Scandinavian journal of haematology最新文献

41 cases of non-T, non-B acute lymphoblastic leukaemia (ALL) were classified by immunological criteria using a panel of monoclonal antibodies against common ALL antigen (cALLa), p24, p20 and HLA-DR antigens. Out of 41 cases, cALLa, p24, p20 and HLA-DR antigens were positive in 31 cases, 34 cases, 35 cases and 41 cases, respectively. 4 cases expressing cytoplasmic mu heavy chains were included in the group of common ALL. We demonstrated that a majority of non-T, non-B ALL would be derived from B-lineage cells. The expression of p24 and p20 was followed by the expression of cALLa and the synthesis of cytoplasmic immunoglobulin. These items of evidence support the idea that the expression of cALLa, p24, p20 and HLA-DR antigens on ALL cells would be universal in the USA, Europe and Japan. Although morphologically identical, non-T, non-B ALL cases could be subdivided into phenotypically-defined subgroups on the basis of cALLa, p24, p20 and HLA-DR antigens.

A manual chromogenic substrate method for the determination of heparin cofactor II (HC II) activity in plasma has been adapted to the Cobas Bio centrifugal analyzer. By reducing the dermatan sulfate concentration used, interference by opacity was avoided, and the correlation between the manual and the new automated method was high (r = 0.96). Plasma samples from 182 females and 197 males were examined. The mean values of HC II activities in this normal population was relatively high, with a total range of 43-150% and a standard deviation of 17%. 4 apparently healthy individuals had levels below 60%. In males, the mean HC II activity was higher in the 50-59 yr age group than in either the 20-29 yr group or in the group above 60 yr (p less than 0.05). In females, the postmenopausal age group had higher mean HC II activities, but the difference was not statistically significant.

Plasma lactoferrin concentration and indices of iron nutrition including P-Ferritin, P-iron, total iron binding capacity and percentage saturation were measured in 75 otherwise normal females. This was done in order to evaluate previous reports that neutrophil lactoferrin content was altered by variations in iron storage status. Since P-Lactoferrin is derived from and hence reflects neutrophil lactoferrin content it would seem reasonable to assume that P-Lactoferrin would vary in accordance with iron stores. However, there was no statistically significant difference in P-Lactoferrin concentration between iron deficient subjects and those who were iron replete. Furthermore, there was no statistically significant correlation between any iron related parameter and P-Lactoferrin concentration.

A patient with monoclonal gammopathy and renal failure due to kappa light chain deposition in the glomerular basement membrane is reported. After 8 months, he developed a progressive and severe renal failure requiring haemodialysis. 30 months later, the monoclonal gammopathy was unchanged. Clinical features, as well as kinetic and immunological studies, were consistent with a diagnosis of benign monoclonal gammopathy (BMG). This case suggests that the renal failure complicating a monoclonal gammopathy is not related to its malignancy.

Severe immune haemolytic anaemia and thrombocytopenia developed in a 71-year-old female within 10 d of starting diclofenac (Voltarol) therapy. These complications resolved within 3 weeks of discontinuation of the drug and corticosteroid therapy. A warm autoantibody of the IgG type together with C3 was found in the direct antiglobulin test of the patient's RBC. The patient's serum and RBC eluate contained a warm autoantibody which reacted with all commercial panel cells without the addition of diclofenac, and gave a negative reaction with Rh null and -D- RBC. This pattern of interactions is similar to haemolysis associated with alpha-methyldopa, indicating the presence of autoantibodies directed against structural components common to all Rh antigens. The coexistence of immune thrombocytopenia and immune haemolytic anaemia is suggestive of an autoimmune disease caused by modified T-cell regulation. Although immune haemolytic anaemia is a rare complication of diclofenac therapy, our observations illustrate the severity of haemolytic anaemia in the occasional patient and stress the need for increased awareness of such a development.

A patient with acute leukaemia was treated with i.v. 2-h infusions of Ara-C at a dose of 3.0 g/m2 every 12 h. During 6 d of therapy the concentrations of the metabolite Ara-U in the CSF reached rather high levels of between 60 and 70 mumol/l from d 2-6 due to high levels of Ara-U in the plasma. The concentration of Ara-C in the CSF after the first infusion was 10.8 mumol/l. After repetitive doses on d 2-6 the drug concentrations increased from about 3 mumol/l just before infusion to about 8 mumol/l at the end of infusion, indicating inhibition of Ara-C influx into the CSF during prolonged treatment. We suggest that the high levels of Ara-U in the plasma interfere with Ara-C transport across the blood-brain barrier.

A 57-year-old man with idiopathic pure red cell aplasia went into remission after plasma exchange. He relapsed after 5 months and then failed to respond to treatment with intensive plasma exchange and immunosuppressive agents. Because of a high proportion of T-suppressor cells in the peripheral blood he was treated with lymphocytapheresis in addition to the previous treatment. The patient achieved a long-term haematological remission which has now persisted for more than 3 yr.

Deferoxamine is a hydroxylamine which binds ferric ions to form a highly stable complex. Since iron is thought to be required at a critical stage for cell proliferation, we investigated the effect of deferoxamine on the proliferative activity of human leukaemic cell populations in vitro by means of 3 permanent cell lines, HL60, U937 and 8402. We found deferoxamine to be a potent inhibitor of DNA synthesis and proliferation of leukaemic cells, acting by accumulating treated cells at the early S phase of the cell cycle. Suppression of leukaemic proliferation was obtained at deferoxamine concentrations in the range usually achieved in the treatment of patients for iron overload. Deferoxamine might therefore warrant further investigation as a potentially useful agent for leukaemia chemotherapy.

We carried out alpha-globin gene analysis by restriction endonuclease mapping in a family with 2 cases of HbH disease. These data show that HbH disease in this family results from the interaction between a common deletional defect and a less common non-deletion alpha-thal lesion (--Med/alpha alpha thal). Furthermore, the presence of a beta-thal determinant in this family was investigated by beta gene polymorphism study. We showed that a patient with HbH disease also inherited a beta-thal determinant from the mother and although this was a beta O-thal gene, it was not sufficient to mask the severe alpha chain deficiency. The --Med/alpha alpha thal genotype is more severe than other types of alpha thalassaemia interactions causing HbH disease, probably because the expression of alpha alpha thal determinant may be lower than that of an alpha-thal determinant containing just a single alpha gene (-alpha) and the output so poor that the presence of one beta-thal gene does not significantly change the clinical picture.

Over a period of 20 yr (1962-1982), 67 apparently fit donors at a Regional Blood Transfusion Service were found to have an unexplained positive direct antiglobulin test (DAT). During 1983, 26 were traced and re-tested. 9 still had a positive DAT only 1 of whom had developed autoimmune haemolytic anaemia. 17 had become negative though in 7 of these an autoantibody could still be detected by an enzyme technique. Unlike patients with established autoimmune disorders, the positive DAT individuals were found to have normal T cell subsets though B cells were significantly increased.