摘要:

Abstract:41 (38 males, 3 females) patients with aplastic anemia received fetal liver infusion (FLI) from 74 abortuses with gestation periods of 8–32 (Median(M)‐16) weeks and cell dose of 0.004–11.1 times 108(M‐2.3 times 108) from September, 1976 until November, 1987. 35 persons received single FLI; those with recurrence or no response received two or more FLI. 8 received two; 7, three; 2, four; and 1, six FLI. There was a slow and incomplete autologous hematopoietic improvement in 40% and expected survival of 52% at 1 year, 45% at 2 yr, and 37% at 5 yr (Kaplan Meier estimate). There was rise in fetal hemoglobin (Hb), 0–15.7%, (M‐3.5) among responders in 3–20 (M‐6) months. Patients who survived for more than 12 months had, on average, a longer duration of disease (4 months or more), and higher granulocyte and platelet counts. Statistically, however, these differences were not significant. Reticulocyte count was significantly lower in those who survived beyond 12 months. 1 patient developed acute undifferentiated leukemia 3 yr post‐FLI. The study indicates that fetal liver infusion is likely to benefit about 40% of individuals suffering from severe aplastic anemia. Longer surviving patients, however, may be at risk of developin

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01854.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:Platelet aggregation (PA) induced by ADP, collagen and epinephrine, plasma levels of β‐thromboglobulin (βTG) and thromboxane B2(TXB2) and serum TXB2generation were studied in 11 patients with primary thrombocytosis (7 with essential thrombocythaemia and 4 with polycythaemia vera) and compared with 16 healthy subjects. 5 patients suffered from peripheral vascular ischaemia and another 3 had venous thrombosis, but none had bleeding complications. The patients showed an abnormal pattern of platelet function and of thromboxane generation distinct from the healthy subjects in three aspects, a) Shape change was 5–26 times greater, the lag‐time of collagen PA was 2.3‐2.9 times longer and the extent of epinephrine PA was nil or very low. ADP‐or collagen‐induced PA was also reduced (p<0.02). b) Plasma TXB2generation (corrected to a normal platelet concentration) stimulated by the three PA inducers was within the range of the healthy subjects in spite of the reduced extent of PA. c) Plasma βTG level and serum TXB2generation (both corrected to a normal platelet concentration) were 2.9‐7.1 times higher (p<0.001) indicating enhancedin vivoplatelet activation and possibly increased thrombin generation. These abnormalities were not detected in another 4 patients with secondary thrombocytosis. The abnormal pattern of platelet function and thromboxane generation can be a useful laboratory method in the evaluation of patients with primary thrombocytosis. It might also explain the thrombotic complications which occurred in 8 of the patients in a manner such that increased or normal TXB2generation overcomes the reduced extent of PA. In this respect, the pronounced serum TXB2synthesis might be a marker of intravas

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01855.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:All patients in the region with newly discovered M components were registered from 15 Aug. 1984‐31 Dec. 1986. Among a total of 393 patients enrolled, 162 had multiple myeloma (MM). The incidence rate was 6.6 per 100000 population per year (age‐adjusted to the European standard population), which is somewhat higher than previous reports based on hospital and autopsy records. In particular, the incidence rate was higher in the>70 age group. Thus, the median age was 72 yr, which is the highest reported. As many as 71 (44%) of the 162 MM patients were asymptomatic; these asymptomatic patients had the same age distribution as the symptomatic ones. The stage distribution, occurrence of risk factors, response to chemotherapy, response duration and survival were similar in the age groups below and above the median and, in multivariate survival analysis, age was not an independent risk fac

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01856.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:During a general population follow‐up study in Health Region I in Norway, 162 patients with multiple myeloma (MM) were diagnosed. 71 of these (44%) were asymptomatic, and were observed without chemotherapy. The great majority (90%) were in stage I, and there were only 2 with light chain disease. 45 of the 71 asymptomatic patients developed progressive disease during the 4–5 year follow‐up period. Estimated median time to disease progression was 26 months. The presence at diagnosis of osteolytic lesions and/or at least 20% plasma cells in the bone marrow defined a group with significantly shorter time to progression (median 10vs39 months). Median survival from diagnosis for the asymptomatic patients was 45 months, which is significantly longer than the 26 months of the symptomatic group. However, when estimated from the start of treatment, the survival was similar for the two g

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01857.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:Type IIB is a special variant of von Willebrand's disease, characterized by an abnormal von Willebrand factor which shows an increased interaction with platelets. This interaction sometimes causes platelet aggregation and thrombocytopeniain vivo.It involves the glycoprotein‐Ib (GPIb) receptor on platelets and corresponding GPIb‐binding sites in the von Willebrand factor. We here demonstrate a C ± T mutation at codon 1308 of the von Willebrand factor gene in 2 related patients with IIB von Willebrand's disease. The transition gives rise to a substitution of arginine by cysteine at position 545 of the mature von Willebrand factor subunit. This position is close to the GPIb‐ as well as the collagen‐ and heparin‐binding domains of the von Willebrand factor. The mutation may change the conformation of the molecule in this region and activate the GPIb‐binding domain, which is normally not exposed in the von Willebrand factor of circu

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01858.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:Veno‐occlusive disease (VOD) is a major cause of toxic death after autologous bone marrow transplantation (ABMT). We studied the potential role of continuous administration of low‐dose heparin for VOD prevention in 234 consecutive patients who underwent ABMT in our institution. The population consisted of 98 patients autografted before October 1984 who did not receive heparin, and a series of 136 patients autografted from October 1984 to March 1989 containing 98 patients included in a randomized trial comparing heparin administration (n = 52)vsno heparin (n = 46), and an additional group of 38 patients who received non‐randomized heparin in view of high‐risk criteria to develop VOD (n = 31) or other reasons unrelated to VOD (n = 7). Overall, 90 patients (38%) received heparin and 144 (62%) did not. The global incidence of VOD was 13/234 (5–5%). Heparin did not reduce the risk of VOD in all subgroups studied. In particular, in the randomized trial, the incidence of VOD was 2.2% in the group without heparinvs7–7% in the group receiving heparin. We analyzed in depth the 13 patients who developed VOD and we compared them to a control group of 13 patients pair‐matched for age, sex, diagnosis and preparative regimen, who did not develop VOD. We found that abnormal LFT before ABMT predisposed patients to VOD; refractoriness to platelet transfusion was observed in 85% of the patients in the VOD groupvs15% in the control group (p<0.05). VOD patients had an increased requirement for red cells and platelet transfusions, a lower recovery (R<25%) after the second and third platelet transfusion, and shorter intervals separating the first four platelet transfusions. Further, the platelet reconstitution after ABMT in the VOD group was slower in comparison to the control group (p<0.01). Again, in this pair‐matched analysis continuous infusion of low‐dose heparin di

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01859.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:12 patients with myelodysplastic syndromes were treated with recombinant human erythropoietin (r‐epo). 5 patients had stable anemia, 78–92 g/l, and 7 were transfusion‐dependent. In 11 patients, r‐epo was given intravenously three times a week, with dose escalation after 4 and 8 wk if hemoglobin did not increase more than 15 g/l. The doses were 600, 1500 and 3000 U/kg body weight/wk. The 12th patient was treated subcutaneously with a dose of 560 U/kg/wk. 3 patients showed a significant response with an increase in hemoglobin of ≥ 15 g/l. 2 of these had stable anemia before treatment and increased in hemoglobin from 87 to 116 g/l and from 80 to 99 g/l, respectively. The 3rd patient was transfusion‐dependent and rose to a stable hemoglobin level between 76 and 80 g/l without transfusions. 2 patients showed a reduction of their transfusion need. Mean initial serum erythropoietin in the responding group was 366 U/l compared to 1049 among the non‐responders (p = 0.367). Response was observed in 5/7 patients without bone marrow sideroblasts and in 0/5 patients with sideroblasts (p = 0.027). Erythropoietin seems to be an effective and well‐tolerated treatment for a certain proportion of patients with MDS. A larger patient material might provide a model for predi

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01860.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:In contrast to many detailed studies on the antigenic profile of hemopoietic progenitor cells from human bone marrow, sparse information, so far, has been gathered with regard to the antigen expression of hemopoietic progenitors present in peripheral blood. Previous studies by multiparameter flow‐cytometry have revealed substantial differences of the coexpression of the CD33‐, CD19‐, and CD74‐antigens, respectively, on CD34‐positive cells from blood versus those from bone marrow, respectively. Immunomagnetic purging with monoclonal antibodies detecting the CD34‐, and the CD33‐antigen, respectively, has been used to further characterize the expression of these antigens on day 8 and d‐14 granulocyte/macrophage and erythroid colonies as grown from circulating progenitor cells. Purging with CD34 monoclonal antibody abrogated all colony formation, whereas purging with CD33 antibody led to differential inhibition of the various progenitors. Purging bone marrow cells with CD34 antibody, an inhibition of only about 25 % was observed with regard to erythroid colonies, whereas an inhibition of about 85 % was observed for CFU‐GM. These findings reinforce the view that circulating progenitor cells represent relatively immature stages of differentiation, when compared to bone marrow progenitors. Particularly, d‐8 erythroid colonies from blood do not represent the equivalent of the genuine CFU‐E as described from bone marrow, but they seem to be early stages

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01861.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:The usefulness of the pre‐incubated acidified glycerol lysis test (AGLT), a laboratory test for spherocytosis, has been investigated in a selected hospital population of 348 patients with haemolytic and non‐haemolytic anaemia. The AGLT was positive in 58 out of 59 patients with hereditary spherocytosis. In all 32 patients with other types of hereditary haemolytic anaemia the AGLT was normal or equivocal, but clearly different from spherocytosis. Adults with a positive AGLT, but without hereditary spherocytosis, had auto‐immune haemolytic anaemia, myelodysplastic syndrome or were pregnant women. In newborn infants the AGLT was positive, in the first week of life, in those babies having hereditary spherocytosis or immune haemolysis due to blood group incompatibility; no positive AGLT results were seen if no haematological explanation for neonatal hyperbilirubinaemia could be found. At the optimal cut‐off point the sensitivity of the AGLT for hereditary spherocytosis was 98.3% and the specificity 91.1%, under the most unfavourable conditions. The AGLT is a very useful and simple test for the diagnosis of hereditary sphero

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01862.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:Cytomegalovirus (CMV) infection is one of the most common causes of morbidity and mortality after allogeneic marrow transplantation. We studied 14 consecutive CMV‐seropositive patients adding ganciclovir (2.5 mg/kg i.v. every 8 hours for 7 days prior to transplant and 6 mg/kg three times a week after neutrophils became>0.5 times 109/1 and the patients were platelet transfusion‐independent until d 70) to our previous prophylaxis regimen which consisted of intravenous immunoglobulin and acyclovir. The result was compared with 30 consecutive patients whom we studied with our previous regimen. The addition of ganciclovir did not cause any extra toxicities. The incidence of interstitial pneumonitis and cumulative probability of CMV excretion in the first 100 d post‐transplantation was significantly reduced (p = 0.038 and p = 0.035 respectively). The result shows that addition of ganciclovir significantly decreased the incidence of CMV infection in the early post‐transplantation

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb01863.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY