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Biomedical subjects

K Lechner

Publications and source records attributed to K Lechner.

At least 361 records · Page 20Linked to original sources
PubMed1984

Efficacy of the M-2 protocol in previously untreated patients with advanced multiple myeloma.

37 consecutive, previously untreated patients with advanced multiple myeloma (16 patients Stage II, 21 patients Stage III) were treated with a five drug regimen consisting of carmustine, melphalan, vincristine, cyclophosphamide and prednisolone (M-2-protocol) in a prospective manner. Remission was achieved in 24 patients (65%). The median time to remission was 10 weeks, the median duration of remission 15,3 months. Median survival time from the onset of treatment was 24 months for all patients. Responding patients have a projected 65% three year survival. Median survival in non-responders was 10 months. 8 patients died during the first year of treatment. These results do not confirm the favourable results with this drug combination obtained in a previous trial. The discrepancy may be explained by a higher proportion of poor risk patients in the present study.

Aged↗
PubMed1984

Hemophilia and thrombocytopenia in a patient with impaired cellular immunity. A case report.

ITP in hemophiliacs may produce severe bleeding complications. We here report on an eight-year-old boy suffering from severe hemophilia A, who developed ITP and an acquired impaired immune function similar to AIDS. Steroid therapy reverted the thrombocyte count to normal, however it had to be discontinued because of a severe Cushing syndrome. The thrombocytopenia also responded to IgG-therapy and the patient is treated with a long term schedule according to Imbach. It is of interest that the impaired T-helper/T-suppressor cell ratio (0.45) improved to a value of 1.0 after initiation of this therapeutic regimen. We conclude from our observation that i.v. immunoglobulin therapy is of particular value for the treatment of ITP in patients with impaired cellular immunity.

Acquired Immunodeficiency Syndrome↗
PubMed1984

CRM+ severe Fletcher factor deficiency associated with Graves' disease.

A 59-year-old male patient with Graves' disease and severe hereditary Fletcher factor deficiency is described. PKK clotting activity as well as the activity by a chromogenic substrate method (Chromozym PK) was less then 0.01 U/ml. In contrast to functional tests, the immunological assay (Laurell method) showed a PKK antigen concentration of 0.25 U/ml, indicating the presence of an abnormal nonfunctional PKK molecule (CRM+ variant). An inhibitor was excluded since the patient plasma did not inactivate partially purified PKK. Investigation of 11 family members revealed a reduction of the PKK clotting activity in 9 relatives of the patient. Since Graves' disease is considered an autoimmune disease, our case represents an example of an association of a severe hereditary deficiency of a contact factor and an autoimmune disease.

Cross Reactions↗
PubMed1984

Range and clinical significance of the number of myeloid-committed stem cells in the blood of patients with acute leukaemia in remission.

Circulating granulocyte/macrophage progenitor cells (CFU-GM) were assayed serially during remission in 17 patients with acute leukaemia (9 ALL, 8 AML). In patients with ALL receiving cyclophosphamide, 6-mercaptopurine and methotrexate, CFU-GM numbers were significantly lower than in normal individuals; cycles of 'reinduction' chemotherapy (vincristine, prednisolone) caused a 10-fold increase in CFU-GM per ml of blood. In 2 ALL patients a substantial increase in CFU-GM numbers preceded the morphologically detectable relapse. In patients with AML receiving repeated courses of cytosine-arabinoside and 6-mercaptopurine, circulating CFU-GM were likewise reduced. In 6 patients who relapsed, a further reduction of CFU-GM was seen. A complete absence of circulating CFU-GM was observed in 10 of the 23 investigations performed within 6 weeks prior to the morphologically detectable relapse, while such a 'zero'-growth occurred in only 1 of 54 experiments performed during stable remission. In summary, in patients with ALL in remission, circulating CFU-GM are increased following treatment with vincristine and prednisolone. In patients with AML, declining numbers of circulating CFU-GM may predict an imminent relapse.

Acute Disease↗
PubMed1983

Protein C deficiency in two Austrian families.

Protein C antigen was determined by Laurell rocket immunoelectrophoresis in 225 patients with a history of venous thrombosis. Among these patients two females with protein C deficiency were detected. Additional studies in the families of the protein C deficient patients revealed further 7 family members with protein C deficiency. In 8 not anticoagulated patients with protein C deficiency the protein C ranged from 36 to 62% (median: 45%). In one patient on oral anticoagulant treatment protein C antigen concentration was less than 10%, F II and FX were 65 and 50%, respectively. The pattern of inheritance was consistent with autosomal dominant inheritance. 5 of the 9 protein C deficient patients had severe thrombotic tendency characterized by recurrent deep venous thrombosis (n = 4), pulmonary embolism (n = 1), probable mesenteric vein thrombosis (n = 1) and superficial thrombophlebitis (n = 2). All protein C deficient patients without thrombosis were less than 17 years old.

Adult↗
PubMed1983

Glycosylated hemoglobin as a long-term parameter in appraising the severity of hemolytic disease.

Reduced levels of glycosylated hemoglobins (GHb) have been found to be closely related to red cell survival. We therefore studied the relation of this parameter to the clinical applicability in patients with hemolytic disease (n = 20). During a 5-week period we repeatedly measured severity of anemia, i.e., hemoglobin (Hb), packed red cell volume (VPRC), and red blood cell count (RBC), as well as reticulocytosis and parameters of red cell destruction such as serum concentration of lactic dehydrogenase (LDH) and bilirubin together with GHb. There was a weak correlation between simultaneously measured GHb and RBC (r = 0.5, P = 0.02), but none was demonstrable between GHb and Hb, VPRC, reticulocyte counts, LDH, or bilirubin. A much closer correlation, however, was found between actual GHb levels and RBC determined 3-5 weeks previously (r = 0.72, P = 0.001), as well as Hb (r = 0.56, P = 0.015), VPRC (r = 0.57, P = 0.013), reticulocyte counts (r = -0.63, P = 0.006), LDH ( r = -0.53, P = 0.02), and serum bilirubin concentrations ( r = -0.55, P = 0.016). Ghb was also significantly decreased in patients with consistently low values of reticulocytes when red cell destruction was demonstrable. These results show that GHb is a measure of red cell destruction and restitution, and thus may be usefull for long-term monitoring of patients with hemolytic disease.

Adolescent↗
PubMed1983

T-cell alterations in hemophiliacs treated with commercial clotting factor concentrates.

Various immunological parameters were determined in 46 patients with severe hemophilia A and in 9 patients with severe hemophilia B. All patients were treated over many years with commercial factor VIII or IX concentrates. Patients with severe classic hemophilia had a significantly reduced relative and absolute number of T-helper cells and a significantly increased relative and absolute number of T-suppressor cells. About half of these patients had an inverse T-helper/suppressor cell ratio. Patients with moderate hemophilia A and severe hemophilia B did not show these abnormalities. Hemophiliacs with an inverse ratio had a significantly higher concentration of serum total protein, IgG and IgM. No relationship between the amount of factor VIII concentrate administered, the HLA-type of the patient, the presence or absence of CMV-antibodies, hepatitis markers, thrombocytopenia and abnormal liver function tests to the T-cell abnormalities could be established. Lymphadenopathy was frequently associated with an inverse ratio. Indirect evidence suggests that the alterations of the immune system began in 1979/80.

Acquired Immunodeficiency Syndrome↗
PubMed1983

Primary Hodgkin's disease of the lung. Case report and review of the literature.

A patient with primary Hodgkin's disease of the lung is described. Special features of this case were alcohol-induced chest pain as the main presenting clinical symptom and the documentation of the evolution of the pulmonary mass by serial X-rays. Complete remission was achieved by lobectomy and subsequent MOPP-therapy. Since then the patient has been in unmaintained remission for 36 months.

Adult↗
PubMed1983

Urothromboplastin evidence for similarity between urothromboplastin and human brain thromboplastin.

Urothromboplastin (UP) is a lipoprotein similar to Human Brain Thromboplastin (HBrTPL), the specific activity being bound to the presence of the whole complex. UP is found in normal human urine to be macroaggregates, therefore the substance is eluted from the Sepharose 2 B column immediately after the void volume and is found in the bottom of the tube after ultracentrifugation. In polyacrylamidgelelectrophoresis UP does not enter the gel and the UP activity can be eluted from the top of the gel. Natrium-desoxycholate reduces the activity of UP greatly, however after removal of this substance the original activity could be regained. There are similarities between UP and HBrTPL: In the same way as HBrTPL UP acts in the extrinsic pathway of the coagulation system, some evidence for binding of factor VII to UP in the presence of calcium could be found. Anti-apoprotein III antiserum (anti-HBrTPL antiserum) neutralizes UP, depending on the concentration of the antiserum. The same antiserum precipitates with UP using the immunodiffusion method.

Apoproteins↗