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Biomedical subjects

A Ferster

Publications and source records attributed to A Ferster.

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

Improved survival for acute lymphoblastic leukaemia in infancy: the experience of EORTC-Childhood Leukaemia Cooperative Group.

Out of 744 newly diagnosed ALL children under the age of 18 years treated according to the EORTC-CLCG protocols 58831 and 58832, 28 (4%) were infants less than 1 year of age. An elevated risk factor, which takes into account the sizes of the liver and spleen and the number of circulating blasts, was present in 25 cases. Most patients had non-common ALL. Among 15 patients studied by cytogenetics, nine present chromosomal abnormalities, six of them having a t(4;11) translocation. Complete remission was achieved in 86% of cases. One patient died in complete remission of therapy-related infection. The overall EFS is 43%. It is not statistically different in very young infants as compared to infants older than 6 months. Except for patients with AUL or with t(4;11) translocation, a continuous complete remission rate above 50% can be achieved with a median follow-up of 4 years. The results obtained in infant ALL with EORTC-CLCG protocols are currently better than those obtained with some other protocols, but remains inferior when compared to the ones obtained in older children. Thus, further improvements are needed and should be evaluated in large cooperative trials.

Age Factors↗
PubMed1994

[Stomatococcus mucilaginosus meningitis in immunocompromised child].

BACKGROUND: Opportunistic infections are responsible for significant morbidity and mortality in patients with malignancy developing granulocytopenia as a result of therapy. A case of Stomatococcus mucilaginosus meningitis is reported. CASE REPORT: A 2 year-old boy was admitted because he had developed fever and neutropenia during chemotherapy given for neuroectodermal tumor. He was previously treated for a Stomatococcus mucilaginosus septicemia with vancomycin given for 15 days through an intravenous catheter which has subsequently been left in place. At admission, fever was associated with severe degree of aplasia (70 WBC/mm3). The patient was given IV ceftazidime plus amikacin. Two days later, the boy developed acute meningitis due to Stomatococcus mucilaginosus; he was then given IV vancomycin (40 mg/kg/d) and imipenem (100 mg/kg/d). Persistence of abnormal clinical and bacteriological findings required subsequent intrathecal administration of vancomycin (1.5 mg/d) for 5 days. CSF cultures were negative 2 days later, leading to stop IV antibiotics after 3 weeks. The catheter was removed 1 week later. CONCLUSION: This patient represents the third reported case of Stomatococcus mucilaginosus meningitis. Combined intrathecal and systemic administration of vancomycin seems to have been useful in our case.

Child, Preschool↗
PubMed1994

Complete remission following donor leukocyte infusion in ALL relapsing after haploidentical bone marrow transplantation.

A 7-month-old boy with a high risk ALL harbouring the translocation (4;11) was grafted with an haploidentical bone marrow from paternal origin. At time of relapse, 11 months after BMT, he received donor leukocyte infusions (DLI) which put him in second CR. GVHD and pancytopenia occurred 2 weeks after DLI and were fully reversed with CsA + prednisolone. Six months later, the child continues to be in second CR, off steroid therapy, without any signs of GVHD. Our limited experience indicates that a second CR can be obtained with acceptable toxicity by DLI in very high risk ALL children who have been previously grafted with haploidentical bone marrow cells.

Bone Marrow Transplantation↗
PubMed1993

Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia.

In sickle cell anemia (SCA), the loss of reticuloendothelial function is the result of vasoocclusive events occurring in the spleen. Such asplenia occurs early in the course of the disease and is considered to be permanent in late childhood. In this report, three patients 10, 11, and 14 years of age suffering from severe SCA and found to be asplenic were treated by bone marrow transplantation (BMT). Before transplantation, all three patients had loss of reticuloendothelial splenic function, as assessed by the presence of abundant Howell-Jolly bodies on blood smears and absence of technetium 99m (99mTc) splenic uptake. After BMT, Howell-Jolly bodies disappeared from blood smear, whereas 99mTc isotopic scan found normal isotope uptake. Our data indicate that BMT can correct "permanent asplenia" in SCA patients. However, it remains to be determined if such treatment can also correct other SCA-related organ dysfunctions.

Adolescent↗
PubMed1993

T helper type 2-like cells and therapeutic effects of interferon-gamma in combined immunodeficiency with hypereosinophilia (Omenn's syndrome).

We characterized the defects of CD4+ cells in a 17-month-old girl suffering from combined immunodeficiency with hypereosinophilia (Omenn's syndrome). Because the vast majority of peripheral blood CD4+ cells expressed the CD45R0 isoform, we purified circulating CD4+ CD45R0+ cells from the patient and healthy individuals in order to compare their production of cytokines. The patient's CD4+ CD45R0+ cells spontaneously produced high levels of interleukin-5 (IL-5) in vitro (1600 pg/ml after 24 h of culture) and this was associated with the presence of IL-5 in serum (323 pg/ml). After stimulation with phorbol 12-myristate 13-acetate (PMA) and calcium ionophore A23187, they produced higher levels of IL-4 (306 vs. 55 +/- 4 pg/ml) and IL-5 (2900 vs. 213 +/- 72 pg/ml) and lower levels of IL-2 (17 vs. 63 +/- 17 IU/ml) and interferon-gamma (IFN-gamma) (16 vs. 299 +/- 70 IU/ml) than controls CD4+ CD45R0+ cells. This T helper type 2 (Th2) pattern was confirmed by the detection using reverse polymerase chain reaction of IL-4, IL-5 and IL-10 mRNA within peripheral blood mononuclear cells. During a therapeutic trial with human IFN-gamma (40 micrograms/day) which ameliorated the clinical status of the patient, we observed a down-regulation of the in vivo expression of IL-5 and IL-10, a normalization of the eosinophil count and an improvement of the T cell response to phytohemagglutinin. This observation indicates for the first time that Th2-like cells might be involved in certain forms of congenital immunodeficiency and that IFN-gamma might down-regulate their activities in vivo.

CD4 Antigens↗
PubMed1993

Cardiac surgery with extracorporeal circulation in severe factor VII deficiency.

We describe here successful open heart surgery with extracorporeal circulation and haemodilution in a child with severe congenital factor VII deficiency. Substitution therapy was realized with factor VII concentrate (SD proconvertin concentrate, Bio-Transfusion). This is the first report case of successful cardiac surgery in severe congenital factor VII deficiency.

Child, Preschool↗
PubMed1993

[Granulocytic sarcoma with mediastinal involvement].

BACKGROUND: Granulocytic sarcoma is more frequent in adults; it can be a tumoral localization of acute non-lymphoblastic leukemia. This report describes an infantile case revealing an acute myeloid leukemia. CASE REPORT: A 14 month-old girl was admitted because of enlarged bilateral cervical lymph nodes. They had increased in volume over the past month and were accompanied by fever and anorexia. The liver and spleen were moderately enlarged. Hematologic data were: Hb = 7.5 g%; leucocytes = 14,900/mm3; platelets = 614,000/mm3. There were no abnormal cells. X-rays and MRI showed a mediastinal mass. Bone scintigraphy was normal but the myelogram showed a few atypical cells that were also seen in a biopsy of the cervical lymph node. Electron microscopic examination of these cells showed that they were myeloid in origin. This was confirmed by immunohistochemistry. The cytogenetic examination showed many chromosomal abnormalities in the lymph node and myelogram. The child is now recovered after nine months of chemotherapy. CONCLUSION: Diagnosis of acute myeloblastic leukemia in children is difficult when it is revealed by granulocytic sarcoma without major infiltration of bone marrow. The clinical presentation as a mediastinal mass is rare. The cytogenetic study was determinant for diagnosis.

Bone Marrow↗
PubMed1993

Bone marrow transplantation in sickle cell disease: the Belgian experience.

Twenty eight patients underwent bone marrow transplantation (BMT) for sickle cell anemia in Belgium. The patients were originating from Central Africa, were young and symptomatic. Engraftment occurred in all patients and was sustained in 25. In 3 patients, a bone marrow rejection was observed. One of these patients underwent a second BMT and had an uneventful recovery. One patient died 3 months after BMT of graft-versus-host disease (GVHD). Twenty seven patients are alive and 25 are free of vaso-occlusive related manifestations with a follow-up ranging from 4 to 78 months. So far, eight patients went back to Africa and continue to do well.

Adolescent↗
PubMed1992

Thyroid function during L-asparaginase therapy in children with acute lymphoblastic leukemia: difference between induction and late intensification.

Parameters of thyroid function were measured in nine children during induction therapy for acute lymphoblastic leukemia (ALL). Seven patients were reevaluated during the late intensification phase. Vincristine, anthracycline drugs (daunorubicin and adriamycin), corticosteroids, and L-asparaginase were used in the two phases of therapy. During induction, L-asparaginase (5,000 U/m2) was given daily on days 1-21. During late intensification, the drug was given on days 1, 4, 8, and 11 (10,000 U/m2). During induction, total T4 significantly decreased from 10.1 +/- 2.3 to 4.0 +/- 1.0 micrograms/dl, FT4 decreased from 1.4 +/- 0.3 to 0.7 +/- 0.2 ng/dl, total T3 decreased from 134 +/- 32 to 57 +/- 21 ng/dl, and thyroxine binding globulin (TBG) decreased from 2.1 +/- 0.6 to 0.8 +/- 0.3 mg/dl. During late intensification therapy, significant decreases in T3 (from 180 +/- 26 to 93 +/- 40 ng/dl) and TBG (from 1.9 +/- 0.5 to 1.1 +/- 0.5 mg/dl) were observed, but there were no significant changes in T4 and FT4. We conclude that during induction, the impairment of thyroid function is attributable to L-asparaginase, whereas during late intensification, low T3 and low TBG is due to glucocorticoid administration.

Adolescent↗
PubMed1992

Bone marrow transplantation for severe sickle cell anaemia.

Five children with sickle cell anaemia underwent bone marrow transplantation (BMT) for severe clinical disease. The conditioning regimen for BMT was in busulfan plus cyclophosphamide. The allograft contained more than 5 x 10(8) nucleated cells per kg recipient. Prophylaxis of GVHD consisted of methotrexate and cyclosporin A. Therapy was well tolerated. Duration of neutropenia (less than 0.5 x 10(9)/l) was short (14-25 d). Platelet recovery (greater than 50 x 10(9)/l) occurred between day 12 and 45. The patients have been followed up for 8-28 months. No major infections occurred and long-term BMT-related toxicity was limited to mild, chronic GVHD in one patient. Mean haemoglobin levels remained above 10 g/dl. Haemoglobin electrophoresis showed AS patterns in all grafted patients--all marrow donors having sickle cell trait. From our preliminary data, we conclude that BMT or sickle cell anaemia is curative, well tolerated and should be proposed for suitable patients.

Anemia, Sickle Cell↗
PubMed1992

[Autologous bone marrow graft in solid tumors in childhood].

Curability in children suffering from malignant solid tumor is 50%. Thus, high dose chemotherapy with or without total body irradiation followed by autologous bone marrow transplantation (ABMT) has been proposed to patients suffering from cancer either at initial diagnosis (poor prognosis tumor) or at relapse. Thanks to these studies, drugs having dose effects properties have been selected. In some tumors, ABMT has significantly improved patients median survival. It remains to be determined if: 1. high dose chemotherapy protocols with ABMT are superior to new aggressive chemotherapeutic protocol without ABMT. 2. ABMT increases the curability of high risk patients.

Bone Marrow Transplantation↗
PubMed1992

[Indications for bone marrow graft in severe hemoglobinopathies].

Allogenic bone marrow transplantation (ABMT) is the only curative approach for sickle cell anemia and major beta-thalassemia. In sickle cell anemia, ABMT can be proposed for severe clinical disease. In major beta-thalassemia, it must be proposed to young patients who have an HLA identical familial donor.

Anemia, Sickle Cell↗
PubMed1992

Performance of an immunoradiometric assay of erythropoietin and results for specimens from anemic and polycythemic patients.

We evaluated a new commercially available two-site immunoradiometric assay (IRMA; BioMérieux 125I-EPO CoatRIA) for erythropoietin (EPO) in human serum. The precision (CV) was 4.1% intra-assay and 8% interassay for a serum pool with an EPO concentration of 17 int. units/L; the detection limit was 0.5 int. unit/L, one order of magnitude lower than by classical radioimmunoassay (RIA), although standardization of IRMA and RIA were similar. Results by both IRMA and RIA are compared for normal subjects, patients with nonrenal noninflammatory anemias, patients with beta-thalassemia major, hemodialysis patients, and patients with primary or secondary polycythemia. Values by IRMA compared well with those by RIA in the upper area of the range; IRMA and RIA values for EPO show parallel expected variations with the degree of anemia. However, because of its greater sensitivity and specificity, we consider the IRMA more appropriate than RIA for investigating patients with sub-normal EPO concentrations.

Adolescent↗