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E Czink

Publications and source records attributed to E Czink.

12 recordsLinked to original sources
PubMed1995

Low activity of the classical complement pathway predicts short survival of patients with chronic lymphocytic leukaemia.

The activities of the classical (CP) and alternative (AP) complement pathways as well as the levels of some complement components and circulating immune complexes were measured in 43 patients with chronic lymphocytic leukaemia (CLL) between 1980 and 1984. Depressed CP activities were frequently found in these patients. Clinical course of the disease in the patients was followed until 1992, and compared with the initial complement values. During the follow-up period 36 patients died, death of 33 patients being related to the underlying disease. A strong positive correlation (P < 0.01) was found between the length of survival of the patients and the initial CP values. Patients were divided into two groups: group A, short-term survivors, i.e patients who died in CLL-related complications within 3 years after the complement measurements; and group B, long-term survivors who died > or = 4 years after the complement measurements due to any cause, or were alive at the end of the follow-up period. Average CP values in Group B were almost twice those in group A (P = 0.002), and a similar but less pronounced difference was found in C3 levels (P = 0.055). These differences were even more marked (P = 0.0006 and P = 0.0015, respectively) when only patients in Rai stage 2 and 3 were considered. Low classical pathway activities predicted short survival time: according to the logrank test, patients in Rai stage 2-3 with low (< mean - 2s.d. of the normal values), and normal CP levels survived for 2.0 +/- 1.1, and 4.6 +/- 3.0 years, respectively. All the nine and 11/13 patients with low CP and C4 levels, respectively, died within 3 years after the complement measurements were made. These findings indicate that complement measurements performed in CLL patients have a clinical value.

Aged↗
PubMed1991

[Calcipenic osteopathy in transfusion hemosiderosis and idiopathic hemochromatosis].

Bone metabolism studies were performed on 5 patients with transfusion haemosiderosis (2 male, 3 female; mean age: 30.8 years) and 5 patients with idiopathic haemochromatosis (2 male, 3 female; mean age: 48.8 years). In the majority of the patients, the modern osteodensitometric method showed a significant decrease of the mineral content of the trabecular and cortical bones. Low calcitonin and dehydroepiandrosterone-sulphate concentrations were measured in the majority of the patients with calcipenic osteopathy. Besides marked bone loss, hypogonadotropic hypogonadism was found in 3 male patients, while normal LH, FSH concentration values were measured in 3 female patients. The prolactin concentration was normal in all 6 patients. The authors suppose that besides the already known pathogenetic factors, insufficient calcitonin effect and especially the partial lack of dehydroepiandrosterone-sulphate can play a role in the development of bone loss connected with iron overload. Further study with a greater number of patients in required to support the above findings.

Adult↗
PubMed1991

[Correlation of HLA antigens and idiopathic hemochromatosis in Hungary].

The aim of our present work was to collect data on HLA distribution in patients with idiopathic haemochromatosis in Hungary. Ten unrelated patients with idiopathic haemochromatosis (6 men, 4 women) were studied. Idiopathic haemochromatosis was diagnosed on clinical, biochemical and histological grounds. HLA typing was performed in 10 probands and in all of their first degree relatives available (24) through 7 pedigree studies. HLA A3 was present in 6 of 10 probands [6/10 vs. 18.8% in the group of healthy blood donors (No 53) and 22.4% in Hungarian population (No 1910]. HLA B7 was present in 4 of 10 probands (40% vs. 11.3% and 14.6%). A3B7 antigen association has been found in 4 of 10 patients. A3B7 and A2B38 haplotypes were present twice in 4 of 7 genotyped probands. Pedigree studies revealed one nonaffected homozygote, 17 heterozygotes and 6 non carriers. Extended family and population studies are necessary to establish the gene frequency in Hungary and the probability of the involved haplotypes other than A3B7.

Female↗
PubMed1991

Is dehydroepiandrosterone-sulphate a possible pathogenetic factor in osteopenia of patients with iron overload?

Bone metabolism studies were performed on 5 patients with transfusional haemosiderosis (2 male, 3 female; mean age: 30.8 years) and 5 patients with idiopathic haemochromatosis (2 male, 3 female; mean age: 48.8 years). In the majority of the patients, the modern osteodensitometric method showed a significant decrease of the mineral content of the trabecular and cortical bones. Low calcitonin and dehydroepiandrosterone-sulphate concentrations were measured in the majority of the patients with calcipenic osteopathy. Besides marked bone loss, hypogonadotropic hypogonadism was found in 3 male patients, while normal LH, FSH concentration values were measured in 3 female patients. The prolactin concentration was normal in all 6 patients. The authors suppose that besides the already known pathogenetic factors, insufficient calcitonin effect and especially the partial lack of dehydroepiandrosterone-sulphate can play a role in the development of bone loss connected with iron overload. Further study with a greater number of patients is required to support the above findings.

Adult↗
PubMed1987

C1 and C4 abnormalities in chronic lymphocytic leukaemia and their significance.

Clinical and laboratory correlations of low C1 and C4 levels previously found to be a characteristic feature of chronic lymphocytic leukaemia (CLL) were analysed. Abnormalities of C4 were restricted to stage 1, 2 and 3 of CLL, whereas in the more advanced disease (stage 4) only low C1 levels were found. It was demonstrated that the observed disorders were associated with an increased susceptibility of the patients for infections and impair the immune complex precipitation inhibiting capacity of the patients' sera as well.

Antigen-Antibody Complex↗
PubMed1987

Study of the immune complex precipitation-inhibiting capacity of sera of patients with chronic lymphocytic leukaemia.

Two procedures measuring the capacity of different sera to prevent the precipitation of nascent immune complexes were compared: a kinetic method with a constant immune complex/serum ratio and a new procedure--the increasing immune complex load method--using different immune complex/serum ratios. Immune complex precipitation inhibition is known to depend on complement. The sera of 25 patients with chronic lymphocytic leukaemia (CLL) and the sera of nine healthy blood donors were compared with the two procedures. The kinetic method discriminated poorly between the patients' sera and the control sera, whereas the increasing immune complex load method showed a highly significant difference between the two groups. Sera with low C4 level had the lowest immune complex precipitation-inhibiting activity. Interestingly, however, in the increasing immune complex load method not only hypocomplementaemic but also normocomplementaemic sera from CLL patients were found to be markedly defective. This finding suggests that the CLL patients' sera contain a factor which modulates complement-dependent inhibition of immune complexes.

Antigen-Antibody Complex↗
PubMed1986

Repressed classical complement pathway activities and clinical correlations in chronic lymphocytic leukaemia.

Haemolytic activities of the classical and alternative complement pathways, and levels of C1, C4, C3, factor B and C1 inhibitor (C1-INH) were measured in 137 serum samples of 69 patients with chronic lymphocytic leukaemia (CLL). In most sera IgG, IgA and IgM concentrations were determined as well. Clinical correlations of these laboratory parameters have been studied. C1 and C4 activities were found to be depressed in almost 50% of the sera tested, and hypogammaglobulinaemia was observed with a similar frequency. Low C1 and C4 levels were found mainly in the early stages of the disease. A strong association between the occurrence of infections and hypogammaglobulinaemia was observed, although low immunoglobulin levels frequently occurred in patients without a history of infections. Low C1 and C4 levels were significantly correlated with the incidence of infections, too, and this correlation was observed mostly in the early stages of the disease. The reason for this is not known. The present results suggest that not only low immunoglobulin levels but low C1 and/or C4 levels may contribute to the increased susceptibility to infections in patients with chronic lymphocytic leukaemia.

Complement Activation↗
PubMed1985

Depressed classical complement pathway activities in chronic lymphocytic leukaemia.

Haemolytic activities of the classical and alternative complement pathways, and levels of C1, C4, C3, factor B and C1 inhibitor (C1-INH) were measured in 85 serum samples from 46 patients with chronic lymphocytic leukaemia (CLL). Significantly decreased mean C1 and C4 levels were found, and the haemolytic activities of these components were low or low normal in more than 50% of the sera tested. In 15 sera from 5 patients a complement profile characteristic of acquired C1-IHN deficiency was observed. These results indicate that the depression of the activity of the classical complement pathway is a frequently occurring feature in CLL.

Antigen-Antibody Complex↗
PubMed1983

Serial complement measurements in patients with leukaemia.

Haemolytic activity of the classical and alternative pathways and the levels of C4, C3 and factor B were serially measured in 153 serum samples of 41 patients with different types of leukaemia (9 AML, 14 ALL, 10 CML and 8 CLL). In parallel, the concentration of circulating immune complexes (CIC) was estimated using two methods, the complement consumption assay and the Clq-solubility test. Different complement profiles were found in different types of leukaemia. In AML, each complement parameter tested was elevated as compared to the control values (sera of healthy blood donors). Similar results were observed in ALL, although the differences were less marked. In CML, dissociation of the classical and alternative pathway activities was found: activities of the classical pathway and C4 were significantly elevated, whereas activity of the alternative pathway as well as C3 and factor B concentration did not differ significantly from the control values. In CLL, normal or slightly depressed complement levels were found. The concentration of circulating immune complexes measured by both methods was significantly increased as compared to control values. No significant positive or negative correlations were found between the complement levels and the immune complex concentrations determined in the same sera. An association between the clinical course of acute leukaemia and the complement levels was observed: significantly elevated complement titres were found in the blastic stage of the disease, whereas in remission normal values were recorded. The results of complement measurements performed in serum samples obtained before and after combination chemotherapy were compared on 32 occasions. In parallel to a significant decrease in the absolute number of blast cells in the peripheral blood, a normalization of the increased complement levels was observed after treatment.

Acute Disease↗
PubMed1983

Acquired C1-esterase inhibitor deficiency and recurrent herpes infection in a patient with chronic lymphocytic leukemia.

Profound hypocomplementemia was observed in a patient with chronic lymphocytic leukemia. On the basis of the results of complement measurements performed in nine serum samples taken in a period of 6 mo it was demonstrated that the patient had an acquired form of C1-esterase inhibitor deficiency: marked reduction of CH50, C1, C4, and C1-esterase inhibitor levels with normal alternative pathway, C3, and factor B activities were found. The patient had no typical attacks of angioneurotic edema; however, angioedema-like facial swelling was observed on several occasions in connection with recurrent severe herpes simplex eruptions. The possible association of the marked and long-lasting hypocomplementemia with the clinical course of the herpes infection is discussed.

Angioedema↗