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Biomedical subjects

B Mascret

Publications and source records attributed to B Mascret.

At least 19 recordsLinked to original sources

The value of splenectomy in chronic lymphocytic leukemia.

Between January 1, 1980, and July 31, 1985, 168 patients were treated at our institution for chronic lymphocytic leukemia (CLL). Splenectomy was performed on 32 (23 men and 9 women). The mean age of these surgical patients was 59.4 years (range, 43-75). Twenty-four patients had received chemotherapy and/or corticotherapy prior to surgery and 1 of these 24 also underwent splenic radiotherapy. The interval between diagnosis and splenectomy ranged from 1 to 108 months (mean, 33.6 months +/- 28.6). Before splenectomy, 9 patients had platelet counts greater than 100,000/mm3 (3 with normal counts), 18 had counts between 50,000 and 100,000/mm3, and 5 had less than 50,000/mm3. Seven patients had anemia with hemoglobin levels less than 10 g/dl (3 associated with thrombocytopenia); in the other 25 patients, hemoglobin levels were between 10 and 11 g/dl in 12 (9 associated with thrombocytopenia) and greater than 11 g/dl in 13. All patients but one had splenomegaly (mean spleen weight 1780 g +/- 938.7 range, 160-4300 g). One postoperative death was recorded. Postoperative morbidity occurred in 7 of 31 cases; severe infection was never observed. The average duration of hospitalization was 15 +/- 4.7 days (range, 9-28). Twenty-two of the 23 patients with thrombocytopenia less than 100,000/mm3 had complete remission after splenectomy. One had a partial remission that was later completed by means of drug therapy. Two patients relapsed within 6 months and one after 32 months. All but two cases of anemia responded to splenectomy; one patient relapsed. In 15 of 31 surviving patients, further treatment was required within a few weeks or months after surgery. Five patients died at 8, 17, 22, 26, and 35 months, respectively. The other 26 patients are alive and well with a mean follow-up of 34 months (range, 11-71 months). Splenectomy may be an effective treatment for CLL patients with splenomegaly, thrombocytopenia, and/or anemia.

Adult↗

Treatment of acute myeloid leukemia and blastic phase of chronic myeloid leukemia with combined eflornithine (alpha difluoromethylornithine) and methylglyoxal-bis-guanyl hydrazone (methyl-GAG).

A combined eflornithine-MGBG treatment was studied in patients with acute myeloid leukemia (AML) or blastic transformation of chronic myeloid leukemia (BT CML). The first ten patients (5 AML, 5 BT CML) received i.v. or p.o. eflornithine 6 g m-2 day-1 and i.v. MGBG 500 mg/m2 once a week. The duration of treatment was 5-37 days (median 15) with one to five MGBG infusions (median 2). The results were complete response (CR) in one patient, partial response (PR) in four, minimal response (MR) in one and failure (F) in four. Pronounced side effects, including severe mucositis, gastrointestinal disturbances and skin infiltration, were observed in eight patients. As the four PRs were achieved in patients with BT CML, it was decided also to study ten patients with this indication. In attempts to decrease the incidence and severity of unwanted effects, lower doses of eflornithine-MGBG were used, i.e., eflornithine 4 g m-2 day-1 and MGBG 200 mg m-2 once a week. The duration of treatment was 9-110 days (median 46), with 2-14 MGBG infusions (median 6). Responses observed were CR in two patients (in one of whom it was only transient), transient PR in two, transient MR in four, and F in two. Treatment at lower doses was better tolerated, thus allowing a longer duration of treatment. Five of ten patients had moderate or severe gastrointestinal disturbances and one patient had a severe subjective hearing loss. The eflornithine-MGBG combination may prove to be a useful alternative treatment for AML and BT CML, but comparative trials will ultimately be necessary for a more definitive assessment of the combination.

Adult↗

[Dysmyelopoiesis and T lymphoma. 2 cases].

The role played by T lymphocytes in myelopoiesis has been established in in vitro studies. Dysregulation of the lymphoid system results in quantitative and, more rarely, qualitative abnormalities of myelopoiesis. The authors report the clinical data of two cases of peripheral T cell lymphoma associated with an AREB type of dysmyelopoiesis. The fact that both conditions were diagnosed simultaneously and progressed in parallel is an indirect argument in favour of the regulation of myelopoiesis by the T lymphocytes.

Aged↗

Treatment of malignant lymphoma with high dose of chemo or chemoradiotherapy and bone marrow transplantation.

Twenty-eight patients with malignant lymphoma were treated with high dose chemo or chemoradiotherapy and allogeneic or autologous bone marrow transplantation. They can be divided in two groups: Group 1: (19 pts) consisted in patients in relapse or in n complete remission (n greater than 2) (high risk patients); Group 2 (9 pts) consisted in patients in first or second complete remission at time of bone marrow graft (standard risk patients). Complete remission was achieved in 11/17 patients evaluable for response (65%). Duration of response is very different for two groups: in group 1, all patients relapsed within a median of 2 months (range: 1-12) and died within a median of 7 months (range: 2.5-15). In group 2, 7/9 are alive and well in unmaintained CCR in a median of greater than 18 months (range: greater than 15- greater than 36) (P less than 0.01). This experience shows the feasibility of this approach, the obvious antitumoral activity of these conditioning regimens and invited us to use such therapy at an earlier stage of the disease.

Adolescent↗

High-dose melphalan with or without marrow transplantation: a study of dose-effect in patients with refractory and/or relapsed acute leukemias.

Dose-effect relationships of high-dose melphalan were evaluated in 37 patients with measurable relapsed or refractory acute leukemias. Thirteen patients (Group 1) received 70-100 mg/m2 of melphalan without marrow rescue and 24 patients (Group 2) received 140-180 mg/m2 of melphalan followed by marrow transplantation. Patients in both groups were comparable with respect to age, sex, diagnosis, and status of the leukemia. The complete remission rate was 23% in Group 1 versus 75% in Group 2 (P less than 0.01). Hematological recovery of remission patients was not statistically different in either group. Nonhematological toxicity was comparable in the two dose ranges examined. These results demonstrate the existence of a dose-response effect of high-dose melphalan regimens in relapsed acute leukemias, without marked increases in nonhematological toxicity with these doses.

Acute Disease↗

Modified chemotherapy with carmustine, cytarabine, cyclophosphamide, and 6-thioguanine (BACT) and autologous bone marrow transplantation in 24 poor-risk patients with acute lymphoblastic leukemia.

Twenty-four poor-risk patients with acute lymphoblastic leukemia received a modified regimen of carmustine, cytarabine, cyclophosphamide, and 6-thioguanine (BACT) followed by autologous bone marrow transplantation (ABMT). Nineteen patients were in second or subsequent complete remission (CR) when treated with this regimen; 3 died early, 2 died of pneumonia in CR, 11 relapsed within 3 months (median), and 3 remain in CR with no maintenance therapy 14-24 months after ABMT. Of the 5 patients with measurable disease who were treated, 3 had CR and 1 remains in CR without maintenance therapy more than 28 months after ABMT. The toxicity of this regimen was acceptable, but late pulmonary toxic effects remain a major concern. These results are poor in terms of efficacy, and new effective methods of eradicating acute lymphoblastic leukemia in patients with poor prognosis should be investigated.

Adolescent↗

[Chromosome polymorphism in allogeneic bone marrow grafts. Methods and results].

Cytogenetic studies using constitutive heterochromatin variations represent one of the best method in documentation of allogeneic bone marrow transplantation (BMT). Sex mismatched transplants are evaluated by gonosomal examination. In sex matched patients, several banding techniques are performed before BMT to detect autosomal polymorphism in donor and recipient. Can be informative: chromosomes 3 and 4 centromere, acrocentric centromere and satellites, chromosomes 1, 9 and 16 secondary heterochromatic constriction. Authors describe their methodology for these studies and results about 81 patients.

Bone Marrow Transplantation↗

[Allogeneic bone marrow transplantation in the treatment of acute leukemias of children. Study of 26 patients].

Twenty-six children with acute leukemia were treated with allogeneic marrow transplantation from HLA identical siblings after a conditioning regimen with Cyclophosphamide-total body irradiation (19 patients), Melphalan-total body irradiation (6 patients) or Busulfan-cyclophosphamide (1 patient). Eighteen were transplanted in complete remission (4 with acute non lymphoblastic leukemia in first remission, 14 with acute lymphoblastic leukemia: 6 in first, 6 in second and 2 in subsequent remission): 2 died of cytomegalovirus pneumoniae, 1 relapsed and 15 survive in continuous complete remission from 5 to 42 months after transplantation (median = 22 months). Eight were transplanted in relapse, 7 achieve complete remission, 5 of them relapsed, 1 died of G.V.H. and 1 survives in continuous complete remission 46 months after transplantation. Actuarial analysis shows a disease free survival rate at 3 years of 82% for patients transplanted in remission and 12% for patients transplanted in relapse (p less than 0.01).

Acute Disease↗

Phase-I-II study of high-dose melphalan and autologous marrow transplantation in adult patients with poor-risk non-Hodgkin's lymphomas.

Eleven adult patients with poor-risk non-Hodgkin's lymphoma were treated with high-dose melphalan (140 mg/m2) or high-dose combination chemotherapy (BCNU, Ara-C, vindesine and melphalan) followed by autologous bone marrow transplantation. Six of the eight patients evaluable for response achieved complete remission and one achieved partial remission. Response duration ranged from 1.5 to 12 months (median 2 months). Prompt hematological recovery occurred in all patients. The duration of aplasia and the extrahematological toxicity were similar in both groups. High-dose melphalan alone or associated with other drugs followed by marrow infusion appears to produce a high response rate and demonstrates the potential for salvaging patients with refractory lymphoma.

Adult↗

[Factor X deficiency and systemic amylosis].

The case of a 52-year-old patient with systemic amylosis diagnosed upon examination of an osteomedullary biopsy specimen is reported. The most prominent clinical manifestation was a major hemorrhagic syndrome which was recognized as being a result of severe factor Stuart deficiency. The new classification of amyloses based on the latest biochemical and immunological findings is recalled. Current physiopathogenic speculations put emphasis on plasmocyte-macrophage cooperation. Lastly, factor Stuart deficiency, which is as classical as it is uncommon, could originate in the particular affinity of amyloid fibers for this factor and in a "mass effect" (quantity of amyloid substance directly exposed to blood flow), explaining why splenectomy is effective in some instances.

Amyloid↗

[Peripheral T cell lymphoma].

We report three cases of malignant T cell lymphoma of peripheral T lymphocyte origin. This morphological entity was first described by Lennert and Waldron. The diagnosis based on morphological criteria only is difficult because of the many lymphoid malignancies. Immunological characterization is necessary for the identification of the proliferative T lymphocyte subset with a panel of T monoclonal antibodies. Correlations were established between immunological phenotype and clinical and evolutive aspects which are particularly varied in T lymphoproliferative disorders.

Adult↗

[Granulocytic progenitors in bone marrow autografts].

Granulocytic progenitor cells (CFC) are a better reflection of stem cell pools than precursors. Their total number can be estimated during hematologic steady-state (N = 70 +/- 29 x 10(5) CFC/kg); the amount of marrow necessary to perform a bone marrow transplantation is only 1% of the total pool. CFC are useful but have a mainly qualitative value in evaluating the viability of marrow stored for 24 h at room temperature (92%) or marrow thawed after DMSO cryopreservation (62%). Sequential study of CFC in patients receiving autologous marrow transplantation enables differentiation of engraftment from autologous recovery. In 11 recipients of autologous marrow transplantation, no statistical relation was observed between number of infused CFC and severity of granulocytopenia. Further studies will be necessary to evaluate a better marker of real hematopoietic stem cells.

Adolescent↗