Hairy cell leukemia: bone marrow changes following splenectomy and alpha-interferon therapy.

Thirty hairy cell leukemia patients were evaluated repeatedly for their bone marrow (BM) histology. At the time of diagnosis, 18 (60%) had diffuse, 9 (30%) had interstitial, and 2 (10%) had a mixed (diffuse and interstitial) pattern of BM disease. The follow-up BM specimens were obtained at intervals of 3-24 months, and the follow-up observation period was 12-94 months. In patients who were nontreated or only splenectomized, no significant changes were observed except of a persistent megaloblastoid picture of the red cell series and an increase of BM fibrosis. In the alpha-interferon treated patients a complete disappearance of hairy cells was observed in one and a dramatic reduction in five. The hairy cell index was reduced from a mean of 0.8 before to 0.1 after alpha-interferon therapy; most patients displayed megaloblastoid erythropoiesis. In the complete responder features of myelodysplasia were present.

Structural study of hemoglobin Knossos, beta 27 (B9) Ala leads to Ser. A new abnormal hemoglobin present as a silent beta-thalassemia.

A new electrophoretically silent hemoglobin variant is described that produces the classical phenotype of beta thalassemic intermedia in association with beta thalassemia trait. This variant has the expression of a silent beta thalassemia trait. The abnormal hemoglobin was detected by acid-urea-Triton-acrylamide electrophoresis and further demonstrated by isoelectric focusing. The amount of the variant in carrier is approximately 30% of the total hemoglobin. No instability was found. Absence of hemoglobin A in the propositus blood facilitated structural studies. Peptides maps were normal but analysis of individual peptide spots showed an Ala leads to Ser substitution in the beta T3. This variant has been previously called Hb Knossos (beta 27 (B9) Ala leads to Ser).

Fluorescent labelling of proteins of lymphocyte plasma membranes.

Fluorescamine has been used for labelling proteins present on the surface of normal human peripheral blood lymphocytes. Under the conditions of study, 12 labelled proteins could be detected by SDS gel electrophoresis. This method may be of value in biochemical studies of lymphocyte membranes.

Beta-glucuronidase activity of lymph node imprints from malignant lymphomas and chronic lymphocytic leukaemia.

beta-Glucuronidase activity was semiquantitatively estimated in the cells of lymph node (LN) imprints from patients with Hodgkin's disease (HD), diffuse non-Hodgkin's lymphomas, chronic lymphocytic leukaemia (CLL), normal lymph nodes, and benign lymphadenopathies. In addition, in some of these cases beta-glucuronidase activity was semiquantitatively determined in peripheral blood smear lymphocytes. The beta-glucuronidase score (betaGS) was very low in the cells of the LN imprints from patients with diffuse non-Hodgkin's lymphomas. The LN lymphocytes of HD had a normal betaGS independently of the histological subtype of the disease, while in the LN imprint of CLL the enzyme activity was low, normal, or high. The betaGS of the lymphocytes in LN imprints of normal controls and HD were in general significantly lower compared with he lymphocytes of the peripheral blood smears in the same cases. The relation of our findings to the B and T cell origin of malignant lymphomas and chronic lymphocytic leukaemia is discussed.

Urinary beta-aminoisobutyric acid excretion in thalassaemia.

The quantity of beta-aminoisobutyric acid (BAIB) excreted in the urine of patients with an intact spleen suffering from thalassaemia major appears to be proportional to the number of the circulating normoblasts and inversely proportional to the haemoglobin level. After splenectomy only minute amounts of BAIB are excreted. Transfusion constantly, but temporarily, reduces urinary excretion of beta-aminoisobutyric acid. Other anaemic but non-thalassaemic patients may excrete low levels.

Proteolysis in thalassemia: studies with protease inhibitors.

Intracellular proteolysis was studied in both thalassemic and normal reticulocytes. The main experiment comprised a short incubation of RBC with [3H] leucine, extensive washings, and further incubation in the presence of cold leucine and protein synthesis inhibitors. In the aliquots removed at various time intervals, the TCA-soluble radioactivity increased in contrast with the TCA-precipitable cpm, which decreased with no exception on prolonged incubation. Results were more pronounced when the initial incubation was carried out in the presence of a valine analog and were markedly inhibited when phenanthroline was added during the second phase of the experiment. Cell-free system gave similar results. "globin" containing analog residues were degraded easier than free alpha- and beta-globin chains; free alpha or beta Hb chains are not proteolysed. Thalassemic and normal reticulocytes did not show any significant differences. It is proposed that the thalassemic RBC contain efficient proteolytic mechanisms up to the end of their maturation, but these prove incapable of proteolysing the excess alpha chains completely. Chemical manipulations aiming to make those chains more digestible constitute another approach to the treatment of thalassemia.

HLA antigens and Adamantiades-Behçet's disease (A-BD) in Greeks.

The distribution frequency of HLA-A and B antigens was determined in 24 Greek individuals with Adamantiades-Behçet's disease (A-BD) as well as in 280 controls. The standard NIH technique was used for tissue typing. There was an increased incidence of HLA-B5 in the patients group (75% compared to controls 32%). The calculated P corrected value was Pc less than 0.02, the relative risk (RR) was 6.23 and the aetiological fraction (EF) 0.68. This study agrees with the reported association between A-BD and HLA-B5 in other populations from the Mediterranean basin, the Middle East and Far East.

HLA antigens and asthma in Greeks.

HLA-A and -B antigens were determined in a group of 76 Greek asthmatic patients: 35 children (1.5-15 years) and 41 adults (18-73 years). The results were compared to those of 400 healthy unrelated controls from the same population. The standard NIH lymphocytotoxicity test was applied. When all 76 patients were compared to the controls, a statistically significant lower frequency of HLA-B5 and -B35 antigens was noted. When adults were analysed alone, an increased frequency of HLA-B8 was found. On the other hand, in the asthmatic children sub-group, the HLA-A10 antigen was significantly higher and the HLA-B5 was significantly lower than in the controls. These data imply that different HLA antigens may be involved in the pathogenesis of several clinical forms of asthma and that, in order to study the role of immunogenetic factor(s) in the pathogenesis of this disease, more adequate grouping criteria are needed.

Carcinomatous monoarthritis.

A case of carcinomatous monoarthritis involving the left knee due to carcinoma of the pancreas is described. Lythic lesions of the bones were noted and malignant cells in the synovial fluid have been detected. Needle biopsy of the affected knee did not reveal carcinomatous infiltration of the synovium. The value of the synovial fluid examination for malignant cells in such cases is pointed out.