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1. |
Hereditary haemochromatosis |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 113-125
Timothy M. Cox,
Deirdre K. Lord,
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ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01200.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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2. |
Treatment of pure red‐cell aplasia and aplastic anaemia with Ciclosporin: Long‐term clinical effects |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 126-133
Thomas H. Tötterman,
Martin Höglund,
Mats Bengtsson,
Bengt Simonsson,
Dick Almqvist,
Andreas Killander,
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摘要:
6 patients with pure red‐cell aplasia were treated with Ciclosporin (Cyclosporine A; CS) alone or combined with prednisolone for a period of 9–46 (median 27) months. Prior to study, 5 cases had refractory disease, steroids were contraindicated in 1, and 4/6 patients, including 2 cases with congenital disease, had a disease duration exceeding 11 years. A complete haematological response was obtained in 5/6 subjects, and a partial response in 1. When the pre‐treatment Hb levels (mean±S.D. = 64±13 g/l, range 41–80) for all 6 PRCA patients were compared with the Hb levels after 6 months of CS therapy (104±17 g/1, 80–125), a significant improvement was registered (p<0.005). In half of the patients, remission is maintained with CS as single drug in a dose‐dependent manner. We also treated 5 patients with refractory severe aplastic anaemia with CS (1 case) or CS plus prednisolone (4 cases) for 3–27 (median 10) months. Only 1 patient responded. In this case, a complete haematological remission was induced with CS alone, and remission has been maintained for 27 months. Side effects of CS therapy were common but were dose‐dependent and reversible, with the exception of persistent nephrotoxicity in 1 patient with pure red‐cell aplasia. Based on our present results and a survey of the literature, we conclude that CS therapy is effective and indicated in refractory pure red‐cell aplasia. In severe aplastic anaemia resistant to conventional immunosuppression, the response rate is lower, but a small proportion (around 15%) of patients may benefit from CS therapy. Longer treatment periods may, however, be needed to evaluate the role of
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01201.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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3. |
Immunoglobulin heavy‐chain gene rearrangement in peripheral blood mononuclear cells in non‐Hodgkin's lymphomas ‐ Correlation with kappa:lambda analysis and clinical features |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 134-142
Jack Lindh,
Anita Lindstrøm,
Per Lenner,
Erik Lundgren,
Göran Roos,
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摘要:
41 patients with non‐Hodgkin's lymphomas were analysed to determine occurrence of B‐cell monoclonality in peripheral blood mononuclear cells using two different methods: determination of kappa:lambda ratio by light microscopic immunofluorescence, and heavy‐chain gene rearrangement by DNA‐technique. In 21 patients (51%) clonal heavy‐chain rearrangement was found in blood, whilst 18 of the patients (44%) showed and abnormal kappa:lambda ratio. Discordant results between the methods were observed in 5 cases. Clones with gene rearrangements suggesting blood involvement were found in 16/25 (64%) patients with low grade lymphomas, in 5/16 (31%) patients with high grade lymphoma, in 17/21 (81%) patients with bone marrow involvement, in 20/27 (74%) of stage III‐IV lymphomas and in all of the 14 patients with high lymphocyte count (≥ 5.0 times 109). The conclusion was that clonal analysis by the DNA‐technique is a more sensitive method than the kappa:lambda determination using immunofluorescence. Even though the method is time‐consuming, it could prove to be valuable
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01202.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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4. |
Sequential expression of CD34 and CD33 antigens on myeloid colony‐forming cells |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 143-149
Hans‐Jörg Bühring,
Bernhard Asenbauer,
Kalizia Katrilaka,
Gabriele Hummel,
Friedrich W. Busch,
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摘要:
The expression of CD33 and CD34 antigens on human bone marrow cells was examined by fluorescence‐activated cell sorting and colony assays. A marked difference of antigen expression was observed on sorted progenitors of the granulocyte/macrophage lineage (CFU‐GM) with respect to enrichment and maturation status. Single‐color cell sorting revealed no difference of enrichment by anti‐CD33 antibody between day‐7 and d‐14 progenitors, while anti‐CD34 antibody preferentially enriched d‐14 colony‐forming units (CFU). By two‐color cell sorting it could be shown that enrichment of d‐14 CFU‐GM occurred mostly in the CD34+CD33‐and to a lesser extent in the double‐positive fraction. In contrast, there was no difference in degree of enrichment between d‐7 and d‐14 CD34‐CD33+CFU‐GM. From these data we conclude that, during early myelopoiesis, CD34 antigens are gradually lo
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01203.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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5. |
Is the histological classification of chronic granulocytic leukaemia justified from the clinical point of view? |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 150-154
Ciril Rozman,
Francisco Cervantes,
Evaristo Feliu,
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摘要:
By means of morphometric techniques, in 100 untreated Ph'‐positive chronic granulocytic leukaemia (CGL) patients the main features from the initial bone marrow biopsy were analyzed, with particular attention being paid to morphological and quantitative study of megakaryocytes. The number of megakaryocytes per mm2of marrow tissue showed a mean value of 25.3 (SD±18.8), and was positively correlated with either platelet counts, blood percentage of basophils and blast cells, or spleen and liver size. Based on the number and morphological characteristics of megakaryocytes, patients were classified as having granulocytic CGL (67 cases) or the so‐called chronic megakaryocytic‐granulocytic myelosis (33 cases), but except for higher platelet counts and blood percentages of basophils and blast cells in the latter, no relevant clinical, evolutionary or prognostic differences were observed between the groups. Such results cast doubt on the validity of histological classification of CGL from the clinical point o
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01204.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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6. |
Follicular low‐grade non‐Hodgkin's lymphoma: Long‐term outcome with or without tumor progression |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 155-163
Jens Ersbøll,
Henrik B. Schultz,
Jens Pedersen‐Bjergaard,
Nis I. Nissen,
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摘要:
Long‐term outcome for 127 patients with follicular low‐grade lymphoma was investigated. Therapy included radiotherapy (n = 23), low toxicity chemotherapy with or without radiotherapy (n = 76), or more intensive chemotherapy (n = 22). 6 patients had no initial therapy. Complete remission was obtained in 67% of patients. For patients under 60 years of age median survival was 8.7 yr compared with 3.8 yr for older patients, but survival from lymphoma was identical for the two age‐groups: 75% at 5 yr, and 58% at 10 yr. The relatively low tumor mortality contrasted with a relapse‐free survival of 30% at 10 yr, and relapse 8–9 yr after first remission. Examining the disease topography and the stability of histologic subtype in 78 patients with recurrent lymphoma, two types of relapse with different prognoses were identified: 1)withtumor progression (lymphoma dissemination to atypical extranodal sites and/or histologic conversion to an intermediate/high‐grade lymphoma) seen in 56% of patients with a survival from lymphoma of 13% at 10 yr; and 2)withouttumor progression (involvement of nodal sites, and unchanged histology) seen in 44% with a survival from lymphoma of 77% at 10 yr. Actuarial risk of tumor progression was 44% at 5 yr, and 67% at 10 yr. Except from the negative impact of a large tumor burden, it was not possible to identify patients with high risk for tumor progression. More important than all pretreatment factors was poor response to initial therapy (p = 0.0001). Due to lack of reliable risk factors, it is recommended that all younger patients be treated with the intention of achieving complete remission; a significant fraction might
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01205.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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7. |
Cell‐mediated inhibition of granulopoiesis in vitro in patients with acute myeloid leukemia in remission |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 164-172
Bengt Sallerfors,
Tor Olofsson,
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摘要:
We investigated the in vitro granulopoiesis in 11 patients with acute myeloid leukemia (AML) in complete remission 3–80 months after diagnosis (median 8.5 months). 3 of the patients had subnormal levels of bone marrow‐derived CFU‐GM. 6 of 10 patients tested had defective recloning capacity of d‐7 CFU‐GM, suggesting a stem cell defect. Most patients (7/11) showed an increased colony growth of bone marrow‐derived CFU‐GM after T‐cell depletion by E‐rosetting, while readdition of isolated autologous T cells to T‐cell depleted marrow caused a dose‐dependent inhibition of colony formation; bone marrow T cells were more effective in this inhibition than peripheral blood T cells. Experiments using cells depleted of either CD4‐ or CD8‐positive cells and CD4/CD8‐enriched cell populations showed that both CD4‐ and CD8‐positive cells had the capacity to inhibit colony growth. Long‐term culture of bone marrow cells in suspension showed that the production of CFU‐GM declined at about the same rate as in normal controls. Our findings suggest that there are persisting stem cell defects in patients with AML in remission and that the cell growth regulatory systems may be altered. These abnormalities could possibly be an effect of residual damage to the hematopoietic syst
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01206.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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8. |
Bone marrow and tissue expression of gpIIb/IIIa, LFA‐1, Mac‐1 and gp150,95 glycoproteins |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 173-181
Davide Soligo,
Giorgio Cattoretti,
Mariangela Colombi,
Nicoletta Polli,
Franco Capsoni,
Franco Rilke,
Giorgio Lambertenghi Deliliers,
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摘要:
Monoclonal antibodies (MAbs) against platelet glycoprotein gpIIb/IIIa and the leucocyte adhesion molecules LFA‐1, Mac‐1, and gp 150,95 α chain (CD11a,b,c) and β chain (CD18) have been tested in normal and leukaemic bone marrows, in different human tissues, and in a patient with leucocyte adhesion deficiency (LAD). The effect of these MAbs on platelet aggregation was also tested. GpIIb/IIIa showed widespread distribution, while reactivity of CD11/18 antibodies was limited to haematopoietic cells. Platelets and megakaryocytes were reactive with one CD11a (25.5.2), and with no CD11b/c or CD18 MAbs. GpIIb/IIIa was present on the platelets of the patient with LAD, whereas 25.5.2, (CD11a) bound to his platelets but not to his leucocytes. These data indicate that LFA‐1, Mac‐1, and gp150,95 are not present on human platelets, but they suggest the existence of crossreacting epitopes on gpIIb/IIIa, which is consistent with the hypothesis that these molecules belong to a supergene family of adhesion
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01207.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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9. |
Treatment of acute myeloid leukemia in elderly patients with oral idarubicin as a single agent |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 182-185
J. L. Harousseau,
F. Rigal‐Huguet,
P. Hurteloup,
H. Guy,
N. Milpied,
J. Pris,
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摘要:
Idarubicin (IDR) is a new anthracycline that can be administered orally. Oral IDR was given at a dose of 30 mg/m2daily for 3 d in 20 patients aged 65 to 79 yr with previously untreated acute myeloid leukemia (AML). 5 patients whose marrow remained blastic at d 14 received a second course. 8 patients achieved complete remission (6 after one single course). There were: 1 early death, 4 deaths in aplasia, 7 failures. The hematologic toxicity was high. All but 1 patient had to stay in hospital and the duration of neutropenia was 12 to 34 d (median 19). Oral IDR is an effective therapy for AML in elderly patients but the total dose of 90 mg/m2is too aggressive to be administered safely outside the hospital.
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01208.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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10. |
Quantitation of platelet antigens after chloroquine treatment |
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European Journal of Haematology,
Volume 42,
Issue 2,
1989,
Page 186-192
F. Langenscheidt,
V. Kiefel,
S. Santoso,
A. Nau,
C. Mueller‐Eckhardt,
C. Mueller‐Eckhardt,
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摘要:
The effect of chloroquine treatment on the expression of various platelet antigens was studied. For this purpose, the binding of several human platelet‐reactive antibodies (polyspecific HLA antibodies; platelet‐specific PlA1, PlA2, Bakaantibodies; platelet autoantibodies) and of murine monoclonal antibodies specific for epitopes on the glycoprotein (Gp) complexes IIb/IIIa or Ib/IX were quantitated by means of a competitive enzyme‐linked immunoassay (CELIA) using fresh and stored chloroquine‐treated platelets. We found that HLA antigens were substantially removed from platelets following chloroquine treatment (confirming earlier results) while platelet‐specific antigens on the Gp complex IIb/IIIa were but little affected. Epitopes on the Gp complex Ib/IX did not show any alteration. Storage of chloroquine‐treated panel platelets known to interfere with immunofluorescence had no effect on quantitative IgG determinations by CELIA. We conclude that chloroquine treatment of platelets is practicable for platelet antibody analysis, but exerts its effect unspecifically and requires cautious int
ISSN:0902-4441
DOI:10.1111/j.1600-0609.1989.tb01209.x
出版商:Blackwell Publishing Ltd
年代:1989
数据来源: WILEY
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