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Biomedical subjects

P Cornu

Publications and source records attributed to P Cornu.

At least 127 records · Page 7Linked to original sources
PubMed1979

[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↗
PubMed1979

[Immunosuppression, bone marrow infusion and low dose androgens, successful therapy of severe aplastic anemia].

18 patients with severe aplastic anemia (SAA) but without an HLA-identical sibling were treated by antilymphocyte globulin (ALG) followed by infusion of marrow cells from a semi-compatible family donor. 13 of these received low dose androgens after ALG: 11 (85%) achieved stable remission without transfusion requirement. One patient relapsed after 4 months, one patient with only partial remission died from infection. None of the 4 patients who did not receive androgens after ALG achieved remission. ALG, marrow and low-dose androgens represent a promising therapy for SAA and can be favorably compared with allogeneic bone marrow transplantation.

Adolescent↗
PubMed1979

Pathological cell aggregates in bone marrow cultures from patients with various hematological diseases.

Non-clonal growth of macroscopic cell aggregates in methylcellulose cultures of abnormal marrow is described. They were seen in all patients with Graft-versus-Host Disease, graft rejection, and autoimmune disease presumably directed against hemopoietic cells, we found them in 35% of patients with primary hematological neoplasias and rarely in patients with solid tumors. They were never encountered in 80 healthy controls. The aggregates originated from small cell clumps which sedimented with the "buffy coat" in contrast to normal bone marrow particles. They contained tumor cells, grafted myeloid cells, or target cells of autoimmune disease in association with a widely varying amount of macrophages. Preliminary results suggest that the frequency of macrophages within the aggregates correlates inversely with the aggressiveness of the clinical condition. We propose that appearance of such aggregates in an indicator of immune activation; we expect that further quantitation of the phenomenon will reveal important clinical correlations and provide a model for the study of host defense to "foreign" cells.

Anemia, Hemolytic, Autoimmune↗
PubMed1979

[Bone marrow transplantation in severe aplastic anemia and acute leukemia].

Much progress has been made in allogeneic bone marrow transplantation for severe aplastic anemia (SAA) and acute leukemia (AL). In SAA it was shown that hemopoietic chimerism and apparently permanent cures can be achieved in the majority of patients by conditioning with cyclophosphamide followed by bone marrow transplantation (BMT) from an HLA-identical sibling. The previous transfusion history is crucial for failure or success: untransfused patients do very well while graft rejection is an enormous problem in most polytransfused ones. We have shown that most patients without HLA-identical sibling donors can be adequately helped as well. After conditioning with ALG followed by transfusion of haploidentical marrow and low dose androgens there is partial to complete autologous hemopoietic reconstitution in virtually all patients. This points to the fact that most of these patients have pluripotent hemopoietic stem cells that are intact, but apparently unable to differentiate to mature cells, because they are inhibited by autoimmune mechanisms. The results of BMT in patients with endstage leukemia are modest. New pilotstudies with early marrow grafts, i.e. for ANLL in first remission and for ALL in second remission indicate that with this type of approach potentially over 50% of all patients with HLA-identical siblings can be cured. We recommend that HLA-typing should be performed early in families with SAA and AL and that the possibility of a marrow graft should be seriously considered before the patients have endstage disease. Marrow grafts are technically simple but they may pose enormous problems such as graft versus host reaction (GvH), interstitial pneumonia, graft rejection and leukemic recurrence. Therefore, the procedure should only be performed in highly specialized centers with much knowledge and experience in the immunobiology of bone marrow transplantation.

Acute Disease↗
PubMed1979

The Basel experience with total body irradiation for conditioning patients with acute leukemia for allogeneic bone marrow transplantation.

We are reporting our experience with 13 patients suffering from end stage acute leukemia that were prepared for allogeneic bone marrow transplantation by combined chemotherapy followed by high dose cyclophosphamide (Cy) and total body irradiation (TBI). Only one patient became a long term survivor. Of the evaluable 12 patients, 6 died of interstitial pneumonia, 4 of GvH and 1 of recurrent leukemia. We conclude that adding combined chemotherapy to the standard conditioning program with Cy and TBI probably increases the risk of developing fatal interstitial pneumonia without eliminating the risk of recurrent leukemia. We suggest that allogenic marrow grafts should be performed earlier in the course of refractory acute leukemias, because in patients with end stage disease its chances of being curative are small.

Adolescent↗
PubMed1978

[Differential diagnosis of primary and secondary erythrocytosis by means of in vitro culture of hematopoietic stem cells].

Peripheral blood precursor cells from all of 12 patients with polycythaemia vera (PV) formed hemoglobinized colonies in vitro without addition os was strictly epo-dependent in 30 normals and 8 patients with erythrocytosis of other origin. By addition of 1 U epo/ml to the cultures, colony formation was increased up to 5-fold in PV. Untreated patients with a short history had the highest numbers of such epo-responsive precursor cells. In 3 patients with concomitant myelofibrosis, erythroid in vitro growth was abundant, but scattered, single colonies could hardly be identified and their hemoglobinization was poor. This picture did not change with addition of epo, and hemoglobinization did not improve. In vitro grown colonies in PV had morphological abnormalities as compared to colonies grown from normal precursor cells: all stages of erythroid differentiation and up to 50% necrotic cells were found within single colonies. Scattered colonies contained an excess of large, immature, vacuolated erythroblasts. It is concluded that these functionaland morphological abnormalities of in-vitro erythropoiesis are a reliable indicator of PV. Further, transition to myelofibrosis is recognized by a characteristic growth pattern with decreased epo-responsiveness.

Cells, Cultured↗
PubMed1977

[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↗
PubMed1977

[Myotonic dystrophy and acute respiratory insufficiency].

Myotonic dystrophy or Steinert's disease may be discovered during acute respiratory failure, sometimes caused by a general anaesthetic. It complicates chronic respiratory failure which is present in almost all cases, both restrictive and obstructive, the clinical signs of which progress with the myopathy. Apart from myotonic degeneration of the respiratory muscles, a hypoventilation syndrome of central origin has been described, but the etiology of this respiratory failure is dominated by repeated aspiration pneumonia favoured by constant dysphagia and passage of food into the trachea and poor cough reflex. One should attempt to correct this.

Acute Disease↗
PubMed1976

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↗
PubMed1976

[Graft versus host disease, a little known complication of blood transfusion].

A case of fatal graft-versus-host disease (GvHD) following blood transfusions is reported. This relatively rare complication of blood and bloodcomponent replacement therapy may occur in recipients with decreased cellular immunity, which may be due to the underlying disease, chemotherapy or radiation therapy, or a combination of both. GvHD is a result of active proliferation of transfused immunologically competent cells which attack host organs. This complication can be prevented by irradiating transfusions with 1500 rads in vitro.

Adult↗