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Biomedical subjects

A Baruchel

Publications and source records attributed to A Baruchel.

At least 73 records · Page 4Linked to original sources
PubMed1994

Treatment of juvenile chronic myelomonocytic leukemia by allogeneic bone marrow transplantation.

Twelve of 15 patients with juvenile chronic myelomonocytic leukemia (JCMML) referred to our unit underwent allogeneic bone marrow transplantation (BMT) between 1982 and 1992. BMT was not performed in the remaining three cases because of poor overall condition in two and disease progression in one. Six patients received marrow from HLA-identical siblings after a chemotherapy conditioning regimen in five cases. BMT failed in one case. Long-term remission was achieved in three patients and two others are in remission 6 and 11 months after BMT. Remission was associated with autologous recovery in one patient and minimal mixed chimerism in another. In one patient, a first BMT procedure resulted in autologous recovery and relapse. A second transplant, with chemotherapy conditioning including TBI, was successful. BMT with marrow from a matched unrelated donor was also successful. IN contrast, BMT with marrow from mismatched related donors (five patients) failed because of graft failure and/or relapse. This single-center series indicates that HLA-identical BMT is an appropriate treatment for JCMML. However, on the basis of these results it cannot be ascertained whether chemotherapy or splenectomy are necessary prior to BMT. The best chemotherapy conditioning regimen remains to be defined, as regimens consisting exclusively of chemotherapy resulted either in long-term remission or in autologous recovery with relapse.

Antineoplastic Agents↗
PubMed1994

Translocation t(11;11)(q13;q23) and HRX gene rearrangement associated with therapy-related leukemia in a child previously treated with VP16.

A child with acute myelomonocytic leukemia, bone marrow eosinophilia and inv(16) received first-line therapy including etoposide (VP-16). Cytopenia and monocytosis appeared 7 months after complete remission while the child was treated with maintenance chemotherapy. Blood abnormalities persisted after discontinuation of treatment. Nine months after complete remission, t(11;11)(q13;q23) and HRX rearrangement were detected. Five months later, overt leukemia of monocytic type occurred. The responsibility of VP-16 therapy in this treatment-related acute myelocytic leukemia is discussed.

Adolescent↗
PubMed1994

Alpha-interferon in childhood: a review.

Pediatric current and potential indications of alpha-interferon (alpha-IFN) are reviewed. Further prospective studies are needed for myeloproliferative diseases, acute leukemias, chronic hepatitis severe hemangiomas, and chronic idiopathic thrombocytopenic purpura (ITP).

Child↗
PubMed1993

Recent advances in B cell acute lymphoblastic leukemia (Burkitt leukemia) therapy in childhood.

Acute lymphoblastic leukemia with Burkitt type cells has been described in 1975. Until the mid-1980, with conventional treatments, the prognosis was very poor with very few long-term survivors. Failures were mainly due to central nervous system involvement at diagnosis or relapse. After that period a dramatic improvement has been observed with intensified treatments based on Burkitt's lymphoma therapy regimens. High-dose cyclophosphamide, high-dose methotrexate, high-dose ara-C in compact protocols completed in 6-7 months have been proven useful. 75% of the patients can be cured.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

Germline mutations of the p53 tumor-suppressor gene in cancer-prone families: a review.

Li-Fraumeni syndrome (LFS) is an autosomal dominant disorder that predisposes individuals to multiple forms of cancer including breast carcinoma, soft tissue sarcoma, osteosarcoma, leukemia and adrenocortical carcinoma. These diverse tumor types develop at unusually early ages. Analysis of the tumor suppressor gene p53 in family members with LFS have demonstrated that germline mutations in the p53 gene were present in most of the LFS family tested so far. Furthermore, germline p53 mutations were also found in cancer-prone individuals which were not indicative of the LFS.

Genes, p53↗
PubMed1992

Multicenter randomized study comparing cyclosporine-A alone and antithymocyte globulin with prednisone for treatment of severe aplastic anemia.

We report the results of a randomized multicenter study comparing the efficacy of antithymocyte globulin (ATG) with that of cyclosporin A (CsA) as first-line therapy for severe aplastic anemia (SAA). Patients were randomized to receive ATG and prednisone (PDN) or CsA; hematologic response and toxicity were compared. At 3-month evaluation, patients who had no or minimal response received the alternative therapy to assess the value of a sequential immunosuppressive therapy for treatment of severe aplastic anemia. One hundred nineteen patients were randomized; 25 were excluded, of whom 3 were misdiagnosed and 22 did not follow the cross-over protocol. Ninety-four patients were analyzed; 46 received CsA, and 48 received ATG-PDN. The actuarial survival was 66.7%, with a median follow-up time of 19 months. There was no significant difference in survival between the groups with, at 3 months, an actuarial survival of 88% in the CsA group and 75% in the ATG group (NS); at 12 months, it was 70% in the CsA group and 64% in the ATG group (NS). The percentage of complete and partial response was 11.6% and 16%, respectively, at 3 months, and 31.6% and 30%, respectively, at 12 months (NS). The main prognostic factor was the absolute neutrophil count (ANC) at entry: Patients with ANC less than 0.2 x 10(9)/L had a significantly lower survival as compared with patients with more than 0.2 x 10(9)/L ANC (P = .0001). At 12 months, 62 evaluable patients were alive, with a complete or partial response in 36 patients. Patients who had responded to the first treatment had a better recovery of bone marrow failure than those who had sequential immunosuppression. The main complication was infection, which was more often observed and more often lethal during ATG and PDN therapy. In this study, initial treatment of SAA with either CsA or ATG-PDN followed by cross-over therapy for nonresponders produced comparable response and survival rates.

Adolescent↗
PubMed1992

A multicenter randomized study comparing cyclosporin-A alone and antithymocyte globulin with prednisone for treatment of severe aplastic anemia. The cooperative group on the treatment of aplastic anemia.

This article reports the results of a randomized multicentric study comparing the efficacy of antithymocyte globulin (ATG) with cyclosporin-A (CsA) as first line therapy for severe aplastic anemia (SAA). Patients were randomized to receive ATG and prednisone (PDN) or CsA; hematological response and toxicity were compared. At 3 months, patients who had no or minimal response received the alternative therapy in order to assess the value of a sequential immunosuppressive therapy for treatment of severe aplastic anemia. One hundred and sixteen patients were analysed, 60 received CsA and 56 received ATG/PDN. The actuarial survival was 55% with a median follow-up time of 19 months. There was no significant difference in survival between the two groups. The main prognostic factor was the absolute neutrophil count (ANC) at entry: patients with ANC less than 0.2 x 10(9)/L had a significantly lower survival when compared with patients with an ANC greater than 0.2 x 10(9)/L (P = 0.0001). At 3 months, 16% of patients had a complete or partial response and a cross-over treatment was given to 68 patients. At 12 months, 77 patients were alive, with a complete or partial response in 47 patients. Patients who had responded to the first treatment had a better recovery of bone marrow failure than those who had a sequential immunosuppression. The main complication was infection which was more often observed and was more often lethal during ATG and PDN. This study demonstrates that CsA is comparable to ATG for primary treatment of SAA, but it is less toxic with fewer infectious deaths.(ABSTRACT TRUNCATED AT 250 WORDS)

Anemia, Aplastic↗
PubMed1992

A common type of the spectrin alpha I 46-50a-kD peptide abnormality in hereditary elliptocytosis and pyropoikilocytosis is associated with a mutation distant from the proteolytic cleavage site. Evidence for the functional importance of the triple helical model of spectrin.

We studied nine individuals from five unrelated families with alpha I/46-50a hereditary elliptocytosis (HE) or hereditary pyropoikilocytosis (HPP), including one of the original HHP probands first reported by Zarkowsky and colleagues (1975. Br. J. Haematol. 29:537-543). Biochemical analysis of erythrocyte membrane proteins from these patients revealed, as a common abnormality, the presence of the alpha I/46-50a peptide after limited tryptic digestion of spectrin. The polymerase chain reaction was utilized to study the structure of the DNA encoding the alpha I domain of spectrin in the affected individuals. The DNA sequence of the alpha-spectrin gene encoding the region of the alpha-spectrin chain surrounding the abnormal proteolytic cleavage site was normal. We identified a point mutation causing the replacement of a highly conserved leucine residue by proline at position 207 in the alpha-spectrin chain, a site 51 residues to the amino-terminal side of the abnormal proteolytic cleavage site. Analysis of the proposed triple helical model of spectrin repeats reveals that the mutation occurs in helix 2 at a position directly opposite the abnormal proteolytic cleavage site in helix 3, making this the first report of a mutation occurring in helix 2 of a repeat in the alpha I domain of spectrin. These results add to the molecular heterogeneity of mutations associated with HE/HPP and provide further support for the proposed triple helical model of spectrin. Disruption of this proposed alpha-helical structure by helix-breaking proline substitutions may result in a functionally defective spectrin chain.

Amino Acid Sequence↗
PubMed1992

Autologous peripheral blood stem cell transplantation after high dose therapy in patients with advanced lymphomas.

Thirty-eight patients with refractory or relapsed non-Hodgkin's lymphoma (19 patients) or Hodgkin's disease (19 patients) were treated with salvage therapy. The peripheral stem cell collection was performed during hematologic recovery after myeloablative chemotherapy. In eight patients with Hodgkin's disease the number of CFU-GM collected was less than 0.5 x 10(4)/kg and these patients were excluded for stem cell transplantation. In the remaining 30 patients, a median of 4 x 10(4) CFU-GM/kg was collected (range 0.8-100 x 10(4)/kg) by three leukaphereses in 25 patients and six to 11 leukaphereses in five patients. Conditioning regimens were CBV (eight), BEAM (six), BEAC (10) and cyclophosphamide + total body irradiation (TBI) (six). Without TBI, the mean time for reaching a granulocyte count greater than 0.5 x 10(9)/l was 18 days and for a platelet count greater than 50 x 10(9)/l was 19 days in 23 out of 24 patients. With TBI, in five patients the mean time for reaching a granulocyte count greater tahn 0.5 x 10(9)/l was 37 days and for a platelet count greater than 50 x 10(9)/l was greater than 100 days. Complications were minor. There was only one toxic death. The outcome in these patients was similar to that observed in patients who received autologous bone marrow transplantation for advanced lymphomas. In conclusion, we observed good hematologic recovery except when TBI was used in the conditioning regimen.

Adolescent↗
PubMed1992

Relapse as acute monoblastic leukemia (AML-M5) of t(6;9) acute myeloblastic leukemia (AML-M2).

Two patients with acute myeloblastic leukemia with t(6;9)(p23;q34) translocation and classified as AML-M2 relapsed as acute monocytic leukemia (AML-M5). Trisomy 8 was found associated with t(6;9) at that time in both patients. Such changes in differentiation of leukemic cells have not previously been reported in this subtype of AML and add data favoring the pluripotent nature of the stem cell involved in leukemia with t(6;9).

Child↗
PubMed1991

Nonrandom t(1;22)(p12-p13;q13) in acute megakaryocytic malignant proliferation.

A case of acute megakaryocytic leukemia (M7) and one of acute myeloid hemopathy affecting megakaryocytic and erythrocytic cell lineages in infants are reported. Both patients had t(1;22)(p12-p13;q13). This translocation was previously observed in a congenital M7 leukemia. These studies suggest that t(1;22) translocation can be nonrandom in M7.

Chromosome Banding↗
PubMed1991

Thiamine responsive anemia: report of a new case associated with a thiamine pyrophosphokinase deficiency.

We report a new case of thiamine responsive anemia in a three years old boy with the characteristic association of anemia, diabetes mellitus and deafness. Thiamine therapy corrected anemia as described. A thiamine pyrophosphokinase deficiency (TPK) was found which was not influenced by treatment. However normal levels of thiamine pyrophosphate (TPP) were obtained without correction of all symptoms particularly dyserythropoïesis. Thus, etiology of the syndrome is not only a thiamine pyrophosphokinase deficiency as previously suggested.

Abnormalities, Multiple↗
PubMed1991

Study of tolerance and effectiveness of a varicella vaccine in leukemic children.

Chickenpox could be a severe disease in immunocompromised patients. Live attenuated vaccine is now available. We conducted a trial in 37 leukemic children with OKA varicella vaccine. Full immunization was observed in all 20 seronegative patients. Few side effects and no generalized vaccinal infection were observed. Further studies are warranted to assess the benefit of vaccination in leukemic patients considering the effectiveness of Varicella Zoster Immuno-Globulins and Aciclovir.

Adolescent↗
PubMed1991

Hypermethylation of the calcitonin gene and leukemia.

Methylation of some genes is associated with tumoral progression in numerous cancers. The short arm of chromosome 11 is a hot spot for such methylations. The calcitonin gene (11p15) seems to be a good marker of these phenomenons, particularly in acute leukemias. The abnormal methylation pattern of the calcitonin gene in leukemic cells is visualized by Southern blotting after digestion of tumoral DNA with isoschizomeric restriction enzymes which are methylation sensitive or not. These studies seem of interest for acute myeloblastic leukemias (AML) where few molecular markers are available. Moreover hypermethylation of the calcitonin gene correlates with acceleration and transformation of chronic myeloid leukemia (CML). Sequential studies are warranted to evaluate if such techniques might help, especially for timing of bone marrow transplantation in CML.

Acute Disease↗
PubMed1991

Treatment of relapsed and refractory Hodgkin's disease with high dose chemotherapy and autologous bone marrow transplantation.

Despite combination of chemotherapy and radiotherapy, some patients with refractory or relapsed Hodgkin's disease (HD) have a bad prognosis. We performed high dose chemotherapy (HDCT) (BCV regimen) with autologous bone marrow transplantation (ABMT) in 26 patients with advanced HD. 11 patients were considered refractory after at least two non cross-resistant chemotherapy regimens and radiotherapy in 8 cases. 15 patients had a poor prognosis relapse (in visceral or irradiated site) after complete remission and they received chemotherapy before ABMT. At the time of transplantation 4 patients had progressive disease with B symptoms and did not respond to HDCT; they all died 3 to 12 months post-transplantation from persistent HD. For the remaining 22 patients, 3 months after HDCT 19 patients (73%) were in complete remission, 2 (8%) in partial remission and one patient died from toxicity. After complete remission 4 patients relapsed at 12 to 18 months and 15 patients are in persistent remission 24 to 46 months after transplantation. These results are encouraging in patients with bad prognostic factors but able to respond to chemotherapy.

Adolescent↗
PubMed1990

Prevention of gram-positive and Candida albicans infections using teicoplanin and fluconazole: a randomized study in neutropenic children.

We have shown that the combination of teicoplanin (T) and ceftriaxone (C) given once daily as first-line empirical antimicrobial therapy is very effective (85%) and safe in febrile neutropenic children (ICAAC, 1988). Like others (ICAAC, 1989) we have stressed the new life-threatening problems with Streptococcus mitis and Strep. sanguis. Considering the regular sensitivity of Gram-positive organisms to T, it is possible that the introduction of the glycopeptide at the onset of aplasia may be helpful. The high incidence of fungal infection or superinfection supports the routine use of an effective antifungal agent when fever is still present or relapse occurs. Our study was designed to assess the prevention of (1) staphylococcal and streptococcal infections using T when the central catheter is being placed, and (2) candida infection by the simultaneous use of a new anti-mycotic agent fluconazole (F) administered once-daily. Patients were randomized into two arms of the study. Arm A received T 6 mg/kg/d and F 3 mg/kg/d starting when the catheter was put in place. If fever occurred, C 50 mg/kg/d and amikacin (A) 15 mg/kg/d were added and T increased to 10 mg/kg/d. If a febrile relapse occurred, amphotericin B was added. In arm B the combination of the three agents T 10 mg/kg/d, C 50 mg/kg/d and A 15 mg/kg/d was started only if there was fever. Amphotericin B was used in febrile relapses. Forty-six patients were eligible (23 in each arm; no statistically significant differences between the two groups). The mean age of the patients was 8 years and mean duration of aplasia 23 and 24 d. Results were as follows: arm A the duration of T + F alone was 10 d, 22 patients had a febrile episode with 1 Gram-positive, 11 Gram-negative and one candida strain isolated in eight patients. C + A were effective in 72% of these patients. There were no deaths. In arm B, fever occurred in all patients during the first 6 d, with eight Gram-positive and six Gram-negative organisms isolated in nine patients. C + T + A were successful in 83% of patients. A bacterial superinfection was documented in four patients, candida superinfection in seven patients and one patient died from Candida parakrusei septicaemia. When the total amount of C and A used in both arms was compared, there was a reduction in 20% of the two agents in arm A, but T was equivalent in both arms. Treatment was well tolerated in both arms.(ABSTRACT TRUNCATED AT 400 WORDS)

Acute Disease↗
PubMed1990

Syndromes of inappropriate macrophage activation in childhood.

The concept of inappropriate macrophage activation (IMA) embraces the clinicopathologic conditions associated with non-malignant lymphohistiocytic proliferation: familial lymphohistiocytosis (FLH) and hemophagocytic syndromes associated with viral, bacterial, fungal or parasitic infections in both immunodeficient and immunocompetent patients. IMA is also observed during the clinical course of primary immunodeficiencies (Chediak Higashi). The mechanisms of IMA are still unknown. Prognosis varies according to the immune status of the patients but is often very poor. Steroids but principally VP16-213 can slow the macrophage hyperactivation. Allogenic bone marrow grafting is indicated in FLH.

Arthritis↗