[IgAk solitary bone plasmacytoma leading to the loss of heavy chain alpha].
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Biomedical subjects
Publications and source records attributed to J A Gastaut.
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We report the case of a patient having Philadelphia-negative, bcr-abl-positive chronic myeloid leukemia. In situ hybridization showed the presence of the bcr-abl fusion on the chromosome 9 long arm in all mitoses observed. Stability of the disease was very difficult to obtain because of serious adverse effects to interferon and chemotherapy, mainly grade IV neutropenia, and a blast crisis occurred 12 months after diagnosis. Only three other patients with such presentation (Philadelphia negative, bcr-abl positive with bcr-abl fusion on the chromosome 9 long arm) have been reported, with a poor therapeutic response and outcome in two of them. Translocation of BCR to chromosome 9 may therefore have a worse prognosis than translocation of ABL to chromosome 22 in Philadelphia-negative chronic myeloid leukemia.
The object of this study supported by the French Agency for AIDs Research (ANRS) was to assess knowledge and attitudes of pregnant women towards HIV infection and testing, and to compare them according to the outcome of the pregnancy (elective abortion vs delivery). Between March 22 and April 26, 1992, all women ending their pregnancy and attending one of the 72 medical centres located in South-Eastern France were asked to complete an anonymous questionnaire (n = 4303). 3,854 (89.6%) responded: 2,825 women at delivery (WD) and 764 who chose an elective abortion (WA). 61.7% of WD and 24.1% of WA declared having been tested for HIV during pregnancy (p < 0.001). Among women who reported not having been HIV tested, very few did so because they refused the test (1.7% among WD and 1.4% among WA-NS). Prior HIV testing was less frequent among WA than among WD (45.8% vs 58.8%--p < 0.001). 2.8% of women tested during prenatal care and 20% in the context of abortion did not know the result of their test (p < 0.001). Knowledge about HIV transmission declared by WD did not differ significantly from that declared by WA. However, risky behaviours were more frequent among WA than among WD (38.9% vs 17.7%--p < 0.001). This research shows that French screening HIV policy in the context of pregnancy remains mainly motivated by foetal concerns. Although women who abort voluntarily report risky behaviours more frequently, the opportunity of information and counselling towards them is relatively neglected in comparison with women who deliver.
The Philadelphia chromosome (Ph) is the cytogenetic hallmark of chronic myeloid leukaemia (CML) and is used to confirm the diagnosis of CML based on clinical and morphological criteria. We investigated 14 patients with features of CML but without detectable Ph chromosome. In seven patients, referred to as BCR+, M-bcr/abl rearrangement was detected by polymerase chain reaction (PCR). The seven remaining patients did not have M-bcr/abl rearrangement and are described as BCR-. BCR- patients were younger, had lower white blood cell counts (WBC) and lower basophilia. Four BCR- and four BCR+ patients underwent blastic transformation (BT). Response to therapy was fairly similar in both populations. According to French-American-British (FAB) Cooperative Leukaemia Group guidelines, all BCR- patients were classified as having classic form CML or 'chronic granulocytic leukaemia' (CGL) when based only on morphological data. This study further confirms the existence of true CML cases without Ph chromosome or M-bcr/abl rearrangement and shows that this entity differs only slightly from classic form Ph+ CML. The Ph-BCR- subgroup raises two problems. First, the differential diagnosis with atypical CML or CMML, based on morphological data, and secondly, the therapeutic follow-up in the absence of a specific marker. In contrast, the residual disease of Ph-BCR- patients can be monitored by PCR. More advanced molecular and biochemical techniques will be required to understand which molecular mechanisms underlie Ph-BCR- CML, resulting in phenotypes sometimes indistinguishable from Ph+ CML.
m-BCR chronic myeloid leukaemia (CML) is a rare entity. We report a patient presenting with Philadelphia (Ph)-positive, m-BCR-positive acute lymphoblastic leukaemia (ALL) who achieved complete remission after induction chemotherapy, but showed a majority of Ph-positive mitoses during this remission. A diagnosis of m-BCR CML was established and the patient was given interferon alpha therapy. This is the first m-BCR CML presenting as ab initio ALL. This report emphasizes the importance of karyotyping Ph-positive ALL during remission so as not to misdiagnose CML patients who can benefit from Interferon therapy.
Clinical and pathological characteristics as well as outcome of 45 Hodgkin's disease (HD) cases collected by the French registry of HIV-associated tumors between January 1987 and December 1989 (all clinically staged according to the Ann Arbor system) were analyzed and compared with those of a cohort of 407 patients with clinical stages (CS) IA to IVB enrolled between September 1981 and August 1988 in a multicentric clinical trial. The route of HIV infection, initial CD4 cell count at the time of HD diagnosis and CDC class of HIV infection were studied as well as the progression to AIDS onset were recorded. HIV-HD is characterized by a predominance of advanced CS (75%), B symptoms (80%) and mixed cellularity histology (49%), as well as by early bone marrow involvement (24%); a specific feature is the rare occurrence of mediastinal involvement (13% in HIV-HD versus 71% in primary HD). With standard therapies, 79% of the patients achieved a complete remission, but hematological and infectious complications were very frequent. The proportion of intravenous drug abusers (IVDA) in HIV-HD (38%) is higher than in French HIV-infected population as a whole (20.8%). Median CD4 cell count was 306/microliters at the time of HD diagnosis, while only 5 cases (11%) were preceded by an AIDS manifestation; progression to AIDS rate was 94% at 2 years. Overall 2-year survival was 41%, with 71% for patients with an initial CD4 cell count over 300/microliters and 0% for those with CD4 cell count lower than 300/microliters (p < 0.01); opportunistic infections were the most frequent cause of death. HIV-HD seems to occur preferentially in.(ABSTRACT TRUNCATED AT 250 WORDS)
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OBJECTIVE: To assess whether postprandial dietary thermogenesis contributes to weight loss during HIV infection. METHODS: The thermogenic response to a test meal (15 kcal/kg) was evaluated with indirect calorimetry in 16 HIV-infected patients in a stable condition and compared with a control group. Patients were compared according to AIDS (n = 8) or non-AIDS (n = 8) status and to body weight loss (WL; n = 9) or no loss (NL; n = 7). Indirect calorimetry was performed after fasting 6 h and during 5 h after the test meal. RESULTS: Maximum value of energy expenditure was reached later in the WL group than in the control and NL group (200 versus 30 min, respectively). Energy expenditure returned to the initial value 300 min after the test meal (last measurement) in the control group but remained elevated in the patient group. Energy expenditure after food intake was more elevated in HIV-infected patients than in controls, especially in patients with detectable clinical change in their nutritional status (0.96 versus 0.72 kcal/kg body weight). CONCLUSION: Both kinetics and quantitative aspect of dietary thermogenesis are modified during HIV infection and the different variations are dependent on the extent of body weight loss.
OBJECTIVES: To estimate HIV seroprevalence in the two main remand and short-stay prisons of south-eastern France and to gather linked anonymous risk-factor information. SETTING: Baumettes prison, Marseille, France between 16 November and 21 December 1992. PARTICIPANTS: Using a self-administered questionnaire about HIV testing and risk factors for HIV infection, 295 male and 137 female inmates were interviewed. The response rate was 96% (100 and 90% for men and women, respectively). At the same time, 279 of a total of 432 (65%) inmates were serologically tested for HIV; 153 (35%) declined to provide a blood sample. DESIGN: Anonymous cross-sectional and surveillance survey. RESULTS: Twenty per cent of participants (84 our of 432) were intravenous drug (heroin) users (IVDU), 51% of whom reported needle-sharing prior to incarceration; 23% reported more than two sexual partners during the last year, and 13% sexual intercourse with an IVDU during the last 5 years. HIV status was available for 356 inmates (82%; 65% from blood samples and 17% from the questionnaire); 39 were HIV-infected (10.9%; 95% confidence interval, 7.7-14.2). The inmates not tested for HIV reported proportionally less risky behaviours than non-HIV-infected inmates. HIV seroprevalence was significantly higher among recidivist inmates (19.9 versus 4.4%; P < 0.0001). The rate of HIV infection was particularly high among IVDU (34 out of 84; 40%). More female non-IVDU were HIV-infected than male non-IVDU (4.1 versus 0.6%; P = 0.04). CONCLUSIONS: This study demonstrates the high prevalence of HIV infection in south-eastern French prisons, especially among IVDU. The rates may be related to the high prevalence of risky drug practices and to delays in the development of HIV prevention programmes for IVDU in France. The higher seroprevalence rate among recidivist inmates might be the result of risk behaviours during imprisonment. Another hypothesis is that recidivist inmates are at greater risk of HIV infection because of higher levels of drug use.
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We have examined 9 healthy volunteers and 63 HIV-patients (16 asymptomatic patients and 47 patients with clinical AIDS-dementia complex, ADC) by magnetic resonance spectroscopy (MRS) and imaging (MRI) on a Siemens Magnetom SP63 (1.5 T). Proton MRS of the brain was performed at 63 MHz using the PRESS sequence (echo time = 135 ms, TR = 1.6 s). Four main results have been found: (1) HIV-related encephalopathy induces significant modifications of brain metabolism analyzed by MRS and the most sensitive metabolic parameter is the N-acetyl-aspartate/Choline ratio, (2) the correlation between MRS and MRI is good in 75% of patients, (3) in 4 of the 16 neuro-asymptomatic patients (i.e. 25%) a metabolic encephalopathy was found while MRI was still normal, and (4) MR spectra describe 3 different pathological metabolic patterns in the brain of HIV patients. Two patterns might correspond to the two entities of HIV-induced lesions i.e. HIV encephalitis and HIV-related progressive leukoencephalopathy.
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