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J J Viala

Publications and source records attributed to J J Viala.

At least 19 recordsLinked to original sources
PubMed1994

Cutaneous findings in hairy cell leukemia. Review of 84 cases.

The authors of this paper present a retrospective analysis of 84 cases of hairy cell leukaemia diagnosed over the past ten years in two haematology departments. Cutaneous manifestations are noted on 47 occasions, 58% of infectious origin with 60% of these infections due to bacterial agents, results which are in relatively close accord with those of the Mayo Clinic. Since no cases of necrotic angeitis or neutrophilic dermatosis are reported, the considerable number of publications concerning these affections give a poor indication of their rarity. Prospective studies will be required to evaluate the incidence of spinocellular carcinoma and melanoma, while specific lesions are only infrequently encountered and do not appear to bear the same unfavourable prognosis as in other haemopathia.

Humans↗
PubMed1993

Results of splenectomy for idiopathic thrombocytopenic purpura. Review of 72 cases.

Seventy-two patients underwent splenectomy for idiopathic thrombocytopenic purpura between 1979 and 1990. Mean age at splenectomy was 36.4 years (range 11-73). Indications for splenectomy were corticodependence in 21 cases and resistance to steroids in 44 cases. Thirty-five patients had platelet kinetic studies by 51Cr alloplatelets; 22 of them had splenic sequestration. Hematologic results were evaluated on discharge, at 3 months and in the long term (median follow-up 5.4 years). We had no mortality, morbidity was seen in 7% of the cases. None of the patients suffered from secondary infectious complications. 89% had good results on discharge (> 120 x 10(9)/l), 72.6% at 3 months and 90% on long-term follow-up. Factors associated with good response to splenectomy included a high postoperative platelet count (more than 120 x 10(9)/l on discharge), younger age at the time of surgery, preoperative corticodependence and predominantly splenic sequestration.

Adolescent↗
PubMed1993

Plasma erythropoietin in essential thrombocythaemia.

Plasma erythropoietin levels were frequently decreased in patients with essential thrombocythaemia (14/31 cases), as in polycythaemia vera. These two diseases or some forms of them might be two facets of a single disease process.

Erythropoietin↗
PubMed1990

Determination of plasma cell labelling index with bromodeoxyuridine using a double immunoenzymatic technique.

Myeloma plasma cells were double stained using peroxidase and alkaline phosphatase labelled monoclonal anti-BrdU and anti-intracytoplasmic immunoglobulins. Samples were methanol fixed; DNA was denatured with formamide. The results allowed easy identification of plasma cells, their cytological examination and the calculation of percentage of plasma cells in S phase. Good correlation was found with the labelling index obtained with tritiated thymidine.

Bromodeoxyuridine↗
PubMed1989

Aggregation to thrombin and collagen of platelets from a Glanzmann thrombasthenic patient lacking glycoproteins IIb and IIIa.

The aim of this study was to investigate the platelets of a Glanzmann thrombasthenic patient, which in citrated PRP failed to respond to various agonists, but aggregated and secreted to high concentrations of thrombin (0.36, 0.72 and 1 U/ml) and collagen (4, 10 and 20 micrograms/ml) when washed and resuspended in a Tyrode-albumin solution (containing 2 mM Ca2+). Aggregation of the patient platelets was not affected by anti-IIb/IIIa monoclonal antibody (P18) which strongly inhibits thrombin or collagen induced aggregation of normal platelets. Washed platelets of this patient did not aggregate to ADP (10-100 microM) in the presence of added fibrinogen (2 mg/ml) nor bind 125I-labelled fibrinogen (40 to 320 micrograms/ml) when thrombin-stimulated. Different anti-IIb/IIIa monoclonal antibodies (P2, P18) when used in binding or crossed immunoelectrophoretic studies showed a complete absence of the IIb-IIIa glycoprotein complex on the patient platelets. Moreover, glycoproteins IIb or IIIa were absent on silver-stained two-dimensional (non-reduced/reduced) polyacrylamide gel separations of the patient platelets and were not detected by Western blots used in combination with anti-PLA1 (antigen present on IIIa), anti-Leka (antigen present on IIb). This study shows that platelets lacking glycoproteins IIb or IIIa can aggregate in response to high concentrations of collagen or thrombin when resuspended in the presence of physiological concentrations of calcium. Results obtained in this study could indicate the existence of other mechanisms (other than the IIb-IIIa glycoprotein complex) involving glycolipids, heparans, proteoglycans, and/or unknown membrane glycoproteins to mediate platelet aggregation of stimulated thrombasthenic platelets.

Adult↗
PubMed1989

Influence of cigarette smoking on the presentation and course of chronic myelogenous leukemia.

It is known that cigarette smoking induces leukocytosis and increased genetic instability in normal individuals. Therefore, a retrospective review was conducted of 173 patients with chronic myelogenous leukemia to detect a possible influence of cigarette smoking on initial characteristics at the time of presentation and on the course of this disease. Thirty-nine patients (23%) were smoking 5 cigarettes/d or more at time of diagnosis. Cigarette smoking was significantly related to male sex (P = 0.0005) and younger age at diagnosis (P = 0.02) and smokers tended to have lower leukocyte counts (P = 0.07) than nonsmokers. Cigarette smoking was significantly associated with early blast crisis (P less than 0.0001) and short survival (P less than 0.0001). Other characteristics associated with a poor prognosis included hepatomegaly, anemia, and a high percentage of peripheral blast cells at time of diagnosis. When studied in a multivariate analysis, cigarette smoking remained the strongest prognostic factor for both occurrence of blast crisis (P = 0.0003) and overall survival (P = 0.0001). Other poor prognosis factors found in the multivariate analysis included a high percentage of blasts in the peripheral blood at time of diagnosis and high platelet count. It is possible that cigarette smoke may act as a promoter or cocarcinogen in the transformation of chronic myelogenous leukemia.

Blast Crisis↗
PubMed1989

Phase II trial of plicamycin and hydroxyurea in acute myelogenous leukemia.

A total of 23 patients with high-risk acute myelogenous leukemia (AML) at diagnosis (2 patients), relapsing AML (14) or resistant AML (6) were treated with 25 micrograms/kg i.v. plicamycin every other day for 3 weeks and 500-4,000 mg hydroxyurea per day p. o. according to the WBC count. Aplasia was observed in only two patients. Severe extrahematologic toxicity included sepsis (four cases), vomiting (four patients), toxic hepatitis (three cases), and fibrinopenia (one patient). No partial or complete responses were observed. The 95% confidence interval limit of the overall response rate (CR + PR) was 0-14%.

Bone Marrow↗
PubMed1989

Increased in vivo biosynthesis of prostacyclin and thromboxane A2 in chronic idiopathic thrombocytopenic purpura.

The production of thromboxane A2 (TxA2) and prostacyclin (PGI2) was studied in patients with chronic idiopathic thrombocytopenic purpura (10 patients) compared to central thrombocytopenia (five patients) and healthy subjects (10 subjects). This production was monitored by the assay of urinary 2,3-dinor-TxB2 and 2,3-dinor-6-keto-PGF1 alpha as respective breakdown products of TxA2 and PGI2 by stable isotope dilution assays employing negative ion-chemical gas-chromatography-mass-spectrometry. Evidence is presented for the existence of an enhanced PGI2 and TxA2 urinary excretion in the group of idiopathic thrombocytopenic purpura (ITP) patients. Moreover, production of serum TxB2 per platelet was decreased in ITP group. These results provide arguments for an in vivo platelet cyclooxygenase hyperactivity during chronic ITP.

6-Ketoprostaglandin F1 alpha↗
PubMed1988

The syndrome of abnormal chromatin clumping in leucocytes: a myelodysplastic disorder with proliferative features?

Five patients presenting extremely exaggerated chromatin clumping in leucocytes, associated with a loss of segmentation, are described. Peripheral cytopenia, a high percentage of circulating immature granulocytes (chiefly myelocytes) with variable leucocytosis, marrow hypercellularity with granulocytic hyperplasia and moderate dysplastic changes in erythroblastic and megakaryocytic lines, constitute, together with the granulocytic nuclear anomaly, the characteristic features of the studied cases. Cytogenetic analysis showed a clonal 12p anomaly in one of the three cases available for study. Survival was poor with a median of 15 months, bleeding and infections being responsible for the majority of deaths. All these findings point to abnormal chromatin clumping in leucocytes as the marker of a true entity with both myelodysplastic and myeloproliferative features. We propose that it take its place among myelodysplastic syndromes (MDS) beside chronic myelomonocytic leukaemia (CMML), with which it shares many similarities in clinical and biological behaviour.

Aged↗
PubMed1987

Prognostic factors in angioimmunoblastic lymphadenopathy.

In order to identify prognostic factors in angioimmunoblastic lymphadenopathy (AIL), 30 directly diagnosed patients were prospectively followed for more than 42 months. Age and sex distribution, clinical and laboratory findings and evolution were not different from previously reported series. Median duration of survival was 24 months. Parameters associated with a longer survival in our series were localized adenopathies (P = 0.01) and the achievement of a remission (P less than 0.0001). Features associated with a shorter survival included drug exposure in relation to the onset of the disease (P = 0.02), rash (P less than 0.0001), lymph node eosinophilia (P = 0.03) and elevated serum lactic dehydrogenase (P = 0.03). Drug exposure and rash were, however, significantly dependent (P = 0.02). In addition, lymphopenia, the presence of circulating immune complexes, and the absence of polyclonal hypergammaglobulinemia may indicate a poor prognosis, although the significance level is not achieved in this short series. None of the parameters tested was significantly related to the lymphomatous transformation of AIL, which occurred in four cases. It is concluded that multicentric prospective studies of AIL are necessary in order to better define this disorder, to find prognostic factors, and to optimize therapy.

Adult↗
PubMed1987

Angioimmunoblastic lymphadenopathy with malignant transformation during long-term remission of ocular melanoma.

A 52-year-old woman presented with angioimmunoblastic lymphadenopathy five years after undergoing enucleation for ocular melanoma. She was first treated by corticotherapy and azathioprine and had two successive remissions over an 18-month period. Lymph node biopsy performed when lymphadenopathy relapsed for the second time showed focal malignant transformation of angioimmunoblastic lymphadenopathy. Intensive polychemotherapy induced a complete remission. Three months after completion of chemotherapy, the patient presented with hepatic metastases from her melanoma and died shortly thereafter. This is believed to be the first documented association between angioimmunoblastic lymphadenopathy and melanoma. Issues regarding the relationship between angioimmunoblastic lymphadenopathy and nonlymphoid malignancies are raised.

Biopsy↗
PubMed1987

Day 4 bone marrow aspirate for prediction of resistance to induction chemotherapy in acute myeloid leukemia.

Bone marrow aspirate was performed on day 4 of a 7-day induction chemotherapy regimen using daunorubicin, cytosine arabinoside and thioguanine in 42 patients with acute myeloid leukemia (AML). The mean percentage of remaining abnormal cells in day 4 bone marrow was significantly higher in patients having resistant disease (RD) than in patients entering complete remission (CR). Except for patients with FAB M3 AML, most patients who had more than 40% abnormal cells in their day 4 bone marrow had RD. In addition, patients entering CR despite having more than 40% abnormal cells remaining in their day 4 bone marrow seemed to have short CR.

Adolescent↗