摘要:

Chronic lymphoid leukaemias are clonal expansions of B and T cells with mature membrane phenotype. Cytogenetic study of these cases usually requires mitogenic stimulation and can often be hindered by a lack of response of the tumour cells to mitogen, poor quality metaphases, complex markers and proliferation of normal cells. In situ hybridisation with fluorescence-labelled chromosome-specific centromeric DNA probe, single or low copy sequences and whole chromosome paints which hybridise to complementary sequences allow the detection of numerical and structural abnormalities on metaphase and interphase cells with much greater efficiency. Comparative genomic hybridisation uses whole genomic tumour DNA as probe which is hybridised to normal metaphases. It is particularly useful for detecting chromosomal changes without being dependent on the dividing tumour cells. The application of these techniques to the investigation of chronic lymphoid leukaemias is reviewed with emphasis on the work done in our laboratory on trisomy 12 and the tumour suppressor region 13q14 in chronic lymphocytic leukaemia, translocation t(11;14) (q13;q32) in mantle cell lymphoma and other chronic B cell leukaemias, inv(14) (q11q32), i(8q) and complex markers in T prolymphocytic leukaemia.

ISSN:0001-5792    

DOI:10.1159/000203621

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

The author summarizes the Hb A2 levels in over 600 β-thalassemia heterozygotes with 32 different base changes or frameshifts and in 22 heterozygotes for 1 of 5 large deletions. Three major groups are recognized: persons with β°-thalassemia or β+-thalassemia (severe) have Hb A2 levels between 4.5 and 5.5%, those with mild β+-thalassemia alleles have levels between 3.6 and 4.2%, with still lower values for those with silent mutations. High values were observed in subjects with the 2 mild β+ alleles with mutations in the β-globin gene promoter (-88, C→T and -29, A→G); unusually high Hb A2 values were also present in several -88 and -29 homozygotes. Data for several members of 8 families in which the -88 (C→T) or the-29 (A→G) mutation, or the -1,393-bp deletion, is present in cis or in trans to a δ-globin gene mutation support earlier observations that an increase in δ-chain synthesis occurs in cis to either one of these 3 alleles. A review of these data confirms the suggestion that the increase in Hb A2 levels results from at least two mechanisms: in a posttranslational system, the formation of αδ-dimers is promoted when excess α-chains are available, while certain promoter mutations increase the transcription of the δ-globin gene in cis because of a change in the binding of t

ISSN:0001-5792    

DOI:10.1159/000203622

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

Blood samples from 118 patients with acute and chronic leukaemia and with more than 50% leukaemic cells were processed with the automated haematology analyser Sysmex NE-8000TM. For 92 out of these 118 the differences in the histograms and scattergrams of the NE-8000 could be used in an attempt to characterise the leukaemia. This interpretation of the histograms and scattergrams appeared to be highly suggestive of the distinction between lymphatic leukaemia and myeloid leukaemia, and could be indicative of the presence of either the chronic or acute form of both the lymphatic and myeloid leukaemias.

ISSN:0001-5792    

DOI:10.1159/000203623

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

Augmentation of γ-gene synthesis by using recombinant human erythropoietin (r-Hu-EPO) represents a new approach to the therapy of β-thalassemia. A prospective study was conducted in 26 transfusion-dependent β-thalassemia major patients. r-Hu-EPO (Eprex/Cilag, Switzerland) was given to the patients at an initial dose of 500 IU/kg s.c. 3 times a week for at least 2 months during which no transfusion was applied. A sustained hemoglobin (Hb) level greater than 8 g/dl was considered as a response to EPO treatment. In the patients whose Hb levels remained under 8 g/dl or did not increase in comparison to pretreatment levels within 4 weeks, the dose of r-Hu-EPO was increased to 1,000 IU/kg 3 times a week and applied for another 4 weeks. Only 16 cases also received oral iron supplementation. The whole blood and reticulocyte counts, the biochemical tests including BUN, creatinine, AST, ALT, alkaline phosphatase and ferritin were done and the percentages of HbF and F cells were analyzed regularly. At the end of the 2nd month, 6 cases qualified to continue with the trial. At the end of the 6th month, r-Hu-EPO therapy was ceased in 3 cases of the 6 since their Hb levels had decreased below 7 g/dl. Only 3 cases (11.5%) continued with the r-Hu-EPO therapy without transfusion for up to 12 months. In conclusion, r-Hu-EPO may be useful in some selected transfusion-dependent patients with β-thalassemia major. Selection criteria should include a mild β-genotype or coinheritance of α-thalassemia, splenectomy and pretreatment reticulocyte response of the patients as well as the patients’ com

ISSN:0001-5792    

DOI:10.1159/000203624

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

Multiple myeloma (MM) is commonly associated with anemia. Several causes have been implicated but inadequate erythropoietin (Epo) production appears to be important. This single-institute open-label, non-comparative clinical trial was undertaken in order to evaluate serum Epo levels in patients with MM and to study the efficacy and toxicity of recombinant human Epo (rHuEpo) in the treatment of MM-associated anemia. MM patients with a baseline hemoglobin (Hb) level of < 11 g/dl received rHuEpo 150 U/kg 3 times/week subcutaneously, with a possible dose increase to 300 U/kg if no response was observed after 4 weeks. The study was designed for 12 weeks, although some responders continued rHuEpo. The study endpoints were determined by an increase in Hb and a decrease in blood transfusion requirements (BTR). Seventeen patients were enrolled in the study. The median serum Epo level was 150 mU/ml (range 11-232). Four patients did not complete the study for reasons unrelated to rHuEpo, but to their underlying MM. Twelve patients (70.6%) responded with an increase in their Hb levels. One patient (5.9%) responded partially. The median Hb level rose from 9.4 g/dl (range 7.3-10.7) at study commencement to 12.5 g/dl (range 9.0-15.2). Six of the 11 patients who were transfusion dependent enjoyed a complete abolition of BTR. The response was also interpreted as an improved quality of life: 3 patients reported a decrease of 1 level in their WHO performance status (PS) score; in 8 patients, the PS declined by 2 grades and 1 patient enjoyed PS reduction by 4 scores. Six patients continue to receive rHuEpo up to 18 months, with a good response and a smaller maintenance dose. Four patients reported flu-like symptoms, 2 suffered from a local irritation and 1 experienced a transient controlled elevation of blood pressure. Summmary: (1) Pretreatment endogenous serum Epo levels were relatively low in all patients studied with MM-associated anemia; (2) rHuEpo was well tolerated in these patients; (3) rHuEpo was highly effective in the treatment of anemia in MM, and (4) the response to rHuEpo is characterized by an increase in Hb levels, a reduction in BTR and an improvement in the WHO PS score.

ISSN:0001-5792    

DOI:10.1159/000203625

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

In this study, three Chinese patients with ticlopidine-induced aplastic anemia were reported and another 13 patients in the English literature were reviewed. We attempted to find underlying similarities, evaluate the risk factors, and identify appropriate treatment for this complication. All but one of the patients were over 60 years old, and the 6 who died were all older than 65. Therefore, old age may be a risk factor for developing this complication. Agranulocytosis occurred 3-20 weeks after initiation of ticlopidine, so frequent examination of white cell count during treatment is recommended. There seemed to be no direct correlation between the dose or duration used and the severity of bone marrow suppression. Treatment for ticlopidine-induced aplastic anemia with colony-stimulating factors seemed to have little effect. The fact that 5 of the 6 patients who received concurrent calcium channel blockers died, should alert clinicians to be more cautious when using these two drugs simultaneously.

ISSN:0001-5792    

DOI:10.1159/000203626

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

A rare case of Hodgkin’s disease which initially presented with a thyroid tumor in an 18-year-old-man is reported. The tumor involved most of the thyroid gland but was well demarcated, and the border between the tumor and the remnants of the thyroid gland was relatively clear, suggesting secondary Hodgkin’s disease involving the thyroid gland. Histologic examination of the tumor revealed nodular sclerosing Hodgkin’s disease composed of many peculiar osteoclast-like giant cells and a few typical Reed-Sternberg cells in the background of dominant eosinophils. With an immunophenotypic study it was shown that these giant cells were positive for the monoclonal antibodies LeuM1 and BerH2, but negative for LCA. Thus the histological diagnosis was clearly conf

ISSN:0001-5792    

DOI:10.1159/000203627

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

We studied a patient with thrombocytopenia associated with Hodgkin’s disease (HD). The megakaryocyte number in the bone marrow and the level of platelet-associated IgG were both increased in the patient. Intravenous γ-globulin therapy and chemotherapy for HD dramatically normalized the platelet count, suggesting that antibody produced by lymphoma cells is likely to account for the thrombocytopenia. Antigen-captured ELISA and Western blotting showed that the patient’s serum had an IgG autoantibody against platelet membrane glycoprotein lb. The patient’s plasma had no inhibitory effect on normal platelet aggregation induced by ristocetin. These findings suggest that the autoantibody found in the patient had a pathogenetic role in the thrombocytopenia, but not in platelet dysfu

ISSN:0001-5792    

DOI:10.1159/000203628

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

There exists a great deal of overlap between many myelodysplastic syndromes and myeloproliferative disorders. This is most evident in the spectrum of disorders classified under the term chronic myeloid leukemia. These include chronic granulocytic leukemia, atypical chronic myeloid leukemia and chronic myelomonocytic leukemia. Current classification often does not clearly separate these entities since they share many features, both clinically and hematologically. We report here a case that satisfies criteria for both chronic myelomonocytic leukemia and atypical chronic myeloid leukemia, appearing to fluctuate between the two. This lends further evidence for the heterogeneity of these disorders and the need for better definition. An improved classification scheme would allow for more accurate reporting and research into etiology and treatment. The complex cytogenetic abnormalities of the case are unique and to our knowledge have not been reported previously. Also, this case report underscores the importance of cytochemical stains when such disorders are under consideration.

ISSN:0001-5792    

DOI:10.1159/000203629

出版商:S. Karger AG    

年代:1997

数据来源: Karger

ISSN:0001-5792    

DOI:10.1159/000203630

出版商:S. Karger AG    

年代:1997

数据来源: Karger