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Biomedical subjects

M A Díaz

Publications and source records attributed to M A Díaz.

At least 19 recordsLinked to original sources
PubMed2005

Long-term outcome of allogeneic or autologous haemopoietic cell transplantation for acute lymphoblastic leukaemia in second remission in children. GETMON experience 1983-1998.

We present a retrospective study of long-term outcome and predictive factors of survival and relapse in 219 paediatric patients with acute lymphoblastic leukaemia (ALL) in second remission. They received allogeneic (allo) or autologous (auto) haemopoietic cell transplantation (HCT) depending on the availability of a matched sibling donor. The probability of event-free survival (EFS) for the total patient group was 0.35+0.03 at 14 years. No significant differences were observed for EFS between allo- and auto-HCT: 0.39+0.05 vs 0.32+0.04 (P=0.43). A better EFS was seen in patients with a late relapse (LR) (P=0.06 and 0.02, for allogeneic and autologous respectively). Significantly better EFS was observed in allo-HCT patients under 10 years of age and in auto-HCT patients with leukocytes at diagnosis below 25 x 109/l and late relapse. Predictive factors of failure in both groups were early relapse (ER), medullary relapse and age over 10 years. The probability of relapse (RP) for the total group of patients was 0.57+0.03, and it was significantly higher in auto-HCT patients: 0.65+0.04 vs 0.42+0.06 (P=0.002). Factors predictive for relapse were medullary and early relapse, auto-HCT and WBC >25 x 109/l at diagnosis.

Child↗
PubMed2004

Engraftment syndrome after autologous peripheral blood progenitor cell transplantation in pediatric patients: a prospective evaluation of risk factors and outcome.

We prospectively analyzed the incidence, risk factors and outcome of engraftment syndrome (ES) in 112 patients undergoing autologous peripheral blood progenitor cell transplantation with different malignancies between January 1999 and December 2003. The median age was 8 years (range 1-18). There were 73 males. There were 37 hematological neoplasias and 75 solid tumors. Disease status at transplantation was early in 49, intermediate in 15 and 48 in advanced phase. The median CD34+ cells infused was 4.6 x 10(6)/kg. With a median follow-up of 23 months (1-116 months), 38 patients developed ES. The cumulative incidence of ES was 34.5 +/- 4.5% and the event-free survival was 58.3 +/- 12%. There were no differences in the causes of death between patients with or without ES. A high number of CD34+ cells/kg infused, patients transplanted in early phase, the type of malignancy (solid tumor) and conditioning regimens other than busulfan based were significantly associated with ES in a multivariate analysis.

Adolescent↗
PubMed2004

A prospective randomized study of clinical and economic consequences of using G-CSF following autologous peripheral blood progenitor cell (PBPC) transplantation in children.

This prospective and randomized study was conducted to evaluate clinical and economic consequences of using granulocyte colony-stimulating factor (G-CSF) following autologous peripheral blood progenitor cell (PBPC) transplantation in children. Between January 1999 and December 2003, 117 patients underwent autologous PBPCT: 51 patients received G-CSF following PBPCT, while 66 patients did not receive G-CSF. Median time to absolute neutrophil count > 0.5 x 10(9)/l was 10 days in the treatment group and 11 days in the control group (P < 0.009). The median time to platelets >20 x 10(9)/l was 12 days in both groups (P = NS). The median time to platelets >50 x 10(9)/l was 15 days in the G-CSF group and 14 days in the control group (P<0.005). In patients who received <5 x 10(6)/kg CD34+ cells, the median time to platelets >20 x 10(9)/l and >50 x 10(9)/l was similar with or without G-CSF (12 and 15 days, respectively). Platelet transfusion requirements were lower in the control group (2 vs 3 U in G-CSF group). There was a trend towards higher total costs with G-CSF: 8146.82 Euros and 7873.34 Euros with and without G-CSF, respectively (P = 0.1). Our data suggest that there is no indication of the standard application of G-CSF in children following PBPC transplantation. The only possible indication is the group of patients with a lower yield of CD34+ cells.

Adolescent↗
PubMed2002

Engraftment syndrome in children undergoing autologous peripheral blood progenitor cell transplantation.

There is limited experience on engraftment syndrome (ES) in children. The present study analyzes the characteristics of ES in pediatric patients undergoing autologous peripheral blood progenitor cells transplantation (PBPCT). From 1993 to 2001, 30 of 156 patients (19.2%) who underwent PBPCT developed ES (skin rash which involved more than 27% of the body surface and temperature >38.3 degrees C with no compatible infectious disease etiology, during neutrophil recovery). Of the 30 patients who developed ES, 20 (66%) developed hypoxia and/or pulmonary infiltrates, seven (23%) had hepatic dysfunction, six (20%) developed renal insufficiency, 16 (53%) showed weight gain and three (10%) experienced transient encephalopathy. Multivariate analysis showed that the only positive predictive factor for developing ES was mobilization with high-dose G-CSF (12 microg/kg twice daily) (RR 3.88, CI 95% 1.73-8.67; P < 0.0005). The overall transplant-related mortality (TRM) was 8.33% and this was significantly higher in the patients who developed ES than in those who did not (23% vs 4.76%; P < 0.0001). We also found a higher morbidity in patients who developed ES, expressed as a statistically significant increase in supportive care (transfusion requirement, parenteral nutrition) and increase in the length of hospital stay. In summary, we have found ES to be the most important cause of morbidity and mortality in children undergoing autologous PBPCT.

Adolescent↗
PubMed2002

Granulocyte colony-stimulating factor alone at 12 microg/kg twice a day for 4 days for peripheral blood progenitor cell priming in pediatric patients.

In children, the optimal mobilization schedule for harvesting peripheral blood progenitor cells (PBPC) is an issue of continuous research. We have studied a schedule based on high and daily divided doses of G-CSF (12 microg/kg body weight twice daily) for 4 days for PBPC priming. Toxicity related to G-CSF was observed in 13 patients (23%), mainly mild bone pain and myalgia. The median CD34(+)cell number collected was 4.4 (0.4-35 x 10(6)/kg body weight), with 46 patients achieving 2 x 10(6)/kg body weight (83.6%) after a single large volume leukapheresis. In conclusion, this mobilization schedule allows safe and efficient collection of the minimum target CD34(+) cell dose in most pediatric patients by only one procedure.

Adolescent↗
PubMed2001

"Pseudotumor cerebri" following allogeneic bone marrow transplantation (BMT).

Pseudotumor cerebri, an uncommon complication following BMT, has been generally associated with cyclosporin A neurotoxicity. However, it has not previously been reported as a clinical presentation of sinusitis in spite of its high incidence after BMT. We report a case of pseudotumor cerebri secondary to sinusitis in a child with acute lymphoblastic leukemia and who underwent unrelated bone marrow transplantation.

Bone Marrow Transplantation↗
PubMed2001

Acute autoimmune hemolytic anemia following unrelated cord blood transplantation as an early manifestation of chronic graft-versus-host disease.

A 16-month-old girl diagnosed with osteopetrosis underwent an unrelated, partially matched (with major mismatch at A locus) cord blood stem cell transplant. Twelve months later she developed severe acute autoimmune hemolytic anemia (AIHA). Immunophenotype analysis of lymphocyte subsets 8 months post transplant showed a low number of T lymphocytes, with normal subsets, and with NK cells and B lymphocytes within normal ranges. When the hemolytic anemia developed, the lymphocytes subsets changed and analysis showed higher numbers of B lymphocytes than previously, lower CD3+ T lymphocytes with inversion of the CD4/CD8 ratio and an abnormal proportion of T lymphocyte subsets. She was being treated with cyclosporine, and steroids and immunoglobulins were added. Initially the AIHA improved, but repeated infectious episodes led us to tail off the immunosuppressive treatment. The AIHA relapsed and cyclosporine was restarted. Currently, she is on cyclosporine and low-dose steroid treatment with no hemolytic features. During the 3 months when the AIHA was being treated, she developed extensive skin cGVHD and recurrent pneumothoraces. AIHA may be the first manifestation of abnormal reconstitution of immunity developing after a hematopoietic transplant. This abnormal reconstitution is also the basis of cGVHD. We suggest that aggressive immunosuppressive treatment with intensive measures against infection could give a better prognosis to such patients.

Acute Disease↗
PubMed2001

Favourable effect of the combination of acute and chronic graft-versus-host disease on the outcome of allogeneic peripheral blood stem cell transplantation for advanced haematological malignancies.

To assess the influence of graft-versus-host disease (GVHD) on the outcome of patients with advanced haematological malignancies (AHM) who received a primary, unmodified allogeneic peripheral blood progenitor cells transplant (allo-PBT) from a human leucocyte antigen (HLA) identical sibling donor, we analysed 136 patients with myeloid neoplasms (n = 70) or lymphoproliferative disorders (n = 66), transplanted at 19 Spanish institutions. Median age was 35 years (range 1-61). The cumulative incidence of relapse for all patients was 34% (95% CI, 26-42%), 41% (95% CI, 33-49) for patients without GVHD and 14% (95% CI, 3-25) (P = 0.001) for patients with acute and chronic GVHD. After a median follow-up of 11 months (range 2-49), 60 (44%) patients remained alive with an actuarial probability of overall survival and disease-free survival (DFS) at 30 months of 31% (95% CI, 21-41%) and 28% (95% CI, 17-39%) respectively. In patients surviving > 100 d, the low incidence of relapse in those with acute and chronic GVHD led to a DFS of 57% (95% CI, 38-76%) compared with a DFS of 34% (95% CI, 17-51%) in the remaining patients (P = 0.03). Our results indicate a reduced incidence of relapse for patients with AHM receiving an unmodified allo-PBT and developing acute and chronic GVHD, which results in an improved DFS.

Acute Disease↗
PubMed2001

Donor age-related differences in PBPC mobilization with rHuG-CSF.

BACKGROUND: Data on the administration of rHuG-CSF to normal donors <18 years old are very limited. STUDY DESIGN AND METHODS: The results of rHuG-CSF administration to 61 donors <18 years old (Group A) were retrospectively evaluated and compared with results from 353 donors > or = 18 years old (Group B) who are included in the Spanish National Donor Registry. The mean age (range) in Group A and B was 14 (1-17) and 38 (18-71) years, respectively (p<0.001). The mean dose of rHuG-CSF was 10 microg per kg per day (range, 9-16) during a mean of 5 days (range, 4-6). Central venous access was placed more frequently in younger donors (25% vs. 6%; p<0.001). RESULTS: The mean number of CD34+ cells collected was 7.6 and 6.9 x 10(6) per kg of donor's body weight in Group A and B, respectively. Fifty-six percent of Group A donors needed only one apheresis to achieve > or = 4 x 10(6) CD34+ cells per kg versus 39 percent of Group B donors (p = 0.01). Side effects were more common in Group B (71% vs. 41%; p<0.001). CONCLUSION: The administration of rHuG-CSF to donors <18 years old leads to CD34+ cell mobilization in a pattern similar to that observed in adults. Greater age was associated with a more frequent requirement for more than one apheresis to achieve a similar number of CD34+ cells.

Adolescent↗
PubMed2001

Ki-1+ anaplastic large cell lymphoma in a child with unpredictable clinical course.

Ki-1+ anaplastic large cell lymphoma (Ki-1+ ALCL) is a subtype of non-Hodgkin lymphoma (NHL) with defined histopathological characteristics but with highly variable clinical presentation and outcome. Although in most of the patients the disease behaves as an intermediate- or high-grade lymphoma, some patients present with an indolent clinical course. Factors that determine the clinical behavior of this lymphoma have not yet been identified. A case is reported of a 13-year-old girl who initially presented with Ki-1+ ALCL but later developed recurrent localized cutaneous disease and followed a clinical course similar to that of a low-grade lymphoma.

Adolescent↗
PubMed2001

Cloning, nucleotide sequence, and expression of the Brucella melitensis sucB gene coding for an immunogenic dihydrolipoamide succinyltransferase homologous protein.

The Brucella melitensis sucB gene encoding the dihydrolipoamide succinyltransferase (E2o) enzyme (previously identified as an immunogenic protein in infected sheep) was cloned and sequenced. The amino acid sequence predicted from the cloned gene revealed 88.8 and 51.2% identity to the dihydrolipoamide succinyltransferase SucB protein from Brucella abortus and Escherichia coli, respectively. Sera from naturally infected sheep showed antibody reactivity against the recombinant SucB protein.

Acyltransferases↗
PubMed2000

High survival rate in infant acute leukemia treated with early high-dose chemotherapy and stem-cell support. Groupo Español de Trasplante de Médula Osea en Niños.

PURPOSE: Infants with acute leukemia have a poor prognosis when treated with conventional chemotherapy. It is still unknown if stem-cell transplantation (SCT) can improve the outcome of these patients. In the present study, we review our experience with SCT in infant acute leukemia to clarify this issue. PATIENTS AND METHODS: We report the results of 26 infants who were submitted to a SCT for acute leukemia. There were 15 cases of acute myeloid leukemia and 10 cases of acute lymphoid leukemia. One patient had a bilineal leukemia. Twenty-two patients were in their first complete response (CR1), three were in their second CR, and one was in relapse. Eight patients were submitted to allogeneic SCT, and 18 underwent autologous SCT. RESULTS: With a median follow-up of 67 months, the 5-year overall survival and disease-free survival (DFS) are 64% (SE = 9%) and 63% (SE = 10%), respectively. Autologous and allogeneic SCT offered similar outcome. There was not any transplant-related mortality, and all deaths were caused by relapse in the first 6 months after SCT. In multivariate analysis, the single factor associated with better DFS was an interval between CR1 and SCT of less than 4 months (P: <.025). CONCLUSION: SCT is a valid option in the treatment of infant acute leukemia, and it may overcome the high risk of relapse with conventional chemotherapy showing very reduced toxicity. This study suggests that SCT should be performed in CR1 in the early phase of the disease.

Acute Disease↗
PubMed2000

Influence of the food matrix structure on the retention of aroma compounds.

The retention of the aroma compounds in a multicomponent medium like the food matrix is influenced by their affinity with the protein when lipid is present at a low level (0.5%). The effect of the structure of the media is also studied by using two media with the same composition; one was emusified, and the other was not. Among the studied aroma compounds, 2-nonanone and isoamyl acetate present opposite behaviors: the volatility of isoamyl acetate is not affected by the change of the medium structure whereas that of 2-nonanone increases. The decrease of retention of 2-nonanone in an emulsified system would be due to a modification of the fixation site for this compound on the protein or to a competition between the lipid and the aroma compound while the protein is adsorbed at the lipid-water interface.

Food↗
PubMed2000

Clinical and economic comparison of allogeneic peripheral blood progenitor cell and bone marrow transplantation for acute lymphoblastic leukemia in children.

There is limited experience in the use of peripheral blood progenitor cells (PBPC) for allogeneic transplantation in children. In the present study we compared engraftment kinetics, incidence of acute and chronic graft-versus-host disease (GVHD) and the outcome and economic costs of allogeneic PBPCT vs BMT in children with ALL in a single institution. All children were transplanted in complete remission (CR) with a similar conditioning regimen and the same GVHD prophylaxis. Patients undergoing PBPCT achieved myeloid and platelet engraftment before patients undergoing BMT (P < 0.001). Platelet recovery was faster for the PBPCT group (P < 0.014 for 50 x 10(9)/l and P < 0.039 for 100 x 10(9)/l). Incidence and severity of acute and chronic GVHD were similar in both groups (acute grade 1-2: 9/13 for PBPCT vs 9/11 for BMT; chronic GVHD: 5/12 for PBPCT vs 3/8 for BMT). Hospital stay was shorter for the PBPCT than for the BMT group (28.8 days vs 42.9 days, respectively) and the PBPCT group used less clinical resources, resulting in overall lower cost for PBPCT (US $14,046) compared to BMT (US $19,840). There was no statistically significant difference in DFS between PBPCT and BMT (68.4% vs 50%, respectively).

Bone Marrow Transplantation↗
PubMed2000

Use of concurrent G-CSF + GM-CSF vs G-CSF alone for mobilization of peripheral blood stem cells in children with malignant disease.

There is limited experience in the mobilization of peripheral blood progenitor cells (PBPC) in children and the optimal method for PBPC mobilization is unknown. The present study was conducted to ascertain whether mobilization with G-CSF + GM-CSF (group I) provides some advantage over G-CSF alone (group II) in terms of collected CD34+ cells and hematopoietic recovery following myeloablative conditioning in children with malignancies. An economic analysis was also performed. Each group comprised 21 consecutive patients. The mean number of aphereses was 1.5+/-0.5 in group I and 1.2+/-0.46 in group II (NS). The mean number of CD34+ cells was 3.8 x 106+/-4.03/kg in group I and 4.2+/-5.4 in group II (NS). The mean number of total blood volumes (TBV) processed was 4.4+/-1.5 in group I and 4.3+/-1.5 in group II (NS). The mean duration of the procedure was 276+/-74.1 min in group I and 286.7+/-75.9 min in group II (NS), and the inlet flow was 45.1+/-12 ml/min in group I and 39.5+/-15.1 ml/min in group II (NS). No significant differences in the neutrophil and platelet engraftment probability were observed between the two groups. The mean overall cost of group II was not statistically significant from that of group I (US$ 9521+/-330 vs US$ 10201+/-1028, P = NS). The cost of mobilization was significantly higher in group I than in group II, conditioning regimen costs were similar in both groups and the costs related to the post-transplant period were similar in both groups. We conclude that PBPC mobilization with G-CSF + GM-CSF in children does not enhance hematological recovery in comparison with mobilization using G-CSF alone. However, the combination of G-CSF + GM-CSF does not significantly increase the overall cost of transplantation.

Adolescent↗
PubMed2000

Using peripheral blood progenitor cells (PBPC) for transplantation in pediatric patients: a state-of-the-art review.

This paper presents a state-of-the-art review of using mobilized-peripheral blood progenitor cells (PBPC) for transplantation in children. Our own data and those from Medline searches and meeting reports, are analyzed and presented for the different sections that involve transplantation. Recommendations concerning the choice of mobilization regimens, venous access, priming of separator extracorporeal line, anticoagulation, and number of CD34+ cells to infuse for rapid engraftment are proposed. In the allogeneic setting, we analyze ethical and safety aspects of pediatric donor mobilization and collection. Data from the literature suggest that the use of cytokine-mobilized PBPC for allogeneic transplantation appears to be safe both for pediatric donors and patients leading a rapid hematopoietic engraftment with a similar incidence of acute graft-versus-host disease (GVHD). The high incidence of chronic GVHD and its management emerge as the most concerning aspect in allogeneic PBPC transplantation.

Child↗