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Biomedical subjects

B Osterwalder

Publications and source records attributed to B Osterwalder.

At least 55 records · Page 3Linked to original sources
PubMed1987

Autoimmune hemolytic anemia associated with IgA--diagnostic and therapeutic aspects in a case with long-term follow-up.

A 51-year-old woman presented with superficial thrombophlebitis and severe hemolytic anemia. The initial direct antiglobulin test using a broad-spectrum antiserum for screening was negative. Later testing with monospecific antisera revealed the rare finding of an autoimmune hemolytic anemia (AIHA) associated with IgA autoantibody. In IgA-AIHA the direct antiglobulin test with a broad-spectrum antiserum may be falsely negative, because a standardized anti-IgA level is not compulsory. This case illustrates the diagnostic significance of monospecific antisera in IgA-AIHA. Our patient responded well to high-dose steroids, but due to repeated exacerbations this treatment could not be tapered. Splenectomy was performed 19 months later. Since then the AIHA remained in remission, even after steroids were stopped, and although the direct antiglobulin test continued to be positive for IgA and became positive again for C3d. Available data about pathogenesis and therapy of IgA-AIHA are reviewed.

Anemia, Hemolytic, Autoimmune↗
PubMed1986

[EDTA-dependent pseudo-thrombopenia].

Pseudothrombocytopenia, a term used to define a spuriously low platelet count produced by automatic cell counters, is most often due to platelet clumping induced by EDTA. In a period of two years we have observed 8 patients with a mean platelet count of 46,000/mm3 in the presence of EDTA. When samples of capillary blood (without EDTA) were tested, the mean platelet count was 202,000/mm3. A further patient had platelet adherence to neutrophils with normal platelet counts. This rare finding can also lead to pseudothrombocytopenia. In the event of unexpected thrombocytopenia a smear from EDTA-blood samples reveals the presence of platelet clumping or adherence to neutrophils. Counts of capillary blood samples reveal the true platelet number.

Adult↗
PubMed1986

[Adverse effects of total body irradiation for bone marrow transplantation: prevention and therapy. Experience at Basel, July 1979-March 1986].

Acute toxicity, delayed complications and the incidence of interstitial pneumonitis (IP) after total body irradiation (TBI) are reviewed. Between July 1979 and March 1986 118 patients with hematological malignancies underwent bone marrow transplantation (BMT): all were conditioned with cyclophosphamide and TBI. 106 patients received single dose irradiation (18 with lung-shielding) and 12 had fractionated TBI. Except for mucositis all other symptoms of acute toxicity such as fever, vomiting, parotitis, headache and abdominal pain were usually of short duration. Fractionated TBI did not produce less acute side effects than single dose irradiation. Irradiation, and particularly the maximum lung dose, plays an important role in the multifactorial pathogenesis of IP. Delayed complications may appear several months or years after TBI. The majority of men suffer definitive sterility caused by azoospermia. In women primary ovarian failure and permanent sterility is a common sequela of BMT with TBI. The risk of developing cataracts after single dose TBI is about 80%, and surgical repair was necessary in 43% of these cases. Secondary tumors after TBI have been reported. So far we have observed no secondary malignancies in our patients after BMT. Rampant dental decay can be avoided by careful dental prophylaxis.

Adolescent↗
PubMed1986

[Antibiotic-resistant Corynebacteria--a new problem of infection in immunosuppressed patients].

Corynebacterium species can normally be found on the skin and mucous membranes but rarely cause infections. They are sensitive to most antibiotics. Two patients with severe aplastic anemia undergoing antilymphocyte globulin therapy developed septicemia with a highly antibiotic-resistant corynebacterium (JK-group) only sensitive to vancomycin. Both patients had prolonged severe neutropenia, defects of the mucocutaneous barrier and intensive antibiotic treatment for gram negative infections. In both cases surveillance cultures already revealed the causative microorganism before fever started. One patient was even colonized with corynebacterium for several months before. If this strain is detected in the blood when new fever develops during prolonged neutropenia and broad-spectrum antibiotic therapy, it indicates serious infection in these highly compromised patients. Both cases illustrate that regular microbial surveillance can help to reveal colonization of high risk patients with multiple antibiotic-resistant corynebacterium strains and thus allow early initiation of treatment with vancomycin, which is the only effective antibiotic.

Anti-Bacterial Agents↗
PubMed1986

Immunoadsorption for removal of anti-A and anti-B blood group antibodies in ABO-incompatible bone marrow transplantation.

About 10-15 percent of all patients undergoing allogeneic bone marrow transplantation have a major ABO-incompatibility with their donors. The risk of acute hemolytic reactions due to the infusion of an incompatible donor marrow into the recipient can basically be prevented by recipient antibody depletion or by donor marrow red cell depletion. Nine patients were treated by immunoadsorption using a cartridge with chemically synthesized human blood group A and B antigen as immunoadsorbent for antibody depletion. Within a four-hour-procedure about 2-4 times the patient plasma volume could be processed, thus lowering the anti-A and -B hemagglutinins by 2 to 3 tubes. There was a tendency of better IgG removal when titers initially were high, showing a high antibody clearing capacity. There was no significant correlation between starting titer or amount of plasma volume processed and titer reduction. No decrease in titers were observed in one case. We propose repeated immunoadsorption procedures over 2-3 consecutive days before BMT. The procedure is largely safe and without serious side effects. A major advantage is the avoidance of nonautologous human blood products compared to the conventional plasma exchange. All 8 patients surviving long enough had prompt and stable engraftment of all three cell lines post BMT. No late serological complications occurred when patients were regularly monitored and in vivo adsorption was used when titers increased.

ABO Blood-Group System↗
PubMed1986

Treatment of severe aplastic anemia.

A total of 100 patients with severe aplastic anemia were treated and evaluated in a prospective study at our hospital between January 1976 and October 1983: 28 patients had an HLA-identical sibling donor and were treated with bone marrow transplantation, and 72 patients without an HLA-identical sibling donor were given antilymphocyte globulin followed by oral low-dose androgen therapy. At 1 1/2-9 years after treatment, 13 patients (46%) survive in the transplant group and 45 patients (75%) survive in the second group. All except one in the second group have self-sustaining hematopoiesis without need for transfusions. There is one major difference between the two therapies. Marrow transplantation restores bone marrow function completely and no late hematologic complications have been seen in this group. The majority of patients treated with antilymphocyte globulin in contrast have residual abnormalities of hematopoiesis: macrocytosis, mild granulocytopenia, and mild thrombopenia. Relapse (11 of 72 patients) and clonal hematologic disorders, such as paroxysmal nocturnal hemoglobinuria (four patients) and leukemia (one patient) can occur years after complete bone marrow reconstitution with antilymphocyte globulin. These late disorders are of concern. Despite this, we conclude that antilymphocyte globulin treatment is an effective therapy with low early mortality and morbidity and a high chance for a long, sustained remission. Results are better or at least equivalent to bone marrow transplantation and patients with donors should be given the option of transplant or antilymphocyte globulin.

Adolescent↗
PubMed1985

[Invasive pulmonary aspergillosis--a diagnostic and therapeutic problem in patients with severe aplastic anemia].

Three young patients with severe aplastic anemia undergoing intensive immunosuppressive therapy developed fever and pulmonary infiltrates during longlasting severe granulocytopenia, despite multiple broad spectrum antibiotic combinations and granulocyte transfusions. Invasive pulmonary aspergillosis was diagnosed only by thoracotomy. In conjunction with high dose amphotericin-B therapy complete resolution of aspergillosis was achieved in two cases, paralleled by slow recovery of bone marrow function, whereas in the third case only a partial remission was possible together with transient amelioration of granulopoiesis. We suggest early aggressive surgical methods to establish the diagnosis of aspergillosis in these severely menaced patients, so that antifungal therapy with high dose amphotericin-B can be initiated at an early stage.

Adolescent↗
PubMed1984

[Current status of allogenic bone marrow transplantation in chronic myelocytic leukemia].

Bone marrow transplants were carried out in 18 patients with chronic myelogenous leukemia (CML) in the chronic phase (CP). 12 (67%) are still alive, 11 without evidence of leukemia after a mean observation period of 24 (3-44) months, 1 relapsed and 6 died. The most frequent cause of death was GvHD and interstitial pneumonia (5). 1 patient died of septicemia. 2 grafts were performed in patients with CML in the accelerated phase (AP); both died, one from leukemic relapse and one from GvHD. The authors also participated in an international study in which 117 patients were evaluated. In CP there was a survival plateau at 63%, in AP at 36% and in blastic crisis at 12%. In CP mortality was primarily age-dependent and relapses occurred in only 7%. It is concluded that bone marrow transplantation (BMT) is a highly successful treatment for CML, with the CP the optimum moment for grafting. Longlasting cytogenetic and clinical remissions with potential for cure are possible in a high percentage of patients. The incidence of transplant-related mortality is acceptable. The incidence of leukemic relapse is low in CP. Patients under age 40 with HLA-identical siblings should be transplanted in CP. At present BMT is the only treatment with curative potential for CML.

Adolescent↗
PubMed1984

[Acyclovir in the therapy of herpesvirus infections].

Intravenous acyclovir was tested both prophylactically and therapeutically in herpes infections in 48 patients undergoing bone marrow transplantation. Pretransplant prophylaxis only partly eliminated reactivated infections. Treatment of established mucocutaneous infections was usually successful, also in relapsing cases, when initiated within hours of the appearance of lesions. Extensive lesions, superinfections and dissemination could thus be prevented in this group of severely immunocompromised patients.

Acyclovir↗
PubMed1983

[Bone marrow transplantation in acute lymphatic leukemia during the first remission].

9 patients with acute lymphoblastic leukaemia (ALL) were transplanted in first remission. There were no leukaemic relapses and 6 patients became longterm survivors. Causes of death were GvHD in 2 and interstitial pneumonia in 1. 14 patients with ALL were grafted in later stages of the disease. 10 had a leukaemic relapse. One attained longlasting remission with chemotherapy, while 9 died. One patient died of CMV infection. Cotton wool lesions and haemorrhages in the retina posed a problem in patients with previous cranial irradiation. We recommend transplantation of ALL in first remission if a syngeneic or HLA-identical sibling is available, particularly if the patient has poor risk factors for chemotherapy. Prophylactic cranial irradiation should be replaced by intrathecal methotrexate and/or cytosin arabinoside in these patients.

Adolescent↗
PubMed1983

[Bone marrow transplants--quo vadis?].

Early bone marrow transplantation (BMT) in leukaemia has enormously improved the results. Leukaemic relapse is rare, as is transplant-related mortality from interstitial pneumonia and severe graft-versus-host disease (GvHD). In aplastic anaemia the most important development is that treatment with ALG offers a real alternative to BMT which is of crucial importance for all patients without a histo-compatible family donor. The main problems of BMT are the lack of histocompatible donors and GvHD. In leukaemia, relapse is still an additional problem. The new immunosuppressive agent cyclosporin-A (CyA) has made an important contribution to the prophylaxis of severe GvHD. In future the results with CyA are potentially improvable by combination with other immunosuppressive agents. It is important that the use of HLA-semicompatible family donors, unrelated donors or alternative stem cell sources should become feasible. The value of autologous BMT is difficult to judge because in most instances it has been performed in patients with endstage disease. In several congenital diseases there are alternatives to BMT, such as genetic engineering. Although many problems of BMT still have to be solved, this procedure has an important place in modern medicine.

Bone Marrow Transplantation↗
PubMed1983

[Bone marrow transplantation in leukemia and aplastic anemia].

In a review of allogeneic bone marrow transplantation (BMT) for leukemia the following points proved to be of crucial importance: (1) combination of cyclophosphamide (Cy) and total body irradiation (TBI) for conditioning, (2) early BMT for acute leukemia (AL) in first remission and for chronic granulocytic leukemia (CGL) in chronic phase, (3) prophylaxis of graft-versus-host disease (GvHD) with cyclosporin-A (CyA). 50 successive BMT for leukemia performed in Basel between July 1979 and September 1982 are analyzed. 7 of 13 acute myelogenous leukemias (AML) and 8 of 9 acute lymphatic leukemias (ALL) grafted in first remission, and 7 of 10 CGL, are alive without signs of leukemia and without chronic problems. Of the patients grafted for AL in second remission or later, 3 of 6 AML and 6 of 12 ALL are alive. Severe GvHD was seen in 5 of 43 BMT between HLD identical siblings, in three of them combined with interstitial pneumonia. Thus the incidence of these two serious complications of allogeneic BMT has been drastically reduced with CyA compared to our previous experience with prophylactic methotrexate (MTX). 4 grafts were performed between HLA-haploidentical siblings. 3 of the 4 patients developed fatal GvHD. This implies that in this histocompatibility setting CyA alone is not sufficient. No problems were encountered in 3 syngeneic BMT. 12 leukemic relapses were observed. Relapse never occurred in ALL in first remission and never in CGL. 4 recurrences were seen in AML in first remission. All other relapses were in patients with AL grafted in stages other than first remission. In 86 successive patients with severe aplastic anemia (SAA) the following important advances were made: (1) it was shown that the majority of patients have sufficient hemopoietic stem cells and that after treatment with antilymphocyte globulin (ALG) over 70% have long-lasting remissions, (2) the combined treatment with ALG and high dose prednisone increases the remission rates to 90% and in addition shortens the supportive care period significantly. These developments are of crucial importance for patients without an HLA-identical sibling, (3) success rates of marrow transplants between HLA-identical siblings could be increased from 36% with prophylactic MTX to 67% using CyA.

Anemia, Aplastic↗
PubMed1983

Bone marrow cell dose and kinetics of recovery following allogeneic marrow transplantation in man.

In 50 patients transplanted for acute or chronic leukemia we studied the correlation between the number of infused bone marrow cells/kg recipient body weight and the time needed for engraftment. Engraftment was arbitrarily defined as the first day of 1 X 10(9) leukocytes/l and of 20 X 10(9) reticulocytes/l after the posttransplantation nadir. There is a negative non-linear correlation between the duration of leukopenia following marrow transplantation and the amount of transfused nucleated cells (p = 0.01). Since the incidence of infectious or hemorrhagic complications depends directly on the duration of aplasia it is justifiable to give a maximal cell dose.

Acute Disease↗