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Biomedical subjects

R Storb

Publications and source records attributed to R Storb.

At least 685 records · Page 38Linked to original sources
PubMed1981

Allogeneic bone marrow transplantation for chronic granulocytic leukemia.

Eighteen patients with chronic granulocytic leukemia underwent allogeneic marrow transplantation from HLA-identical sibling donors. The preparative regimen included cyclophosphamide and 1000-1500 rad total body irradiation in either single or fractionated doses. Eleven patients were transplanted in blast crisis. One died too early to evaluate. Five had recurrent leukemia, three died of interstitial pneumonia (IP), and two are living in remission after 20 and 39 months. One additional patient with blast crisis was transplanted while in remission after chemotherapy and is living in remission 28 months after transplantation. Two patients were transplanted in the accelerated phase; one died early of infection and one died of IP. Four were transplanted in the chronic phase; one died of IP, one with graft-versus-host disease, and two are living in remission 11 and 25 months after transplantation.

Adolescent↗
PubMed1981

Treatment of severe aplastic anemia using antithymocyte globulin with or without an infusion of HLA haploidentical marrow.

Nineteen patients with severe aplastic anemia were treated with a 4-day course of horse-anti-human thymocyte globulin (ATG). Thirteen of these patients also received an infusion of HLA one haplotype-identical bone marrow. Toxicity of ATG included fever, chills, rash, arthralgias and elevated liver function tests. Platelet transfusion requirements increased during therapy. Eleven patients died 0.2-9.4 months after beginning ATG therapy. None of the 11 patients had any improvement in hematologic status prior to death. The eight surviving patients have been followed for at least 24 months. Six had evidence of hematologic improvement within 6-8 weeks after ATG therapy and are transfusion-independent. The other two patients improved more than one year after treatment. Survival after ATG therapy did not correlate with the presumed etiology of aplasia, duration of aplasia, patient age or sex, prior therapy, or admission granulocyte count. Addition of bone marrow infusion to ATG treatment also did not affect survival. This study demonstrated the necessity for a randomized trial of ATG versus supportive care alone for the treatment of severe aplastic anemia.

Adolescent↗
PubMed1981

Platelet and fibrinogen kinetics in canine tumors.

Fifty-three dogs with spontaneously occurring tumors were evaluated for abnormalities in the concentration and in vivo survival of platelets and fibrinogen. Thrombocytopenia occurred only in animals with extensive tumor involving spleen or marrow. Platelet survival was shortened in 6 of 15 (40%) dogs with localized tumor [mean 4.4 days +/- 0.3 (S.E.); normal 5.4 days +/- 0.1] and 30 of 35 (80%) dogs with metastatic tumor (mean 3.2 days +/- 0.2). Platelet survival progressively shortened during studies performed in dogs with ongoing disease. Fibrinogen concentration was increased (mean 420 +/- 30 mg/dl) in 44 of 53 (83%) of tumor-bearing dogs (normal 210 +/- 10 mg/dl). Neither history nor extent of disease, including presence of hepatic metastases, appeared to influence fibrinogen concentration significantly. Fibrinogen survival was below the normal range in 3 of 15 (20%) dogs with localized tumor and in 9 of 34 (26%) dogs with metastatic tumors. Thus, platelet consumption appeared to be the most significant hemostatic abnormality in tumor-bearing dogs. This model may be useful in evaluating the efficiency of antithrombotic therapy in preventing tumor-related hemostatic abnormalities.

Adenocarcinoma↗
PubMed1980

Chronic graft-versus-host syndrome in man. A long-term clinicopathologic study of 20 Seattle patients.

This study of chronic graft-versus-host disease (GVHD) describes the clinical, pathologic and laboratory features, and the causes of morbidity and mortality in 20 patients who received allogeneic marrow transplants from HLA identical sibling donors. Chronic GVHD is a pleiotrophic syndrome with variability in the time of onset, organ systems involved and rate of progression. The clinical-pathologic features resemble an overlap of several collagen vascular diseases with frequent involvement of the skin, liver, eyes, mouth, upper respiratory tract, esophagus and less frequent involvement of the serosal surfaces, lower gastrointestinal tract and skeletal muscles. Major causes of morbidity are scleroderma with contractures and ulceration, dry eyes and mouth, pulmonary insufficiency and wasting. Chronic GVHD has features of immune dysregulation with elevated levels of eosinophils, circulating autoantibodies, hypergammaglobulinemia and plasmacytosis of viscera and lymph nodes. In this study, three patients had limited chronic GVHD with relatively favorable prognosis characterized by localized skin involvement and/or hepatic disease without chronic aggressive histology. Most patients, however, had extensive disease with a progressive course. Survival was largely determined by the presence or absence of serious recurrent bacterial infections. The over-all severity of disease was best assessed by using the Karnofsky performance rating.

Adolescent↗
PubMed1980

Varicella-zoster virus infection after marrow transplantation for aplastic anemia or leukemia.

Nearly one-half of marrow transplant recipients who survive at least 6 months develop varicella-zoster virus (VZV) infection. Of 92 cases studied, 82 occurred within the first 12 months after transplant. Only one patient had recurrent infection. Seventy-seven patients had herpes zoster, 22 with subsequent cutaneous dissemination, and 15 had varicella. The overall mortality rate was 8%, and all deaths occurred within 9 months of transplant. Twenty-six of 32 patients studied had significant rises in VZV antibody during recovery. Among patients with acute leukemia, those with syngeneic transplants had a significantly lower incidence of VZV infection than those with allogeneic transplants. Incidence was slightly, but not significantly, decreased among patients with aplastic anemia. In contrast to other infections, the incidence of VZV infection was not influenced by graft-versus-host disease or predicted by the results of dinitrochlorobenzene skin testing.

Adolescent↗
PubMed1980

Prediction of marrow graft rejection by the lymphocyte-mediated cytotoxicity and antibody-dependent cell-mediated cytotoxicity assays.

We previously reported on 26 patients with severe aplastic anemia who were studied using the lymphocyte-mediated cytotoxicity (LMC) and antibody-dependent cell-mediated cytotoxicity (ADCC) assays. Eighty-one additional patients have now been studied. Overall, lymphocytes of 47% of the patients in LMC and sera of 30% in ADCC were reactive against the lymphocytes of HLA-identical siblings. LMC reactivity was more frequent in patients studied less than 1 month after diagnosis, but the frequency of ADCC reactivity was unrelated to time after diagnosis. In patients receiving the basic cyclophosphamide (CY) regimen, LMC results correlated with rejection in patients receiving transplants greater than or equal to 1 month after diagnosis but not in patients receiving transplants less than 1 month after diagnosis. Reactivity in ADCC correlated with graft rejection regardless of the time between diagnosis and study. No correlation between results of either assay and graft outcome was found in patients who received conditioning regimens aimed at abrogating rejection including total body irradiation or addition of donor buffy coat cells to the marrow inoculum. The finding of reactivity in the LMC assay early after diagnosis and in 8 or 15 untransfused patients indicates that mechanisms in addition to transfusion-induced sensitization must be involved in LMC reactivity. This LMC reactivity may be an epiphenomenon secondary to the stem cell defect of aplastic anemia. Alternatively, this LMC reactivity may be involved in the etiology of aplastic anemia.

Antibody-Dependent Cell Cytotoxicity↗
PubMed1980

Marrow transplantation in thirty "untransfused" patients with severe aplastic anemia.

Thirty patients with severe aplastic anemia had no transfusions of blood products until just before marrow transplantation from HLA-identical family members. They were conditioned for grafting with cyclophosphamide, 50 mg/kg body weight on each of 4 successive days. All 30 had prompt initial marrow engraftment, which was sustained in 27. Twenty-five of the 30 are alive between 9 to 84 (median, 19.5) months. The actuarial projection of survival for 2 to 6 years is 75%. Twenty of the 25 surviving patients have no problems. Five have chronic graft-versus-host disease, resolving in two and active in three. Five patients died with infection or hemorrhage, four of whom had graft-versus-host disease. These data show that early transplantation should be carried out before transfusions are given for any patient with severe aplastic anemia who has an HLA-identical family member. If sensitization to minor transplantation antigens contained in blood products is avoided, the incidence of marrow-graft rejection will decrease, and survival will improve.

Adolescent↗
PubMed1980

Canine lymphocyte subpopulations.

Ten to 23% of cells in blood, lymph node and bone marrow from normal dogs formed rosettes with human erythrocytes, and 12-27% formed rosettes with erythrocyte-antibody-complement (EAC) complexes. In contrast, only 3% of thymocytes, and 1% of thoracic duct cells formed rosettes with human erythroyctes, and 0 and 15% respectively formed EAC rosettes. When peripheral blood mononuclear cells were separated by rosette sedimentation into populations depleted of, or enriched for, cells forming rosettes with human erythrocytes (H-RFC), the population depleted of H-RFC responded more vigorously to alloantigens in mixed leukocyte culture (MLC) (P < 0.01) and to the mitogens phytohemagglutinin (PHA) (P = 0.01) and concanavalin A (P = 0.01) than did the population enriched for H-RFC. Passage of peripheral blood mononuclear cells over nylon wool columns produced a nonadherent population depleted of H-RFC, EAC rosette-forming cells and cells binding surface immunoglobulin (SIg), while the adherent population was enriched for each of these markers. In 3 dogs 36%, 44% and 64% of adherent cells that formed rosettes with human erythrocytes also possessed SIg, suggesting that canine B cells form rosettes with human red cells. The nonadherent population showed a more vigorous response to alloantigens in MLC (P < 0.01) and to PHA (P < 0.05) than the adherent population, and also stimulated the growth of autologous erythroid colonies better than the adherent population (P = 0.02). A T cell rich population can thus be obtained from canine peripheral blood, but no specific marker for T cells has been identified. Specifically, the capacity to form rosettes with human red cells is not a marker for the canine T cell.

Animals↗
PubMed1980

Functional subsets of peripheral blood mononuclear cells in transfused dogs separated by size with the fluorescence activated cell sorter.

We studied the capability of the fluorescence activated cell sorter (FACS II) to separate, by size, functional subsets of peripheral blood mononuclear cells (PBMC) in normal and transfusion sensitized dogs. PBMC were analyzed for their effect on erythroid colony (EC) growth from marrow of the transfusion donor or unrelated dog. Data indicate that 1) in normal dogs a population of small PBMC which stimulate EC growth could be separated from larger PBMC which had no significant effect on EC, 2) in transfused dogs small PBMC also stimulated EC growth but the large PBMC significantly inhibited EC, and 3) following transfusion-induced sensitization there was an increased number of large PBMC in peripheral blood.

Animals↗
PubMed1980

Autoimmune and alloimmune phenomena in patients with aplastic anemia: cytotoxicity against autologous lymphocytes and lymphocytes from HLA identical siblings.

We have studied peripheral blood lymphocytes of 117 patients with severe aplastic anemia and 237 healthy individuals for reactivity against autologous lymphocytes and/or lymphocytes from HLA-identical siblings using a 51Cr release assay. Lymphocytes from 29% of the patients exhibited reactivity against their own lymphocytes, while only 3% of lymphocytes from normal individuals showed such reactivity. Lymphocytes from 49% of the patients showed reactivity against lymphocytes from their HLA-identical siblings compared to 4% of normal individuals. Correlation existed between allogeneic and autologous reactivities (p < 0.001), suggesting a common pathway for cytotoxicity. Both reactivities showed an association with extremely low granulocyte counts (p < 0.01) and an inability of the patient's mononulcear cells to stimulate in allogeneic mixed leukocyte culture (p < 0.05) as well as an inverse correlation with time from diagnosis (p < 0.001). At least two explanations exist for the transfusion-independent autologous and allogeneic cytotoxicities: (1) they could be epiphenomena secondary to the stem cell defect, such as the loss of a cell that suppresses (or regulates) naturally occurring cytotoxic cells, or (2) they may be involved in the cause of the disease.

Anemia, Aplastic↗
PubMed1980

Cell-mediated immunity to non-HLA antigens of the host by donor lymphocytes in patients with chronic graft-vs-host disease.

Forty-four patients with aplastic anemia or leukemia were given marrow grafts from siblings selected on the basis of HLA-A and -B identity and mutual nonreactivity of their lymphocytes in mixed leukocyte culture (MLC). Twenty-two to 1089 days after grafting, their lymphocytes (of donor origin) were tested for reactivity in MLC to lymphocytes from the host (cryopreserved before grafting), the marrow donor, and unrelated individuals. Lymphocytes from 14 of 22 long-term survivors with chronic graft-vs-host disease (GVHD) showed unidirectional reactivity in response to host lymphocytes manifested as high stimulation indices (SI) and high relative responses (RR). Lymphocytes from only 1 of 12 long-term survivors without chronic GVHD showed unidirectional reactivity to host lymphocytes. Statistical analysis showed that lymphocytes from patients with chronic GVHD displayed anti-host responses that were significantly higher than those of lymphocytes from either marrow donors (p < 0.001) or patients without GVHD (p = 0.03). Lymphocytes from 5 patients with and 5 without acute GVHD, tested shortly after marrow grafting, failed to show responses to host cells. The results are consistent with a participation of cell-mediated immunity of graft against host in chronic GVHD.

Bone Marrow↗