摘要:

The clinical reaction and the immunological response to influenza virus vaccine were studied in 43 B-cell chronic lymphocytic leukaemia patients. The Vaxigrip vaccine was administered containing the antigens A/Ghizhou/54/89, A/Singapore/6/86, and B/Yamagata/16/88. The side-effects observed were minimal and well tolerated. Antibody production with titres > 1:20 on day 15 was observed at least for one antigen in 35 patients (81%). In 23 of them (63%) this response was retained on days 30 and 60. Patients with IgG levels ( 700 mg/dl).

ISSN:0001-5792    

DOI:10.1159/000204315

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Ineffective erythropoiesis is a prominent defect leading to anaemic status in thalassaemic patients. Reticulocyte enumeration in the peripheral blood is a non-aggressive method of measuring bone marrow erythropoietic activity. We used an automated reticulocyte counter (Sysmex R-3000) to determine the number and maturation level of circulating reticulocytes among various types of thalassaemia: non-splenectomized β-thalassaemia/haemoglobin E (βE) and splenectomized cases (βE-S), classical haemoglobin H disease (H), haemoglobin H disease with haemoglobin Constant Spring (H/CS), homozygous haemoglobin Constant Spring (CS/CS), homozygous haemoglobin E (EE), heterozygous thalassaemics and other rare combinations. Haemoglobin H disease has a higher absolute count than β-thalassaemia (βE), indicating relatively better compensatory erythropoiesis in haemoglobin H disease. Those with CS genes (H/CS and CS/CS) have poorer reticulocyte maturation than any other type of thalassaemia with rather high absolute numbers, especially in H/CS. This indicates a severer degree of ineffective erythropoiesis in β-thalassaemia (βE), which reflects an insufficient rise in reticulocyte number in comparison with α-thalassaemia (H). The presence of haemoglobin Constant Spring is associated with abnormally low reticulocyte maturation due to enhanced erythrocyte production or direct effect of Constant Spring globin itself, both still unexplained with the current information. The splenectomized βE has increased reticulocyte number and cells with high DNA content, probably nucleated red cells, designated as the upper particle count parameter. However, there is the same degree of reticulocyte maturation in non-splenectomized and splenectomized βE patients, suggesting a role for splenic pooling of reti

ISSN:0001-5792    

DOI:10.1159/000204316

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

We report the disease characteristics and therapeutic results for 25 patient suffering from essential thrombocythaemia (ET), treated with recombinant in terferon-alpha-2b (IFN-α2b). ET was diagnosed according to the criteria·the Polycythaemia Vera Study Group. All patients were programmed to re ceive a subcutaneous induction treatment consisting of 3 MU of IFN-α2b daily for 6 months. In responding patients, treatment was continued for a further months with 3 MU of IFN-α2b three times a week. Complete response wa achieved in 13 of 25 patients, partial response in 10 of 25. In 2 cases, therapy wa unsuccessful. Side effects were usually mild, consisting of flu-like symptoms in most cases, and were easily controlled by paracetamol. After a median follow-up of 14 months after discontinuation of the treatment, most patients re tained the therapeutic response in the absence of toxic

ISSN:0001-5792    

DOI:10.1159/000204317

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Erythropoietic response to exogenously administered recombinant human erythropoietin (rHuEpo) was examined in If maintenance haemodialysis patients with iron overload (IO). All had required numerous blood transfusions earlier ( > f 2 units/year). Diagnosis of IO was established by high serum ferritin (SF) levels ( > f ,100 μg/l), high hepatic CT density ( > 70 Hounsfield units; HU) and excessive iron stores in bone marrow aspirate (grade 6). None of the patients had osteitis fibrosa cystica, aluminium intoxication, haemoglobinopathy or haemochromatosis alleles (HLA A3, B7 and B14). All patients responded to rHuEpo treatment (target haemoglobin level of 9-10 g/dl). None of the patients required iron supplementation or developed ‘functional anaemia’. During 30 ± 3 months of therapy, the initial maintenance dose of rHuEpo (103 ± 12 units/kg/week) and median SF levels (2,250 μg/l) fell (50 ± 8 units/kg/week and 1,060 μg/l, respectively) (p = 0.0003 and 0.0007). The initial and final rHuEpo doses correlated well with the respective SF levels (r = 0.89, p < 0.001). The maintenance dose of rHuEpo required for patients with IO at the start of the treatment period was significantly higher than that (50 ± 5 units/kg/week) required by a control group of patients with adequate iron stores (SF = 100-600 μg/l) who were matched for age, sex and frequency of previous blood transfusions (p = 0.002). The findings suggested that excessive IO caused relative resistance to erythropoiesis on exogenous administration of rHuEpo and that iron supplementation was not warranted during rHuEpo therapy in tho

ISSN:0001-5792    

DOI:10.1159/000204318

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Characterization of β-thalassemia mutations was attempted for 13 unrelated Japanese patients heterozygous for β-thalassemia. We have systematically analyzed β-thalassemia genes using polymerase-chain-reaction-related techniques; dot blot hybridization with oligonucleotide probes complementary to known mutations, restriction endonuclease assay and direct sequencing of amplified genomic DNA. Seven different mutations were detected. Six of them are an amber mutation in codon 90 (GAG to TAG), a four-base-pair deletion in codons 41 and 42 causing premature termination due to frameshift, a C-T substitution at position 654 of IVS-2, a G-A substitution at position 1 of IVS-2 and a C-G substitution at position 848 of IVS-2, leading to splicing defects, and an ocher mutation (GAA-TAA) in codon 121 causing a thalassemia intermedia phenotype with inclusion body formation in erythrocytes. A silent mutation (CTG-TTG) was also detected in codon 91 of the allele with the IVS-2 position 1 mutation. These mutations have been reported previously in the Japanese population. The other mutation is a novel one in the Japanese, an amber mutation (TGG-TAG) in codon 15, causing a β°-thalassemia phenotype by premature termination of the β-globin chain synthesis. We analyzed haplotypes of chromosomes bearing each β-thalassemia mutation. Origins and a spectrum of mutations in comparison with those detected in malaria-endemic regions are dis

ISSN:0001-5792    

DOI:10.1159/000204319

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

There is general agreement on the fact that bone marrow macrophages present a non-proliferating cell population. Using a sequential double-immunostaining technique, a morphometric analysis was performed on routinely processed bone marrow biopsies derived from 70 patients. The purpose of this study was, firstly, to determine the frequency of bone marrow macrophages in a variety of lesions and, secondly, to elucidate whether there is any proliferative activity detectable by immunohistochemical markers. Bone marrow pathology included reactive myelitis (RM), secondary aplastic anaemia (AP), AIDS-related myelopathy, primary (idiopathic) osteomyelofibrosis (OMF) and myelodysplastic syndromes (MDS). The monoclonal antibody PG-M1 which recognizes a formalin-resistant epitope on macrophages and PC10 raised against proliferating cell nuclear antigen (PCNA) were employed. For comparison with the PCNA-labelling index, the newly developed monoclonal antibody Ki-S1, which is associated with cell proliferation, was applied. In comparison with normal bone marrow, morphometric evaluation revealed a significant increase in macrophages in MDS, OMF, RM and especially in HIV-infected patients. Moreover, a positive immunostaining of single macrophages with PC10 was noted very infrequently. This rather inconspicuous PCNA labelling increased in AIDS. By contrast, Ki-S1 expression was found in none of the other pathologies studied. The prevalence of the macrophage population in certain disorders may have a multifactorial origin, such as inflammatory changes like intercurrent infections in AIDS and enhanced cell turnover in MDS as well as involvement of the complex pathomechanisms generating bone marrow fibrosis. In keeping with previous studies, the insignificant PCNA expression of macrophages should not be related to cell proliferation, but to unscheduled DNA strand repair which may be generated in the course of viral infection in AIDS.

ISSN:0001-5792    

DOI:10.1159/000204320

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

The spleen acts as a major site of clearance of antibody-coated platelets from circulation in immune thrombocytopenia (ITP). Splenectomy carries a high cure rate. The biological effect of a single therapeutic dose of ultrasound directed transthoracically to the spleen at 1 MHz and 1 W/cm2 with a mean treatment time of 5 min as generated by Sonopuls 463 (Enraf Nonius) was studied in 30 children with ITP (20 chronic, 10 acute) aged 8-14 years (median 10) and 10 control children. The chronic ITP cases had platelet counts (PC) of 20-50 ×109/1 (mean 36×109/1), showed peak responses at 4 h after exposure 5-18 × 109/1 (mean 10 × 109/1) in 70% of cases, while the remaining 6 patients showed either no change in PC (n = 3) or a decline in PC (n = 3) 5-7 × 109/1. Children with acute ITP had pretreatment PC of 30-50 × 109/1 (mean 40 × 109/1). All had increments of PC after ultrasonic exposure (10-30 × 109/1; mean 18 × 109/1) peaking at 4 h. Six patients with acute ITP maintained the rise in PC while in an other 4, PC returned to baseline in 24 h. The control group showed no change in PC. This therapy was well tolerated and was not associated with significant change in serum lactate dehydrogenase (LDH) levels except in 2 cases with chronic ITP, in whom the LDH levels doubled. All above results were reproduced when therapy was repeated 2 weeks later. In conclusion, this therapy would seem to be safe and well tolerated at such a dose. The effectiveness, rapidity and low cost of this therapy compared with conventional approaches may suggest its use as an alternative therap

ISSN:0001-5792    

DOI:10.1159/000204321

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

A 22-year-old male with a history of hemoglobin E β°-thalassemia presented with thoracic spinal cord compression due to extrameduUary hematopoietic tissue in the epidural space. Following decompressive laminectomy, this epidural mass was irradiated to 20.4 Gy in 12 fractions. The patient made a complete recovery from his neurological symptoms following treatment. Follow-up radiographs showed significant reductions in size of the extradural tumor. Results from this case supported by review of the literature suggest that radiation therapy, with or without decompressive surgery, is an effective therapy in treatment of patients with spinal cord compression due to epidural extrameduUary hematopoietic tissu

ISSN:0001-5792    

DOI:10.1159/000204322

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

A 42-year-old man developed primary non-Hodgkin’s lymphoma of the central nervous system (CNS). Immunohistochemical examination suggested that tumor cells were derived from T cells. Primary T cell non-Hodgkin’s lymphoma of the CNS is a rare tumor, with only 12 well-documented cases in the literature. The clinical features of these 12 cases were similar to those of other CNS lymphomas, and the effect of treatment and prognosis were usually worse than those of extranodal lymphoma. Our patient, who was treated with partial tumor resection and whole-brain irradiation with a boost to the primary site and 5 courses of CHOP therapy (cyclophosphamide, doxorubicin, vincristine, prednisolone), is still alive and in remission 38 months after diagno

ISSN:0001-5792    

DOI:10.1159/000204323

出版商:S. Karger AG    

年代:1994

数据来源: Karger

ISSN:0001-5792    

DOI:10.1159/000204324

出版商:S. Karger AG    

年代:1994

数据来源: Karger