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Biomedical subjects

T Miale

Publications and source records attributed to T Miale.

14 recordsLinked to original sources
PubMed1995

Allogeneic marrow grafts from donors with congenital chromosomal abnormalities in marrow cells.

To determine whether siblings with chromosomal abnormalities in marrow cells which are associated with cellular defects (e.g. Down syndrome or heterozygosity for Fanconi syndrome) are suitable donors for allogeneic bone marrow transplants, we have reviewed the patient files at the Fred Hutchinson Cancer Research Center (FHCRC) and carried out a survey among member centres of the International Bone Marrow Transplant Registry (IBMTR). The 57 of 253 (23%) member centres which responded to the survey reported seven transplants from donors with the following conditions: Down syndrome (n = 2), suspected heterozygotes for Fanconi syndrome (n = 3), and 47,XXX syndrome (n = 2), among a total of 5,561 allogeneic transplants from HLA-identical siblings. Adding the three cases seen at the Fed Hutchinson Cancer Research Center among 2,927 HLA-identical sibling transplants during 1992 resulted in 10 transplants among 8,488 cases transplanted overall: four with Down syndrome, four suspected of being heterozygous for Fanconi syndrome, and two trisomy X. Three out of four grafts from siblings with Down syndrome had complications, including poor graft function (n = 2) and graft failure (n = 1). Two of four recipients of marrow from presumed Fanconi syndrome heterozygotes presented with poor graft function and a third recipient developed graft failure after initial evidence of engraftment. The two patients given marrow from siblings with 47,XXX syndrome engrafted uneventfully. The experience reported here shows a low frequency of encountering an HLA-identical sibling donor who has chromosomal abnormalities in marrow cells consistent with Down syndrome or heterozygosity for Fanconi syndrome, about one case among 1,000 transplants. The much higher than expected incidence of graft problems with marrow from such a donor would make it reasonable to look either for an alternative marrow donor or consider an autologous transplant, in case a sibling marrow donor with Down syndrome or heterozygosity for Fanconi syndrome is encountered, although a donor with trisomy X seems acceptable.

Adolescent↗
PubMed1987

Difficulty in establishing diagnosis from lung biopsies and bronchial washing analysis in children with leukemia following bone marrow transplantation.

Three children developed severe respiratory distress at days +12, +11, and +11 following allogeneic bone marrow transplantation from donors. The first child was a 13-year-old Hispanic boy transplanted in relapse of Philadelphia chromosome-positive acute lymphoblastic leukemia (ALL). At day -14, a bronchial washing done for a streaky pulmonary infiltrate was negative for acid-fast bacilli. Miliary tuberculosis was discovered at postmortem examination. A second child, transplanted in remission of null-cell ALL, developed severe hypoxia and hypercarbia on day +11 but recovered fully following prolonged mechanical ventilation. An open-lung biopsy showed a pattern of nonspecific, diffuse alveolar damage compatible with respiratory distress syndrome. The third child was transplanted in remission of B-cell ALL and developed fatal fungal and cytomegalovirus pneumonia on day +12. In these latter two cases, it is likely that open-lung biopsy would have missed the diagnosis because of the uneven pulmonary involvement and multiple etiologies observed. All three children received cyclosporine, granulocyte transfusions, and multiple antimicrobials, including amphotericin B. Hyperfractioned total-body irradiation with lung shielding was used in the latter two patients.

Adolescent↗
PubMed1983

Hereditary ring sideroblastic anaemia and Christmas disease in a Swedish family.

The association of hereditary ring sideroblastic anaemia with Christmas disease in a Swedish family is described. We have studied the transmission of the sideroblastic trait, in relation to HLA groups and Christmas disease, and also evaluated the erythrocyte morphology, uroporphyrinogen-I-synthetase activity and S-ferritin for the detection of latent cases of ring sideroblastic anaemia. The proband had ring sideroblastic anaemia, Christmas disease and haemochromatosis. 3 cases of ring sideroblastic anaemia were found among the 12 family members studied. Using the factor IX deficiency as a marker of the X chromosome, it appeared that autosomal transmission of the sideroblastic trait was most likely. The sideroblastic trait did not seem to be linked to HLA-A3-alloantigen. Erythrocyte morphology was normal in all non-anaemic subjects. S-ferritin was found to be increased in all 3 cases of sideroblastic anaemia as well as in 1 non-anaemic relative. Erythrocyte uroporphyrinogen-I-synthetase was elevated in 10 of the 12 family members; those with sideroblastic anaemia had the highest values indicating that uroporphyrinogen-I-synthetase is of importance in the disturbed haem-synthesis of ring sideroblastic anaemia. This interpretation is supported by the positive correlation between S-ferritin values and the uroporphyrinogen-I-synthetase activity.

Anemia, Sideroblastic↗
PubMed1980

Adjuvant chemotherapy in nonmetastatic osteosarcoma: a Southwest Oncology Group Study.

This report updates experience with the CONPADRI-I, COMPADRI-II, and COMPADRI-III adjuvant chemotherapy programs for the treatment of nonmetastatic osteosarcoma. A total of 200 patients received one of the three regimens. The analysis of response to treatment is based on disease-free survival time (DFS time). The effect of treatment, age, sex, site of disease involvement, and race on DFS time were investigated. Cox's life-table regression analysis found only sex to have a significant effect on DFS time with males having 1.8 times the risk of recurrence or death per unit time as female patients (P = 0.004). An analysis of the 81 patients still alive and disease-free 18 months after the start of treatment shows significantly longer DFS time for CONPADRI-I than COMPADRI-II patients (P = 0.01). This trend is true for both male (P = 0.12) and female (P = 0.08) patients.

Adolescent↗
PubMed1979

Long-term survival in childhood acute leukemia: "late" relapses.

The "late" relapse patterns of childhood acute leukemia were studied in 83 children in their first continuous complete remission for more than three years prior to randomization for stopping therapy (40 patients) or continuing therapy (43 patients) for a total of six years. Twenty of 83 (22.9%) have relapsed: Ten in the bone marrow, one in the central nervous system, and nine in the testes. The testes relapse rate of 41.1% (7/17) in males discontinuing therapy at three years was much higher than that of 8.7% (2/23) in males continuing therapy. This difference is significant at P = 0.01 (Wilcoxon test).

Bone Neoplasms↗
PubMed1977

Evaluation of a MOPP-type regimen in histiocytosis X--a Southwest Oncology Group study.

The overall 38% response rate in histiocytosis X for cyclophosphamide, vincristine (Oncovin), prednisone, and procarbazine was not superior to responses that can be achieved with the component single agents. This is in contrast to the higher response rates that can be achieved by the combination of these agents in Hodgkin's disease. This is the second study of combination chemotherapy with individually effective agents that has failed to improve the response rates in histiocytosis patients with poor prognostic indicators.

Child↗
PubMed1976

Polyamine concentrations in bone marrow aspirates of children with leukemia and other malignancies.

High-pressure liquid chromatography analysis of polyamines in bone narrow from leukemic and nonleukemic subjects demonstrated increased concentrations of putrescine, spermidine, and spermine associated with increased cellularity. The most striking abnormality was the marked elevation of putrescine. Bone marrow polyamine analysis may be an adjunct for evaluation of leukemia patients.

Bone Marrow↗
PubMedDate not supplied

Changes in urine polyamines in childhood leukemias.

Urine polyamine:creatinine ratios (nm per mg) measured in eight children with active hematologic cancers were compared with those of age-matched controls and children with hematologic cancer in remission. Polyamine:creatinine ratios in the children with active disease were significantly higher than those of the controls (p less than 0.0025) and of the children in remission (p less than 0.0025). Putrescine:creatinine ratios were, in general, higher in children with hematologic tumors than in those with solid tumors. Urinary polyamines are thought to reflect variations in bone marrow polyamine content and have been postulated to be an indicator of clinical status.

Adolescent↗
PubMedDate not supplied

Cyclocytidine in the treatment of refractory acute childhood leukemia: a Southwest Oncology Group Phase I-II study.

Thirty-seven children and adolescents with acute leukemia in relapse were treated with cyclocytidine in a cooperative group setting. Only one of the 27 evaluable patients achieved complete remission. A significant decrease (greater than or equal to 20%) in circulating and/or bone marrow leukemia cells occurred in an additional five patients. Drug toxicity was evaluated in 35 patients and included ten cases of jaw pain, two cases of hypotension, one case of fever, and one case of severe vomiting.

Adolescent↗