Search PubMed⌕ Search

Biomedical subjects

R Storb

Publications and source records attributed to R Storb.

At least 559 records · Page 31Linked to original sources
PubMed1984

Allogeneic marrow transplantation in the treatment of preleukemia.

Ten patients with preleukemia and life-threatening pancytopenia were treated with marrow transplantation. In all ten cases allogeneic marrow was successfully engrafted. In three patients prepared for transplantation with cyclophosphamide alone, the abnormal cell clone either persisted or reemerged within 6 months of transplantation. The other seven patients were treated with cyclophosphamide plus total body irradiation before transplantation and six of the seven are alive and well without evidence of disease from 7 to 25 months after transplantation. These results suggest that cyclophosphamide alone before marrow transplantation is incapable of eradicating the abnormal clone. Cyclophosphamide combined with total body irradiation appears more effective, although more time is needed for full evaluation of results. This experience also emphasizes the importance of performing chromosome studies before transplantation for pancytopenic states in order to identify cases of preleukemia.

Adolescent↗
PubMed1984

Long-term survival after marrow transplantation for paroxysmal nocturnal hemoglobinuria with aplastic anemia.

Four patients with paroxysmal nocturnal hemoglobinuria and severe marrow aplasia were given marrow grafts either from allogeneic human-leukocyte-antigen-identical siblings (three patients) or from a syngeneic donor (one patient). The patients with allogeneic grafts were conditioned with regimens that included cyclophosphamide and had sustained and complete marrow engraftment; subsequent tests were negative for paroxysmal nocturnal hemoglobinuria. One patient developed chronic graft-versus-host disease that resolved over 4 years. The patient receiving a syngeneic graft received marrow infusion without preceding immunosuppression. He had prompt engraftment, and hematologic variables returned to normal. A Ham's test done at 3 years was negative, but a complement lysis sensitivity test done 10 years after grafting was positive; the patient, however, remains asymptomatic. All four patients are alive and well 4, 9, 10, and 12 years after transplantation. Paroxysmal nocturnal hemoglobinuria apparently can be treated successfully by allogeneic or syngeneic marrow transplantation without subsequent maintenance therapy.

Adolescent↗
PubMed1984

Cellular interactions in marrow-grafted patients. II. Normal monocyte antigen-presenting and defective T-cell-proliferative functions early after grafting and during chronic graft-versus-host disease.

The proliferation of T cells of marrow donor origin in response to Escherichia coli, an ubiquitous antigen, presented by circulating monocytes of marrow donor origin was investigated in 30 human allogeneic marrow transplant recipients. Compared with cells from healthy marrow donors, T cell proliferation was found to be deficient in all recipients studied 36-71 days after grafting, regardless of the presence or absence of acute graft-versus-host disease and in most patients with chronic graft-versus-host disease studied 118-1804 days postgrafting . In contrast, lymphocytes from most long-term patients without chronic graft-versus-host disease studied 363-2673 days had immune reactivity comparable to that of lymphocytes from their marrow donors. Results of cell-mixing experiments showed that (1) monocytes from most marrow recipients were capable of presenting antigens to normal T cells of marrow donors, and (2) T cells from short-term patients and from long-term patients with active chronic graft-versus-host disease were not induced to proliferate by E-coli-pulsed monocytes from the marrow donors. This inability of T cells to proliferate was likely the result of ineffective interactions among T cell subsets.

Adolescent↗
PubMed1984

Bone marrow transplantation: a review of delayed complications.

In one and one-half decades, marrow transplantation has become a life-saving procedure for an ever-increasing number of patients. With continued observation, however, it became apparent that marrow transplantation as used presently is associated with significant acute, delayed, and chronic toxicity. These complications may be related to chemo- or radiotherapy used in conditioning for transplantation, to immunosuppressive treatment given after transplantation and to the immunodeficiency associated with transplantation and the development of GVHD. Attempts need to be made to develop less toxic conditioning regimens. Most importantly, however, we need to learn more about mechanisms of graft-host interaction in order to eliminate the problem of GVHD and to accelerate the recovery of immunocompetence in allogeneic chimaeras.

Adolescent↗
PubMed1984

Therapy of severe aplastic anemia with anti-human thymocyte globulin and androgens: the effect of HLA-haploidentical marrow infusion.

Fifty-four patients with severe aplastic anemia were treated with horse anti-human thymocyte globulin (ATG) and androgens. Thirty of these patients also received an infusion of HLA-haploidentical marrow cells. Only those patients having evidence of hematologic recovery within 3 mo after ATG therapy were considered responders to the immunosuppressive regimen. Of 53 patients evaluable for response, 21 had complete or partial responses and 7 had minimal improvement by defined criteria. The remaining patients did not respond or died. Factors correlated with response to therapy included a short duration of aplasia and a high admission granulocyte count. Thirty-six patients (66.7%) are surviving between 18 and 43 mo, and 18 have died. Deaths were due to hemorrhage and/or infection. Short duration of aplasia and high granulocyte counts also correlated with survival, as did younger age. Four patients with complete or partial responses had a recurrence of severe aplasia 6-17 mo after their first course of ATG. Three of these patients were retreated with ATG (and oxymetholone in two cases). All three had second responses to therapy, but two of the three have had second relapses. The fourth patient responded to oxymetholone alone, but died after a second relapse. Mismatched marrow infusion had no effect on the incidence of response or survival.

Adolescent↗
PubMed1984

Rapidly progressive air-flow obstruction in marrow transplant recipients. Possible association between obliterative bronchiolitis and chronic graft-versus-host disease.

Severe obstructive airways disease developed in 4 young nonsmoking adults after marrow transplantation. They were free of respiratory disease until symptoms developed 277 to 600 days after transplant. Pulmonary function testing showed that the mean forced expiratory volume in one second was 35% of predicted (range, 23 to 49%). All patients had active or inactive extensive chronic graft-versus-host disease that included oral mucositis, esophagitis, sinusitis, and oral and ocular sicca. Three patients had subnormal serum IgA levels. Bronchitis was apparent during fiberoptic bronchoscopy in 3 patients. An open-lung biopsy specimen from 1 patient showed obliterative bronchiolitis. Treatment has included bronchodilators and corticosteroids without objective benefit. The disorder stabilized in all 4 patients, but a severe reduction in air flow persisted. Awareness of this complication may lead to earlier diagnosis and more effective treatment.

Adolescent↗
PubMed1984

Immunoglobulin synthesis after HLA-identical marrow grafting. V. The role of peripheral blood monocytes in the regulation of in vitro immunoglobulin secretion stimulated by pokeweed mitogen.

The ability of purified monocytes to regulate in vitro immunoglobulin (Ig) production was examined in 12 patients after HLA-identical marrow grafting. Five patients were studied less than 3 mo after grafting and seven more than 1 yr after grafting. One of the former had acute graft-vs-host disease and five of the latter had chronic graft-vs-host disease. Ficoll-Hypaque-separated peripheral blood mononuclear cells from patients, normal marrow donors, or healthy unrelated individuals were separated into T and non-T cells by sheep erythrocyte rosetting. Highly enriched monocyte and B cell subpopulations were obtained by placing the non-T cells over discontinuous Percoll gradients. Co-cultures of patient or normal monocyte populations with either normal or patient T and B cells with pokeweed mitogen were performed. A hemolytic plaque assay was used to assess Ig secretion after 6 days of culture. Co-culture of T and non-T cells from 10 of 12 patients failed to produce Ig. Monocyte-enriched fractions from all patients provided normal accessory cell functions when co-cultured with normal T and B cells. Two of five patients with chronic graft-vs-host disease had monocytes that suppressed Ig synthesis at high ratios of monocytes to normal T and B cells. Normal monocyte-enriched fractions did not restore Ig production to T and B cells of patients whose T and non-T cells failed to produce Ig. These data indicate that the observed defects in pokeweed mitogen-driven Ig secretion after marrow grafting are due primarily to defective T and B cell functions and that the monocyte accessory function is intact in most patients studied.

Adolescent↗
PubMed1984

Oral manifestations of chronic graft-v-host disease.

Sixty patients were studied 180 to 500 days after allogeneic marrow transplantation to determine if late oral abnormalities were associated with the presence of chronic graft-v-host disease (GVHD). Lip and intraoral mucosal surfaces were evaluated for color, keratinization, atrophy, and erythema. Subjective complaints of oral pain and xerostomia were also recorded. Abnormalities were scored on a scale of 0 to 3 and tested for association with GVHD by chi 2 test. Oral manifestations most strongly associated with chronic GVHD included atrophy and erythema or lichenoid lesions of the buccal and labial mucosa and oral pain. Oral manifestations resembled several naturally occurring autoimmune disorders. Recognition of these changes can aid in the clinical diagnosis and assessment of established chronic GVHD.

Adolescent↗