[Self-sufficiency with factor VIII in Norway. Use of freeze-dried cryoprecipitates instead of purified factor VIII concentrates].
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Biomedical subjects
Publications and source records attributed to P Stavem.
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Peripheral blood (PB) and bone marrow mononuclear cells from 23 patients with smouldering leukemia were analyzed for natural killer (NK) cell activity and various surface cell markers. Significantly reduced NK activity was detected in the patients' PB compared with the activity in the healthy controls (p less than 0.0005). A similar difference in NK cell activity between the 2 groups was also observed in bone marrow mononuclear cells (p = 0.005). In contrast, no significant differences in cells positive for the NK cell markers Leu-7 and Leu-11b were found between patients and controls, either in PB or in bone marrow. The patients' PB and bone marrow mononuclear cells had, however, a reduced percentage and absolute number of Leu 3a+ and T8+ cells. Patients with smouldering leukemia have immunological derangements which may make them predisposed for the later development of florid leukemia.
In a family with hereditary thrombocytopenia, transfused normal platelets as well as their own platelets had a shortened survival in the patients' circulation (11). On the other hand, the patients' platelets survived normally when transfused to a normal recipient. Platelet-associated immunoglobulins or circulating platelet antibodies were not detected (immunofluorescence method). About 200 ml platelet poor plasma (PPP) from each of 4 normal donors was infused daily for 5 consecutive d to 1 afflicted family member, causing a rise of platelet count from 65 X 10(9)/l to 163 X 10(9)/l and a decrease of bleeding time from 28 to 11 min 7 d after the PPP was discontinued. The platelet count then gradually decreased and was 65 X 10(9)/l with a bleeding time of 30 min 16 d after the PPP was discontinued. In a repeated PPP infusion experiment a similar response was evoked. It is concluded that the afflicted family members must have a deficiency of some plasma principle necessary to keep platelets fit and circulating.
An intermittent combination chemotherapy program was initiated in 1971-79 in 172 patients, aged 15-59 years, with acute leukemia (131 myelogenous (AML) and 41 lymphoblastic (ALL]. Sixteen patients with AML and 6 with ALL have survived for more than 5 years. These long-term survivors represent 24% of AML and 18% of ALL patients who obtained complete remission. Twelve patients (10 AML and 2 ALL) are in continuous first remission 5.5-13.5 years after diagnosis. Occasional late relapses up to 9 years after diagnosis make it impossible to declare any individual patient cured.
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222 patients aged 15-59 years with acute leukaemia were treated with intermittent combination chemotherapy consisting of six 5-day courses of cytotoxic drugs as induction treatment followed by 3 years of maintenance therapy in patients who obtained complete remission (CR). CR was achieved in 50.3% of 161 patients (early deaths included) with acute myelogenous leukaemia (AML). The observed cumulative 5-yr survival rate (observation time 1-12.25 yr) calculated by the life table method was 12% in AML. Among patients who obtained CR, those aged 40-59 yr appeared to fare better than younger patients (5-yr survival: 24%). The M-3 subtype was an adverse prognostic factor. In acute lymphoblastic leukaemia (ALL) the CR rate was 83.6% and the observed cumulative 5-yr survival rate was 14%. Women fared better than men.
Ferrochelatase deficiency of the bone marrow was found in 2 sisters with a syndrome of congenital hypochromic anaemia, hyperferraemia and heavy iron deposits in the liver.
Two sisters had congenital hypochromic microcytic anemia with hyperferremia, heavy iron deposits in the liver, and reduced bone marrow iron. Liver ferrochelatase activity was within normal limits, but in the bone marrow ferrochelatase activity was only 20% of that in healthy controls. There were no findings suggestive of lead intoxication, sideroblastic anemia, or erythropoietic protoporphyria.
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Neutrophils, serum opsonins and staphylococci were incubated at 37 degrees C with continuous end-over-end rotation. The observed frequency of neutrophils with 0, 1, 2... staphylococci deviated systematically from that expected for a Poisson distribution. This suggests marked differences in phagocytosis/adherence between the neutrophils, indicating different neutrophil populations.
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