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Biomedical subjects

C Rozman

Publications and source records attributed to C Rozman.

At least 379 records · Page 21Linked to original sources

Acute respiratory failure in severe hematologic disorders.

The development of acute respiratory failure (ARF) in patients with hematologic disorders (HDs) is a life-threatening condition which does not respond well to intensive therapy. We present our experience in the use of intensive therapy for a group of 30 patients suffering from leukemia (21 cases), bone-marrow aplasia (6 cases), or lymphoma (3 cases). Seven had undergone bone-marrow transplantation. All 30 patients were hypoxemic and responded poorly to the administration of high oxygen concentrations via face mask. All were admitted to our intensive respiratory unit (IRU), where 26 received oxygen via mechanical ventilation, and 4 received continuous positive airway pressure (CPAP). Definitive diagnosis was established in 19 (63%) patients. A premortem diagnosis obtained in 8 (26%) cases did not change therapy. The diagnostic accuracy of serology and transbronchial biopsy was low. Of the 6 (20%) patients who recovered from ARF, only 2 were discharged from the hospital. The remaining 24 (80%) patients died in the IRU.

Adolescent↗

Pyrimidine 5'nucleotidase and several other red cell enzyme activities in beta-thalassaemia trait.

The activity of 18 red blood cell (RBC) enzymes and reduced glutathione (GSH) content were measured in 70 normal subjects, in 50 heterozygous beta-thalassaemia carriers and in 50 non-thalassaemic patients with haemolytic anaemia and high reticulocyte counts. In addition, pyrimidine 5'nucleotidase (P5N) activity was also determined in 34 patients with hypochromic, microcytic, iron deficiency anaemia. beta-Thalassaemia trait was associated with an increase in almost all of the enzyme activities, except for 2,3-bisphosphoglycerate synthetase (BPGS) and glutathione reductase (GR) which were normal and for acetylcholinesterase (AChE) and P5N which were slightly and markedly decreased respectively. The increases in enzyme activities were similar to those observed in patients with non-thalassaemic reticulocytosis except for glyceraldehydephosphate dehydrogenase (GAPD), phosphoglyceratekinase (PGK), pyruvate kinase (PK), glutathione peroxidase (GPX) and adenylate kinase (AK) which were higher than in non-thalassaemic group of patients with increased number of reticulocytes. No correlation was found between the severity of P5N deficiency and the intensity of basophilic stippling which was present in 46 of 50 thalassaemic carriers here studied. In addition, GSH content and UV absorption spectra of deproteinized thalassaemic RBC extracts were also found to be normal. The present findings provide further information on the metabolic status of RBC in beta-thalassaemia trait and suggest a possible molecular explanation for the frequently observed basophilic stippling in this disease.

5'-Nucleotidase↗

Iron stores in chronic granulocytic leukaemia at presentation.

In 87 patients with Ph1 positive chronic granulocytic leukaemia (CGL), the bone marrow iron content was studied on smears obtained at diagnosis. A low sideroblast score and a decreased or absent marrow iron on semiquantitative estimation were found in 91% and 85% of cases, respectively. These findings did not correlate with blood parameters reflecting iron status such as Hb concentration, mean corpuscular volume, mean corpuscular haemoglobin, serum iron, total iron-binding capacity and transferrin saturation, which were normal in most cases. In 30 patients, initial serum ferritin was estimated, normal or slightly increased levels being as a rule found. In 24 of such patients, serum ferritin was again measured in remission following busulphan and, although values remained normal, a significant decrease was observed with respect to the initial levels (P less than 0.001). Thus, in spite of the consistent marrow pattern of iron depletion, initial iron stores appear to be normal in CGL. It seems, however, that the disease activity may partially influence the serum ferritin levels.

Adolescent↗

Acute myeloblastic leukaemia presenting with superior vena cava syndrome due to mediastinal mass.

A patient with acute leukaemia presenting with superior vena cava syndrome due to a large mediastinal mass is reported. The presence of blast cells with Auer rods in peripheral blood and bone marrow allowed the diagnosis of acute myeloblastic leukaemia. Following intensive chemotherapy the patient achieved a complete remission, with disappearance of the signs of venous obstruction and normalization of the chest roentgenogram. Although no histopathological documentation of the mediastinal tumor was available, its myeloblastic origin was assumed in view of the clinical course.

Acute Disease↗

Generalized Castleman's disease with urinary elimination of heavy chain fragments.

A generalized form of Castleman's disease of transitional type is reported in a 66-year-old male who was found in addition to have gamma heavy chain fragments in his urine. One portion of them was constituted by Fc fragments and the other ones by fragments which reacted with anti-Fab/IgG and anti-Fc/IgG antisera but not with anti-kappa and anti-lambda antisera. The findings of the present case support an immunological basis for Castleman's disease.

Aged↗

Viscometric methods for assessing red cell deformability and fragmentation.

Erythrocyte deformability is an important determinant of red cell life span and depends on at least three key factors: shape, internal viscosity and membrane mechanical properties. In this paper we describe three viscometric methods for assessing red cell deformability and fragmentation: 1) Plasma and whole blood are sheared with a coneplate viscometer at high shear rates. A mathematical model for liquid behaviour is applied to the results from plasma and whole blood viscosity, and a rheological parameter called internal viscosity is obtained. 2) Shearing packed red cells (Haematocrit greater than or equal to 95%) at high shear rates, a mechanical fragmentation curve is obtained, and a new rheological parameter called rupture stress is determined. 3) Shearing whole blood at 230 Sec-1 and increasing continuously the temperature from 37 degrees C to 57 degrees C, a thermic curve is obtained. A viscosity increase is observed at about 48 degrees C and thermic curve changes are related to effects of heat on the red cell membrane. Internal viscosity, mechanical fragmentation curve and thermic curve assess red cell deformability and fragmentation and can be used as diagnostic tools in diseases with red cell abnormalities.

Blood Viscosity↗

Bone marrow histologic pattern--the best single prognostic parameter in chronic lymphocytic leukemia: a multivariate survival analysis of 329 cases.

In previous studies, the prognostic value of bone marrow (BM) histologic patterns in chronic lymphocytic leukemia (CLL) has been demonstrated. In order to investigate whether such a value is independent of other prognostic parameters, a multivariate survival analysis (Cox's regression model) was undertaken in a series of 329 CLL patients in whom a BM had been performed. The following binary variables were included in the analysis: age (more than 60 years), lymphadenopathy (more than two areas involved), splenomegaly, hepatomegaly, absolute lymphocyte count (more than 30,000 microL), anemia (hemoglobin less than 10 g/dL), thrombocytopenia (less than 100,000 microL), and BM pattern (diffuse v nondiffuse). Three variables entered the regression at significant level: BM pattern (P less than .001), anemia (P less than .001), and hepatomegaly (P = .03). The model was also tested by expressing the variables in a continuous way when possible. Again, BM pattern entered first in the regression (P less than .001), followed by the hepatomegaly (P = .002), hemoglobin level (P = .02), and lymphadenopathy (P = .04). When both the binary and the continuous models were tested separately in 227 patients with BM as initial staging procedure and in 102 patients in whom this was performed later during the course of the disease, in all instances, BM pattern entered first in the regression at a highly significant level. BM histologic pattern appears to be a better single prognostic parameter than any one of the variables employed in current clinical staging systems. A combined clinicopathologic system incorporating the BM pattern, together with the usual clinical variables, is presented.

Actuarial Analysis↗