hodgkin's disease associated with thrombotic thrombocytopenic purpura (TTP). Although the two diseases coexisted originally, TTP was diagnosed alone at first. Because of the very rare occurrence of this association the diagnosis of TTP is discussed according to the accepted criteria and taking into account the haematological complications which can reveal or be observed in Hodgkin's disease. No evident relationship between the two diseases can be suggested. But immunologic disorders observed in the two cases suggests that their coexistence is not necessarily accidental. The evolution of these two diseases is particularly good.

Adult↗
PubMed1982

[Early evaluation of therapeutic response in acute lymphoblastic leukaemia. A study of cytoreduction in blood and bone marrow (author's transl)].

Cytoreduction was evaluated during the first week of induction treatment in 29 adult patients with acute lymphoblastic leukaemia. In 25 patients a slope of decrement of circulating blasts between the 1st and 7th days of treatment could be drawn on a semilogarithmic scale. The fall of the medullary blast index was calculated in bone marrow obtained by aspiration in 17 cases and by aspiration plus biopsy in 11 cases. The diminution of circulating and medullary blasts was more pronounced in patients who achieved complete remission than in those who failed to respond (p less than 0.001), irrespective of the induction treatment. Early therapeutic cytoreduction, as measured by these simple mathematical methods, therefore has a bearing on the prognosis, and the induction treatment should be reinforced in cases where cytoreduction is insufficient.

Adult↗
PubMed1982

Reticulocytosis, hypochromia, and microcytosis: an unusual presentation of the preleukemic syndrome.

Two patients exhibiting a highly unusual preleukemic syndrome with marked reticulocytosis, hypochromia, and microcytosis are reported. This red cell phenotype has been investigated by means of HbF, HbA2, and i-antigen activity dosages, immunofluorescence labeling of F cells, reticulocyte survival, globin chain synthesis, and electron microscopy study. The marked reticulocytosis is explained by a delayed disappearance of the reticulum. Serum iron is normal, and a thalassemic syndrome is excluded because of a balanced alpha/non-alpha globin chain synthesis. Electron microscopy studies are consistent with a defect in iron uptake by erythroid cells. All the hematologic data and investigations are similar to those observed for the Belgrade laboratory rat. It is hypothesized that the low expression of HbF and i-Ag associated with microcytosis are related to a prolongation of erythroid maturation as reflected by abnormal reticulocyte survival.

ABO Blood-Group System↗