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Biomedical subjects

M Tulliez

Publications and source records attributed to M Tulliez.

At least 55 records · Page 3Linked to original sources

[Acute mast cell leukemia disclosed by vasomotor flushing].

INTRODUCTION: Acute mast cell leukemia is a rare and severe disease. We report herein a case associated with a flush syndrome. CASE REPORT: A 44-year-old man, presented with a flush of face and trunk. Bone marrow was infiltrated with immature mast cells. In spite of chemotherapy and bone marrow transplantation the patient deceased. DISCUSSION: Pheochromocytoma, carcinoid tumor, and mastocytosis are associated with a flush syndrome. In our patient the diagnosis was an acute mast cell leukemia. Acute mast cell leukemia can follow systemic mastocytosis or occur de novo. This disease is of poor prognosis. No treatment is available.

Adult↗

Loss of primitive hematopoietic progenitors in patients with human immunodeficiency virus infection.

A number of hematologic abnormalities, including cytopenias, have been observed in patients with human immunodeficiency virus (HIV) infection. To elucidate their mechanisms, primitive cells from bone marrow aspirates of 21 patients with HIV-1 infection were quantitated by flow cytometry. The mean percentage of CD34+ cells is not significantly altered in HIV-1-infected patients in comparison with HIV-1-seronegative controls. In contrast, two- and three-color immunofluorescence analysis showed that in all HIV-1 samples, most CD34+ cells coexpressed the CD38 antigen. The proportion of HIV-1-derived CD34+ cells that did not express the CD38 antigen was significantly lower (HIV-1+: mean, 1.73%; controls: mean, 14%; P < .0005) than in controls. Moreover, of Thy-1+ cells, the proportion of CD34+ cells was twofold lower in HIV-1-infected patients (HIV-1+: mean, 12%; controls, 25%, P < .0005), which suggests that phenotypically primitive cells are depleted in HIV-1 infection. In vitro functional analysis in long-term cultures of sorted CD34+ cells from seven HIV-1 patients showed that CD34+ cells from HIV-1 patients generated much fewer colonies both in the nonadherent and adherent layers than CD34+ cells from controls after 5 weeks of culture (10-fold and four-fold less, respectively). Precise long-term culture initiating cell (LTC-IC) frequency in the CD34+ cell population was determined in three patients by limiting dilution and was markedly decreased in comparison to that of normal controls (from twofold to > sevenfold decreased). To determine if primitive cells were infected by HIV-1, both methylcellulose colonies generated from long-term culture of CD34+ cells and various CD34+ cell fractions purified by flow cytometry were evaluated for the presence of HIV-1 by polymerase chain reaction (PCR). Progeny from long-term culture was HIV-1-negative in three samples. In addition, using a sensitive PCR technique, the HIV-1 genome could not be detected in CD34+, CD34+/CD38-, and CD34+/CD4+ cells. These data show that hematologic disorders in HIV disease may be the consequence of a deficit of primitive cells. However, direct infection of these cells by HIV-1 does not seem to be responsible for this defect.

ADP-ribosyl Cyclase↗

[Cytology of acute leukemia].

Cytological analysis of peripheral blood and bone marrow films is a quick and simple method for diagnosis of acute leukemia. In association with cytochemistry techniques it allows the rapid identification and subsequent classification of most types of acute myeloid leukemias. Although morphological analysis can suggest the diagnosis of an acute lymphoblastic leukemia, it is necessary to have the corroborating evidence of immunological markers for confirmation. In addition, ultrastructural studies contribute to the identification of rare forms of acute myeloid leukemias (minimally differentiated, megakaryoblastic). Cytological studies have also proven useful for the follow-up activities of confirming complete remission and detecting relapses.

Acute Disease↗

Splenic lymphoma with villous lymphocytes: clinical presentation, biology and prognostic factors in a series of 100 patients. Groupe Francais d'Hématologie Cellulaire (GFHC).

The diagnosis of splenic lymphoma with villous lymphocytes (SLVL) was assessed by a panel of cytologists in a series of 100 patients. Clinical and biological characteristics were analysed in relation to prognosis. SLVL is a chronic B-cell lymphoproliferative disorder characterized by splenomegaly and the presence, in peripheral blood, of lymphocytes with "villous' projections. The cytological diagnosis can be difficult in patients without an absolute lymphocytosis which was observed in 24% of cases. B-cells expressed CD19+, CD20+, CD22+, CD24+ and DBA44+, whereas the expression of CD5, CD10 and CD25 was usually negative. SLVL is a disease of the elderly with a relatively benign clinical course. In the present series the 5-year overall survival was 78%. Deaths in 15 patients were related to disease progression or treatment (nine cases). Patients with a leucocyte count > 30 x 10(9)/1 or lymphocyte count < 4 x 10(9)/1 or initially treated with chemotherapy had significantly (P < 0.001) lower overall survival than other patients. From these findings, treatment abstention should be considered in patients with favourable prognostic factors; on the other hand, the efficacy of conventional chemotherapy remains to be evaluated in patients with unfavourable prognostic factors.

Adult↗

[Peritoneal mesothelioma revealed by episodes of recurrent spontaneous peritonitis].

A case of a malignant peritoneal mesothelioma revealed by recurrent episodes of peritonitis is reported. This presentation had never been described. A 54 year-old man presented successive episodes of appendicitis, cholecystitis and sigmoiditis associated with purulent aseptic peritonitis. There were no signs of immunodepression or vasculitis. Initial analysis of peritoneal biopsies showed non specific inflammation. Because of long-term asbestos exposure, these biopsies were reviewed. A diagnosis of peritoneal mesothelioma was made on subserous infiltration by mesothelioma cells, and confirmed by immunohistochemistry. The patient died ten months after the diagnosis of mesothelioma despite chemotherapy (cisplatine+interferon).

Antineoplastic Combined Chemotherapy Protocols↗

Polymyositis associated with Ki-1 lymphoma.

We report here a case of acute polymyositis associated with a Ki-1 non-Hodgkin's lymphoma (NHL). This anaplastic large cell malignant lymphoma was a primary T-cell NHL lymphoma of the bone marrow. The malignant cells expressed the CD30 (Ki-1), CD3, and CD4 antigens, the beta chain of the interleukin 2 receptor (CD25), and the betaF1 antigen (alpha/beta T-cell receptor). Chemotherapy and high dose methylprednisolone pulse therapy were initiated. However, no clinical improvement was noticed, because the patient rapidly died of an acute respiratory distress syndrome. To our best knowledge, this represents the first case of Ki-1 lymphoma associated with Polymyositis.

Acute Disease↗

Expression of the L-type pyruvate kinase gene and the hepatocyte nuclear factor 4 transcription factor in exocrine and endocrine pancreas.

The L-pyruvate kinase (L-PK) gene is slightly active in normal and tumoral endocrine pancreatic tissues while, in vivo, this gene is not transcribed in the exocrine pancreas. Nevertheless, the L-PK gene is re-expressed at a very low level in cultured 266.6 cells derived from an exocrine pancreas carcinoma. The L-PK gene is early activated in endodermal tissues, e.g. yolk sac and primitive intestine; it remains transcribed in fetal pancreas. In adult, L-PK gene expression is restricted to some endocrine cells. Hepatocyte nuclear factor (HNF) 1 and HNF4 are the main tissue-restricted transcription factors involved in tissue-specific expression of the L-PK gene. HNF1 concentration is similar in liver and all pancreatic cells. HNF4 concentration is high in liver, much lower in islets of Langerhans, endocrine pancreatic tumors, and cultured insulinoma cells, and is scarcely detectable in adult exocrine pancreas. This distribution of HNF4 parallels the expression of the L-PK gene. In vivo footprinting experiments show that the HNF1 binding site is similarly occupied in both adult liver and adult pancreas, in which this gene is practically inactive. In this latter tissue, however, the HNF4 binding site is differently occupied with respect to the liver. Since the chromatin structure remains open around the L-PK promoter in pancreas, the L-PK gene can probably be re-expressed under certain circumstances, for instance in cancerous pancreatic cells.

Animals↗

Alpha-chain disease: analysis of alpha-chain protein and secretory component in jejunal fluid.

BACKGROUND: It is unclear why different forms of alpha-chain disease protein appear in intestinal fluid. This was studied in a 23-year-old Mauritanian man in whom alpha-chain disease was diagnosed localized to the duodenum and jejunum, nasopharynx, and bone marrow. METHODS: The duodenal infiltrate was studied by immunohistochemistry. Forms of alpha chain-containing proteins in serum and jejunal fluid were analyzed by ultracentrifugation and radioimmunoassays. RESULTS: The infiltrating cells contained alpha-1 chain but no light chains, and approximately 66% showed variable expression of J chain. Serum contained a large fraction of monomeric alpha-chain disease protein, whereas both monomeric and heavier forms appeared in jejunal fluid. Some of the latter were bound to secretory component, and the fluid contained virtually no free component. CONCLUSIONS: Linkage of polymeric alpha-chain disease protein to secretory component depends on balanced synthesis of alpha chains and J chain in the proliferating B cells, giving rise to polymers with binding site for secretory component expressed as an epithelial receptor. Insufficient receptor-mediated transport capacity (either relative and/or because of intestinal crypt reduction) results in passive external transfer of polymers without bound secretory component along with leakage of serum-derived or locally produced monomeric alpha-chain disease protein, the latter presumably originating from immunocytes with little or no J-chain synthesis.

Adult↗

Role of human immunodeficiency virus replication in defective in vitro growth of hematopoietic progenitors.

A number of hematologic abnormalities, including cytopenias, have been observed in patients with human immunodeficiency virus (HIV) infection. To elucidate their mechanisms, a group of 27 patients with HIV-1 infection was studied. In all patients, a marked reduction of in vitro colony formation by erythroid, granulomacrophagic, and megakaryocytic bone marrow progenitors was observed in comparison to normal donors. HIV-1 infection of marrow progenitors was investigated in studying individual colonies with the polymerase chain reaction (PCR) technique. No HIV-1 DNA could be detected in these colonies, suggesting either that marrow progenitors were not infected or that infected progenitors were not able to generate colonies in vitro. The addition of antisense oligonucleotides directed against HIV tat or nef sequences in the culture medium led to a significant increase in colony formation, suggesting that HIV replication in hematopoietic progenitors could be responsible for their defective growth. However, no HIV-1-infected colonies could be detected by PCR after the antisense treatment, indicating that the increase in colony number was not due to the proliferation and differentiation of infected progenitors but to an inhibition of HIV replication in an accessory cell. This last hypothesis was further confirmed by the absence of effects of antisense oligomers on the plating efficiency of hematopoietic progenitors grown from CD34+ cells. These data indicate that hematologic abnormalities of HIV-infected patients cannot be explained by a direct infection of hematopoietic progenitor cells and suggest that a defective modulation of progenitor cell growth by HIV replication outside these cells might play a role in these abnormalities.

Acquired Immunodeficiency Syndrome↗

Pulmonary Hodgkin's disease in HIV-infected patient. Diagnosis by bronchoalveolar lavage.

We report herein the pulmonary recurrence of Hodgkin's disease coincidental with a marked decrease in the peripheral blood CD4 lymphocyte count in an HIV-seropositive patient with alveolar consolidation on chest roentgenogram. The diagnosis of lung parenchyma involvement was made by bronchoalveolar lavage cell analysis and illustrates the reliability of Reed-Sternberg cell identification in bronchoalveolar lavage for the diagnosis of pulmonary localization of Hodgkin's disease.

Adult↗