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Biomedical subjects

A Ferrant

Publications and source records attributed to A Ferrant.

At least 127 records · Page 7Linked to original sources
PubMed1994

Laparoscopic splenectomy for idiopathic thrombocytopenic purpura.

Laparoscopic splenectomy was performed on eight patients with idiopathic thrombocytopenic purpura refractory to medical treatment. Preoperative infusion of immunoglobulin G gamma-globulin was used to boost the platelet count. Accessory spleens were sought by preoperative computed tomography and peroperative examination of the usual anatomical locations. Seven patients underwent successful laparoscopic splenectomy, with a mean postoperative stay of 3.6 days. One patient with an accessory spleen detected before operation but not during laparoscopy required conversion to open surgery for control of haemorrhage from the splenic hilum. Another patient had a transient pancreatic fistula. Laparoscopic splenectomy is feasible and sfe in patients with idiopathic thrombocytopenic purpura. Long-term results require evaluation as detection of accessory spleens can prove difficult during laparoscopy.

Adolescent↗
PubMed1994

High expression of bcl-2 is the rule in acute lymphoblastic leukemia, except in Burkitt subtype at presentation, and is not correlated with the prognosis.

Bcl-2 expression and its prognostic value were evaluated using the APAAP technique in 40 ALL patients. Because of a possible synergy between c-myc and bcl-2 proteins in cell lines, we compared 40 ALL of either T or B lineage with seven Burkitt's ALL or sporadic Burkitt's lymphomas (BL). We found the same high expression of bcl-2 in adult (Ad) and childhood (Ch) malignancies examined at presentation, regardless of the T or B phenotype. No bcl-2 expression was detectable in BL cases, except for two investigated at relapse. Bcl-2 staining intensity was also not related to the stage of blast differentiation, except in Ad T-ALL, or to the relapse rate. Ki-67, a marker of proliferation, was used to investigate the possible correlation between bcl-2 expression and the proliferative activity. An inverse correlation was found only in BL at presentation. We confirm that bcl-2 expression is the rule in ALL, regardless of the immunophenotypic characteristics at presentation, and that high expression is not correlated with a bad prognosis. Sporadic BL may represent a particular type of tumor, with bcl-2 expression in relapse, but not at presentation.

Adolescent↗
PubMed1994

Minor myeloid component in Ph chromosome-positive acute lymphoblastic leukaemia: correlation with cytogenetic pattern and implication for poor response to therapy.

Morphological, immunological and cytogenetic features were studied in 27 adults presenting with Ph chromosome-positive acute lymphoblastic leukaemia (ALL), in correlation with clinical outcome. Twenty patients (group 1) were diagnosed as having typical ALL according to the FAB criteria supported by immunological findings. Less than 1% blast cells with azurophilic granules were detected in all cases. Myeloid cytochemistry, i.e. peroxidase and Sudan black-B stain, was negative in all cases. A minor phenotype deviation consisting of the expression of the CD13 myeloid-associated marker was detected in two patients. In seven patients (group 2) a diagnosis of ALL with a minor myeloid component was made because of the presence of a majority of lymphoid blasts and of 5-15% blast cells with morphological cytochemical and immunological features of the myeloid lineage. Abnormal metaphases were found in 6/20 (30%) patients in group 1, compared with 7/7 (100%) patients in group 2. All patients were treated by antilymphoid regimens; however, complete remission was achieved in 17/20 (85%) patients in group 1 versus 1/7 (14.3%) patients in group 2. Median survival was 16 months, range < 1-120+ in group 1 and 9 months, range < 1-15 in group 2. It is concluded that morphological, immunological and cytogenetic studies allow for the recognition of two cytological subsets of Ph+ ALL. The presence of a minor myeloid component in otherwise typical Ph chromosome-positive ALL may be associated with a distinct cytogenetic pattern and poor responses to antilymphoid therapy.

Adult↗
PubMed1994

Anti-HCV seroconversion in multitransfused and immunocompromised patients. A long-term longitudinal study.

In a series of 6 multitransfused, immunocompromised patients, the diagnosis of posttransfusion hepatitis C was based upon the analysis of long-term follow-up serum samples. The HCV RNA was detected by a nested PCR assay using primers located in the 5' noncoding region (5'NCR), and anti-HCV antibodies were assayed with third-generation tests. The interval between the first rise in alanine aminotransferase and seroconversion varied from less than 5 months to more than 38 months. Five out of 6 patients seroconverted after 14 months or later. In most cases, the anti-NS3 and anti-NS4 antibodies appeared first. In such patients, the etiology of chronic liver disease may thus be overlooked for 1 or more years, a definitive diagnosis requiring the detection of HCV RNA.

Adult↗
PubMed1994

A single course of 2-chloro-deoxyadenosine does not eradicate leukemic cells in hairy cell leukemia patients in complete remission.

The nucleoside analog 2-chlorodeoxyadenosine (2-CdA) has recently emerged as a most promising treatment for hair-cell leukemia (HCL). The response rates are high regardless of prior therapy, and the duration of complete responses (CR) after a single course of treatment is longer than with any other therapeutic agent. We investigated the presence of minimal residual disease (MRD) in ten HCL patients treated in our institution with 2-CdA. The presence of residual leukemic cells was investigated in patients in CR following one course of treatment, using the polymerase chain reaction (PCR) and heavy-chain immunoglobulin genes (IgH), or TCR delta derived clonospecific probes. Eight patients achieved a complete remission after a single course of treatment, as evaluated at 6 months. Among these patients, seven are still in CR with a median follow-up of 12 months (range, 6-20 months) and one has relapsed after 15 months. Using PCR, all the evaluable patients remaining in CR showed persistent evidence of detectable MRD with no sign of decrease over the observation period. From this small series, we conclude that a single course of 2-CdA does not eradicate HCL and that persistence of residual leukemic cells appears to be common in patients in complete morphologic remission. Whether persistence of MRD will have an impact on long-term outcome, or whether HCL patients in morphologic CR with persistent MRD will remain so, is a matter of longer follow-up.

Aged↗
PubMed1994

2-Chlorodeoxyadenosine therapy in advanced chronic lymphocytic leukaemia.

The therapeutic potential of 2-chlorodeoxyadenosine (CdA) in patients with advanced chronic lymphocytic leukaemia (CLL) remains controversial with response rates in clinical trials ranging from 44 to 67%. This report describes our experience with CdA in 22 CLL patients having already undergone previous treatment. CdA was given by continuous intravenous infusion at a dose of 4 mg/m2/day for 7 days (4 patients) or as 2-h intravenous infusions at a dose of 5.6 mg/m2/day for 5 days (18 patients). Partial (n = 5) or complete (n = 2) response was obtained in 7 cases. As compared to unresponsive patients, responding subjects received CdA earlier in the course of their disease (mean interval between diagnosis and CdA therapy 58 vs 102 months), were less thrombocytopenic at initiation of CdA (mean platelet count 165 x 10(9)/L vs 81 x 10(9)/L) and experienced less severe neutropenia during the first course of therapy (mean minimal neutrophil count 1.55 x 10(9)/L vs 0.43 x 10(9)/L). None of 6 patients with CLL refractory to fludarabine responded to CdA. An evaluation of haematological toxicity during the first course of treatment showed grade 4 neutropenia (< 0.5 x 10(9)/L) in 7 cases and grade 4 thrombocytopenia (< 25 x 10(9)/L) in one of 19 cases where the platelet count was greater than 25 x 10(9)/L at initiation of CdA. In comparison with earlier reports, the present series of patients had received relatively heavy prior therapy, experienced more severe haematological toxicity and demonstrated a lower total response rate.

Aged↗
PubMed1994

2-Chlorodeoxyadenosine therapy in Waldenstrom's macroglobulinaemia.

Despite some encouraging first results, experience with 2-chlorodeoxyadenosine (CdA) in the treatment of Waldenström's macroglobulinaemia (WM) has not as yet been very extensive. The present paper reports a clinical trial of the use of CdA in 18 patients having previously treated (n = 13) or untreated (n = 5) WM. CdA was administered by continuous intravenous infusion at a dose of 4 mg/m2/day for 7 days (5 patients) or as 2-h intravenous infusions at a dose of 5.6 mg/m2/day for 5 days (13 patients). Partial response was obtained in 7 cases. In this small series, no correlation could be found between response to CdA and patient characteristics at inclusion. During the first course of therapy, grade 4 neutropenia (< 0.5 x 10(9)/L) and thrombocytopenia (< 25 x 10(9)/L) developed in respectively 4 and 6 cases. In comparison with earlier reports haematological toxicity was more severe and the overall response rate lower in the present series of patients.

Aged↗
PubMed1994

What clinical and laboratory data are indicative of polycythemia and when are blood volume studies needed?

An increased hematocrit can be caused by primary proliferative polycythemia (PPP), by secondary polycythemia, by relative polycythemia (reduced plasma volume with a normal red cell mass), or by modifications of the red cell mass and the plasma volume within their normal ranges. As an increased hematocrit by itself is a risk factor for thrombosis, it is important to diagnose not only polycythemia, but also its possible cause, in order to offer optimal therapy. Smoking is the most frequent cause of an increased hematocrit. Splenomegaly, aquagenic pruritus, and erythromelalgia often exist in PPP, whereas other symptoms such as dyspnea are more likely to be associated with secondary polycythemia. Smokers with an increased hematocrit will be asked to stop smoking before ordering blood volume studies. These studies are not indicated in patients with obvious pulmonary disease. Male patients with an hematocrit over 60% and female patients with an hematocrit over 55% always have absolute polycythemia. The associations of an increased hematocrit with splenomegaly, a raised white blood cell count or thrombocytosis are indicators for PPP. The necessity for blood volume studies is questionable in these patients. However, blood volume studies are useful in patients with an increased hematocrit and no other clinical or biological signs suggestive of any form of polycythemia.

Blood Cell Count↗
PubMed1993

Long-term follow-up of residual disease in acute lymphoblastic leukemia patients in complete remission using clonogeneic IgH probes and the polymerase chain reaction.

We sequentially studied bone marrow (BM) samples of 25 patients in complete remission of an acute lymphoblastic leukemia (ALL) using a simplified polymerase chain reaction (PCR) strategy (direct use of the PCR product as a clonogenic probe recognizing rearranged Ig heavy chain sequences) as a first approach. BM aspirates were serially investigated after obtention of a complete response. When sensitivity was less than 1:10(4), the PCR fragment was sequenced and a specific oligonucleotide was synthetized and used as a probe (five cases). Cases in which minimal residual disease (MRD) became undetectable were cross-controlled using either TCR delta rearrangement or a specific translocation to circumvent the problem of false-negative results arising from clonal evolution. The median follow-up was 30 months (3 to 51 months). Within the first 3 months of complete remission, MRD was detectable in 22 of 23 investigated patients and remained so in 19 of 21 patients examined at 6 months, regardless of the long-term clinical outcome. In patients remaining in complete remission at 30 months or more, two patterns of MRD emerged during the follow-up. Either it continuously decreased to ultimately become undetectable (five patients) or remained detectable (five patients) with an increase after discontinuation of treatment in two. In the eight patients who relapsed, MRD persisted throughout the clinical course, and eventually increased 3 to 12 months before relapse was clinically detectable. In one case, clonal evolution of the VDJ heavy chain region was observed and recurrence of MRD shown by the use of TCR delta rearrangement as a control. We conclude that the use of this simplified methodology is a valuable tool for the follow-up of MRD in a majority of ALL patients, though in a few cases, sequencing needs to be performed to achieve a relevant sensitivity. The possibility of clonal evolution requires a cross-control of any sample becoming negative whatever the initial rearrangement used to generate a probe. In patients on therapy, sequential search for MRD seems to be a good tool for predicting the long-term outcome. In addition, patients remaining positive at the time treatment is discontinued or with a high tumor burden after a few months therapy may be at a higher risk of subsequent relapse, although a longer follow-up is needed to answer this question.

Adolescent↗
PubMed1993

52Fe for additional marrow ablation before bone marrow transplantation.

The effectiveness of bone marrow transplantation (BMT) for malignant blood diseases remains limited by the inability of the preparative regimen to eliminate the disease without causing toxicity to normal organs. We have used 52Fe to deliver radiotherapy selectively to the BM. Fourteen patients with hematologic malignancies received 52Fe before a conventional BMT conditioning regimen. The median 52Fe dose was 58 mCi (range, 32 to 85 mCi). As evaluated by quantitative scanning, the median percentage of 52Fe taken up by the BM was 82% (range, 36% to 90%). This resulted in a median radiation-absorbed dose to the BM of 632 rad (range, 151 to 1,144 rad). The median uptake of 52Fe by the liver was 18% (range, 10% to 64%) and the median radiation-absorbed dose to the liver was 239 rad (range, 82 to 526 rad). The median whole body radiation-absorbed dose was 46 rad (range, 22 to 68 rad). No untoward effects were noted after the injections of 52Fe. The patients recovered hematopoiesis without toxicity in excess of that expected with conventional conditioning alone. The median follow-up was 8 months and three patients have relapsed. 52Fe should provide a way to boost the radiation dose to marrow-based diseases before marrow transplantation without increasing toxicity.

Absorption↗
PubMed1993

Reflex sympathetic dystrophy after bone marrow transplantation.

We report on three patients of 201 who, after bone marrow transplantation (BMT), developed reflex sympathetic dystrophy (RSD). The syndrome occurred on days +40, +43, and +147, respectively, without known causes of RSD. The ankles, the feet, and the knees were affected. Long-lasting immobilization, slow bone marrow recovery, and other BMT-related factors could have predisposed to RSD.

Adult↗
PubMed1993

Breast infiltration by acute lymphoblastic leukemia during pregnancy.

A case of bilateral breast infiltration as the initial manifestation of FAB-L3 leukemia during pregnancy is reported. This rare presentation is usually associated with Burkitt's lymphoma or FAB-L3 leukemia. A high suspicion of the presence of hematologic malignancy and appropriate evaluation are indicated in the presence of this clinical manifestation.

Adult↗
PubMed1993

Minimal residual disease after allogeneic bone marrow transplantation for chronic myeloid leukaemia in first chronic phase: correlations with acute graft-versus-host disease and relapse.

We have studied 61 patients who underwent allogeneic bone marrow transplantation (BMT) for chronic myeloid leukaemia (CML) in first chronic phase. Minimal residual disease was detected by the amplification of the leukaemia-specific BCR-ABL fusion mRNA with the polymerase chain reaction (PCR) using a highly sensitive nested primer strategy. As a general pattern, patients often had detectable BCR-ABL (PCR positive) for up to 6 or 9 months post BMT after which time BCR-ABL became undetectable (PCR negative). The conversion from PCR positive to PCR negative was not associated with the time at which cyclosporin A treatment was stopped. Six patients (10%) have relapsed during the period of this study, two within 1 year and four more than 1 year after transplant. The relationship between PCR positivity more than 1 year post transplant and relapse was significant (P = 0.036) but 15 patients who were PCR positive beyond 1 year remain in complete clinical and cytogenetic remission. Thus late positivity identifies a group of patients at increased risk of relapse but is of little predictive value for individual patients. Of the four late relapses, two had been persistently PCR positive and two were initially PCR positive, converted to negative and subsequently to positive again. Although all relapses were preceded by PCR positivity, relapse may occur only 12 months after a PCR negative result. The proportion of patients PCR negative at 3/4 months after BMT was found to increase significantly with the severity of acute GVHD (P = 0.002) but no relationship was found between acute GVHD and subsequent PCR results. There was no clear association between severity of chronic GVHD and PCR result.

Acute Disease↗
PubMed1993

Oxidative injury to erythrocytes, cell rigidity and splenic hemolysis in hemodialyzed patients before and during erythropoietin treatment.

The oxidative injury to erythrocytes, red blood cell (RBC) rigidity and splenic hemolysis was assayed in 17 chronically hemodialyzed patients before and during recombinant erythropoietin (EPO) treatment. When a stable hematocrit between 30 and 35% had been established for at least 4 months, a statistically significant increase in RBC volume, hemoglobin concentration, hematocrit, reticulocyte count, and several RBC enzymes (2,3-diphosphoglycerate, glucose 6-phosphate dehydrogenase, pyruvate kinase, hexokinase) was noted. This indicated significant RBC rejuvenation under the influence of EPO. However, no significant improvement in the RBC oxidative sensitivity, RBC deformability, splenic RBC volume, slow mixing splenic RBC volume, and the intrasplenic RBC transit time could be disclosed. These data confirm the existence of an extra-erythrocytic factor in uremic plasma, which is partly responsible for a reduced RBC life span in hemodialysis patients despite EPO treatment.

Adult↗
PubMed1993

Splenic irradiation before bone marrow transplantation for chronic myeloid leukemia: update of a prospective randomized study.

Two hundred and twenty-nine patients with chronic myeloid leukaemia in chronic phase awaiting bone marrow transplantation from an HLA-identical sibling donor were randomized as part of their conditioning, to receive splenic irradiation (SI+, 115 patients), or not (SI-, 114 patients). Both groups were identical in regard to age, sex, donor/recipient sex combination and disease activity. Survival, leukaemia-free survival, incidence of transplant-related mortality, acute and chronic graft versus host disease, incidence of rejection and probability of relapse were not different in either groups at a median follow-up time of 4.5 years (minimum follow-up 2 years). Recovery of peripheral white blood cell counts to 1 x 10(9)/l but not of platelet counts to 50 x 10(9)/l was significantly faster in patients with SI+ (21 vs 24 days). This small benefit does not justify routine splenic irradiation prior to BMT, in CML.

Adolescent↗