[Apropos of a protein profile].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to B Grosbois.
Explore the source record for details and available documents.
Two large epidermoid cysts of the spleen have been accidentally discovered in two young men. These cysts are uncommon and represent about 10% of the non parasitic benign cysts of the spleen. From these cases, the authors reviewed the literature. These cysts, which are caused by an abnormal development during the seventh week of the intra-uterine life, are often asymptomatic but the occurrence of a complication may enable the diagnosis. This diagnosis relies on the surgical ablation of the cyst and the histopathologic examination. The surgery should be as conservative as possible.
Explore the source record for details and available documents.
There are no well-defined host markers to determine which patients with a diagnosis of monoclonal gammopathy of undetermined significance (MGUS) will progress to multiple myeloma (MM). In this preliminary study we measured plasmatic soluble Fe gamma receptor type III (sFe gamma RIII or sCD16) in 54 individuals with MGUS. 35 patients with multiple myeloma (MM) and 29 healthy controls. We confirmed, through receiver operating characteristic (ROC) curve analysis, that a low level of sCD16 discriminates MM patients from controls. Indeed, for a sCD16 value of 1.3 micrograms/ml, the sensitivity, as well as the specificity, of this discrimination were both equal to 83%, i.e. 83% of MM patients had a plasmatic sCD16 value < 1.3 micrograms/ml compared with only 17% of controls. Moreover, ROC curve analysis showed that a low sCD16 level also identifies among MGUS patients a subgroup of patients who rapidly progress towards multiple myeloma: in this comparison, for a sCD16 level of 1.3 micrograms/ml. sensitivity and specificity were 70% and 79% respectively. Therefore a low sCD16 level in MGUS indicated a high likelihood of rapid evolution of MM. In contrast to sCD16, soluble IL-6R did not appear to be discriminant in this study.
The multidrug resistance phenomenon can be observed in cases which do not express the P170 protein and these cases are suspected as having activated different resistance phenomena. Four phenomena were studied at the time of diagnosis in a series of 35 lymphoblastic and 25 myeloblastic acute (de novo) leukemias, by an immunocytochemical method. Two energetic drug transport processes were investigated: the classical MDR/P170 and the P110/LRP56 proteins, and two physiological detoxifying activities such as the glutathione transferases (GST alpha, mu, pi) and the metallothioneins (Mts). The results demonstrate that these phenomena are independent but their synergic activity can increase their impact on the outcome. P110/LRP56 positive cases demonstrated 48.8% complete remission (CR) rate compared to 71.4% for negative tests. When P170 and P110 were both positive or negative, the CR rates were 27.3% and 81.8% respectively (p = 0.0120), and survival curves were also different (p = 0.030). The CR rate in AML or ALL is weakly affected by GST pi, alpha or mu but relapses are more frequently observed for Positive-GST pi ALL (p = 0.0658). Patients with both P170 and GST pi positive reactions had a 53.3% CR rate compared to 78.9% for both negative reactions. Survival curves for these two groups were different. The CR rate in AMl was 100% for Mts positive and 43.7% for negative cases (p = 0.050), however the median survival was totally different for these two groups (p = 0.046). CR rates were 26.6% for patients who were P170 positive and Mts negative compared to 100% for P170 negative and Mts positive (p = 0.038) patients. Survival curves were also different (p = 0.0510). We conclude that these four mechanisms induce an independent drug resistance but their synergic action increase their impact on the outcome. The metallothioneins seem to have a major impact on the drug resistance phenomenon and its effect should be investigated with high priority, in the light of these results.
This retrospective study was conducted to evaluate the incidence of primary malignant tumors in patients with chronic lymphocytic leukemia (CLL). Between 1974 and 1994, 313 patients were hospitalized for CLL. Patients lost to follow-up were excluded (n = 65). Among the remaining 248 patients (167 men and 81 women), 22 patients (8.8%) developed primary malignant tumors (17 males, 5 females; age range 48 to 90 years); those who developed Richter's lymphoma (n = 9) or basal cell epithelioma (n = 5) were excluded from the cohort of the cancer patient. There were 18 epithelial tumors (3 lung adenocarcinoma) and 4 parenchymal tumors. We observed only one cancer of the prostate, and no breast cancer. The malignant tumor occurred at the same time as CLL in 8 cases and after a delay of 3 to 11 years in the 14 other cases. There was no particular features in the stage of duration of CLL before diagnosis of cancer. Outcome was particularly unfavorable, 20 deaths, compared with mortality in the other 226 patients (53.5%). These findings, together with the data in the literature, emphasize the importance of careful follow-up in patients with CLL.
Between January 1985 and July 1989 we diagnosed asymptomatic stage I multiple myeloma according to Durie and Salmon [Durie and Salmon: Cancer 36:842, 1975] in 91 patients. All patients were followed without chemotherapy. Disease progression occurred in 41 patients and the median time to progression for all patients was 48 months. In the Cox multivariate regression analysis, hemoglobin levels < 12 g/dl (P < .01), bone marrow plasmacytosis > 25% (P < .01), and M-component size > or = 30 g/l for Ig G or > or = 25 g/l for Ig A (P < .01) were the only significant prognostic factors for progression. The 38 patients without any harmful factor remained free of progression for a median of more than 50 months. The 18 patients with two or three of these characteristics (high-risk group) had the shortest median time to progression of 6 months. Despite different times to progression, the response rate and survival after chemotherapy were similar for all groups of patients. Patients in the high-risk group for progression have to be frequently monitored for disease progression and might benefit from early treatment.
Forty-seven patients who underwent splenectomy for splenomegaly > or = 1000 g were studied retrospectively. There were 29 men and 18 women of mean age 56 (range 19-87) years. Haematological malignancy was the most common disorder (42 patients). The main indications for splenectomy were cytopenia (20 patients), diagnosis (14), initial treatment of leukaemia (eight), pain (four) and spontaneous rupture (one). Thirteen patients underwent an associated surgical procedure. One patient died (mortality rate 2 per cent) and 12 (26 per cent) had postoperative complications. The advantages of splenectomy included histopathological diagnosis in 13 of 14 patients with splenomegaly of unknown origin, effective initial treatment in prolymphocytic and hairy cell leukaemia, definitive relief of pain in all affected patients, and long-term improvement of cytopenia in most.
CD16, the type III receptor for IgG, is expressed on neutrophils, natural killer cells, and some T lymphocytes, mast cells, and activated monocytes but not on cells of the B-lymphocyte lineage including plasma cells. It is also produced in a soluble form found in serum. We analyzed sera from 165 multiple-myeloma patients, 29 patients with monoclonal gammopathies of unknown significance, and 20 normal disease-free donors. We found that the level of soluble CD16 was significantly decreased in sera from patients with multiple myeloma compared to sera from healthy and monoclonal gammopathies of unknown significance donors (P = 0.0001). In addition, a stage-dependent decrease in soluble CD16 was observed, with a highly significant difference (P = 0.004) between stage I and stage II+III myeloma patients. The correlation between the myeloma stage and the serum level of soluble CD16, which is related to the host response, was found to be more sensitive than that of beta 2-microglobulin, which reflects the tumor burden. The concomitant evaluation of the serum levels of these two markers allows better staging and therefore has a more precise prognostic value.
Soluble CD16 (sCD16) are soluble part of Fc receptor for IgG which control in mice, proliferation of hybridoma B cells and IgG production. The levels of sCD16 in 165 myeloma patients and 29 patients with MGUS appear significantly different:25a.u./ml versus 144a.u./ml and values of stage I are significantly higher than those of stages II + III.sCD16 might be a prognosis factor in myeloma.
A retrospective study of twelve cases of Felty's syndrome was performed. The main points of this syndrome (clinical presentation, physiopathology, complications, treatment) are described.
Explore the source record for details and available documents.
In autoimmune haemolytic anaemia, the presence of antibodies on the erythrocyte membrane results in haemolysis through an immune process, but does it not alter the rheological properties of red blood cells (RBC), thus adding a mechanical factor to haemolysis? This study was designed to examine the rheological properties of erythrocytes sensitized with IgG-type antibodies. The study involved 20 patients with anaemia and positive direct antiglobulin test, including 12 with straightforward haemolysis, 10 samples sensitized in vitro, and 20 controls. The following haemorheological parameters were studied: erythrocyte filtration, blood and plasma viscosities, titration of adenosine triphosphate (ATP) and 2-3-DPG, erythrocyte morphology under scanning electron microscopy. The results showed increased erythrocyte rigidity (P less than 0.025) as well as higher blood viscosity compared to controls with similar haematocrit values, and unaltered ATP and 2-3-DPG (consistently with scanning electron microscope observations). These haemorheological disorders were more noticeable in patients with clear-cut haemolysis, and there was a correlation between the increase in erythrocyte rigidity indices and the haemolytic parameters, especially haptoglobin (P less than 0.001). The in vitro study confirmed the results obtained ex vivo. To conclude, the mechanical properties of antibody-coated erythrocytes are impaired, which may promote the immunological mechanism favouring haemolysis in the spleen.
A randomized trial has been performed in which 91 patients with stage III myeloma and additional severe criteria were randomly allocated to either VAD or VMBCP. No significant difference was noted between these two groups using the following criteria: response rate (VMBCP: 54%; VAD: 39%), impact on symptoms, median survival (VMBCP: 14 months, VAD: 17 months). However, toxic effects and refusal to pursue treatment were more frequent with VAD than with VMBCP (12 v 6). Therefore, in this trial, VMBCP appears to be more useful than VAD.
The authors report three cases of percutaneous fetal blood sampling at 38 weeks of pregnancies complicated by immunologic thrombocytopenia. In each case, the purity was perfect which is essential for using the examination with a view to the obstetrical management. The birth is programmed 24 or 48 hours later. In two cases, the neonatal control is in agree with the fetal blood sampling. In the third observation, the fetal platelet count in 142,000/mm3 and the newborn control 55,000/mm3: the only explanation is a very active process as usually state with newborns. There was no accident, and these samplings led to natural births. This approach seems to be the more effective for the evaluation of fetal platelet count and allow the choice of birth management. A cesarean section is decided in case of fetal thrombopenia lower than 50,000/mm3.
Explore the source record for details and available documents.
During the neonatal period, 6 infants born to mothers with auto-immune thrombocytopenia purpura were diagnosed as having severe thrombopenia (platelet counts below 30 X 10(9)/l). High doses of intravenous immunoglobulins (IVIG) (0.4 g/kg/injection for 2 infants and 1 g/kg/injection for 4 infants) were administered as of the first week of life. Short-term efficacy was good in all cases (platelet counts over 50 X 10(9)/l, 2-10 days after the first injection). During the second week of life, 5 newborns had relapses which were rapidly reversed by one or several IVIG injections. The thrombopenia was cured between days 19 (shortest) and 41 (longest) and, more over, no side effects were observed. High doses of IVIG seem to be an effective and well-tolerated treatment for neonatal auto-immune thrombopenia, and they can be recommended over classical treatments (corticotherapy, exchange transfusion, platelet transfusion) as a first line of attack.