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Biomedical subjects

H P Burri

Publications and source records attributed to H P Burri.

16 recordsLinked to original sources

[New development in clinical bone marrow transplantation in leukemia].

39 clinical bone marrow transplants (BMT) for leukemia are described. In a historical control series of 18 patients in whom BMT was performed after all chemotherapeutic resources had been exhausted, there is only 1 long-term survivor (5.5%), 8 patients died from GvH reaction, 6 from interstitial pneumonia and 3 from recurrent leukemia. Since 1979 an attempt has been made to transplant patients under optimal conditions (1st complete remission) and cyclosporin-A (CyA) has been used for prophylaxis of GvH reaction instead of MTX. 11 patients were transplanted according to our original proposal (AML and ALL in first remission, CML in chronic phase): 10 have survived without evidence of leukemia (91%), 1 AML died in relapse. 10 patients were grafted in second or later remissions or early relapse: 5 have leukemia-free survival (50%), 1 is living with a relapse. In this group 3 deaths were due to recurrent leukemia and 1 to CMV-infection. In our experience BMT under optimal circumstances does not involve a risk of early mortality and the chances of recurrent leukemia are reduced. Severe or chronic GvH reaction is not seen under CyA. BMT is the treatment of choice for patients with histocompatible sibling donors.

Bone Marrow Transplantation↗

Treatment of severe aplastic anaemia with antilymphocyte globulin or bone-marrow transplantation.

Fifty-three patients with severe aplastic anaemia were admitted to this hospital between January 1976 and June 1980, of whom three arrived in terminal condition and died before treatment for their basic disease could be given. Thus 50 patients were treated and evaluated in a prospective study according to one protocol. Eighteen patients with an HLA-identical sibling underwent bone-marrow transplantation with the aim of achieving haematopoietic chimerism. Thirty-two patients without an HLA-identical sibling were given antilymphocyte globulin with or without an infusion of HLA-haplotype-identical marrow. All these 32 patients received low-dose androgens after the procedure. In the first group eight patients (44%) survived. In the two other groups, 22 patients survived (69%), of whom 20 were completely self-sustaining (63%). Engraftment and graft-versus-host disease did not occur in the group who received antilymphocyte globulin and haploidentical marrow, and the haematopoietic reconstitutions in these patients were all autologous. These results confirm the efficacy of antilymphocyte globulin in the treatment of severe aplastic anaemia and show that such treatment is at least as good as bone-marrow transplantation. Its mechanism of action remains unknown, but most patients with aplastic anaemia have a pool of haematopoietic stem cells able to repopulate the marrow after this type of treatment.

Adolescent↗

[Progress in bone marrow transplantation in acute leukemia and aplastic anemia].

Experience with 17 allogeneic bone marrow transplantation (BMT) patients with refractory acute leukemia is reported. The authors show that it is extremely difficult to cure patients with end stage disease by this procedure. Based on experience that, if performed early, BMT can bring about over 50% potential cures, it is postulated that marrow grafts should be performed much earlier than is done at present. From the results in 45 patients with severe aplastic anemia it is concluded that the majority do not have a defect at the pluripotent stem cell itself. There is disturbed maturation to functional end cells which can be corrected in most instances by ALG, infusion of allogeneic marrow and low dose androgens. BMT between HLA-identical siblings need not cause major problems, but it involves a considerable risk of fatal complications such as irreversible marrow rejection and GvH.

Acute Disease↗

[Clinical and immunological studies on a patient with myasthenia gravis before and after repeated plasma exchange].

A 56-year-old patient with severe acquired myasthenia gravis refractory to conventional therapy underwent a series of plasma exchanges. Three single exchanges did not improve her status. A series of intensive plasma exchanges of 6 liters each on 4 consecutive days reduced her anti-acetylcholine receptor antibodies to one-tenth of the initial value. The reduction was accompanied by an impressive clinical improvement. This study confirms that antibodies can be removed by plasma exchange. It further shows that the clinical improvement appears to be closely related to the quantity of antibodies removed.

Acetylcholine↗

[Allogenic bone marrow transplantation in severe aplastic anemia and refractory acute leukemia. 35 cases].

23 patients with severe aplastic anemia have been treated. 10 marrow grafts between HLA matched siblings were performed after conditioning with cyclophosphamide. 5 are still alive at over 7 months to over 2 1/2 years after grafting. 3 are hemopoietic chimeras and 2 show autologous marrow reconstitution. 13 patients were treated by ALG with or without transfusion of allogeneic marrow. 9 are still alive with partial to complete autologous hemopoietic reconstitution at over 1 month to over 4 years. It is concluded that in many instances aplastic anemia is an autoimmune disease rather than a primary failure of the hemopoietic stem cells. In acute leukemia allogeneic marrow grafts still pose enormous problems and only one of our 12 grafts has become a longterm survivor.

Acute Disease↗

Failure of isogeneic marrow engraftment in a patient with acute leukemia.

Marrow transplantation was carried out in a patient with acute myelogenous leukemia using a healthy identical twin as donor. The patient was prepared for grafting with combination chemotherapy, high dose cyclophosphamide, and 1,000 rad total body irradiation. Repopulation of marrow did not occur despite a second marrow infusion 21 days following the first attempt. The patient died 44 days after the initial marrow infusion. To our knowledge this is the first patient transplanted from an identical twin who did not achieve engraftment after high dose chemotherapy and supralethal irradiation. The survival time was well in excess of that required for evaluation of engraftment. The possible mechanisms for graft failure are discussed. The use of prophylactic interferon against viral infections is considered as the most likely cause of failure of engraftment.

Bone Marrow Cells↗

Autologous marrow recovery following allogeneic marrow transplantation in a patient with severe aplastic anemia.

A 45-year-old woman with severe idiopathic marrow failure was prepared for marrow transplantation by administration of cyclophosphamide (cy) 50 mg/kg on each of four successive days. She then received an intravenous infusion of 20 X 10(9) nucleated marrow cells from an HL-A matched and mixed lymphocyte culture (MLC) non-reactive sister. There was evidence for minimal marrow recovery in 1-2 months and a second marrow infusion was carried out 69 days after the first without additional immunosuppression. There was a continued slow recovery of peripheral blood counts with complete reconstitution of erythropoiesis, return of the white blood cell count to between 3 and 4000/mm3, with 50% granulocytes, and platelets to 60--70,000/mm3, 11 months after the initial grafting attempt. Red cell antigens and gamma globulin allotypes were of recipient type. The MLC and the indirect cell mediated lympholysis (CML) test became positive possibly indicating cellular sensitization to non HL-A antigens. This report of a patient with severe marrow failure documents autologous recovery of marrow function after receiving a large dose of cy and allogeneic marrow. The implications of this are discussed.

Anemia, Aplastic↗

[Thrombocyte replacement with HL-A typed non-related single donors].

Thrombocytopenic patients who have become refractory to platelet transfusions from random donors will usually respond to HL-A compatible platelets with normal increments and hemostatic improvement. In our experience with unrelated HL-A typed donors, HL-A identity is unnecessary. Donors without excess antigen are compatible and even many donors with 1 excess antigen can be used, at any rate in immunosuppressed patients. Thus successful substitution of platelet becomes possible in refractory patients when family donors cannot be used.

Anemia, Aplastic↗

[Experiences with allogeneic and isogeneic bone marrow transplantations in aplastic anemia and acute leukemia].

A report is presented on 13 clinical bone marrow transplantations (BMT). Six of 6 patients with refractory aplastic anemia are presently alive with partial to complete hemopoetic reconstitution now lasting up to 3 years and more. It is shown that after immunosuppression with ALG and cyclophosphamide, followed by BMT, the patient's own hemopoetic system can recover. Two failures occurred in patients over 50 years of age and in one who had donor transfusions before the graft. Two of 4 patients grafted for acute refractory leukemia are alive, one being in complete remission on day 140. One died from interstitial pneumonia and one had no take although the donor was an identical twin. This patient had been on a maintenance program which included active immunotherapy.

Adolescent↗