摘要:

Twenty-four infants and children suffering from glucose-6-phosphate dehydrogenase (G6PD) deficiency during hemolytic crisis were included in this study. Their ages ranged between 3 and 36 months with a median of 10 months. 22 were males and 2 were females. Fourteen out of them received a single bolus dose of desferrioxamine B 500 mg intravenously followed by packed red cell transfusion, while the remaining 10 cases were only transfused. Sequential estimation of hemoglobin level, reticulocytic count and hemoglobinuria was done before treatment, 3, 24, 48 and 72 h thereafter. The hemoglobin level was higher in the desferrioxamine B group. The degree of increase was statistically significant at 48 and 72 h (p < 0.01). Hemoglobinuria stopped in 78.5% in the first group and only in 30% of the second group at 72 h. It was concluded that desferrioxamine B is helpful in shortening the course of hemolytic crisis in G6PD-deficient patients. It could be used as an adjuvant to packed red cell transfusion.

ISSN:0001-5792    

DOI:10.1159/000205320

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Forty-six induction courses were administered to 32 patients with acute promyelocytic leukemia. There were 28 males and 18 females with a median age of 39.5 (range 19–68). Twelve patients were previously untreated, 32 were in relapse, and 2 were refractory to primary induction chemotherapy. Heparin 7.5–10 units/ kg/h by continuous infusion, 4–6 units of platelets and 1–2 units of fresh-frozen plasma (FFP) every 12 h were given to all patients. Previously untreated patients received either daunorubicin, idarubicin or mitoxantrone in combination with cytarabine (Ara-C). For relapsed and refractory patients, regimens included amsacrine with high-dose cytarabine (Amsa/HiDac), homoharringtonine (HHT) alone, or with Ara-C, mitoxantrone and bi-santrene. Hemorrhagic complications occurred in only 1 out of 46 courses (2%). Complete remission rates (CR) were as follows: previously untreated 83% (10/12), relapsed 66% (21/32), primary refractory 50% (1/2). Amsa/HiDac resulted in a 71 % (10/14) CR and HHT-based regimens achieved a 46% (6/13) CR. These regimens are effective and the value of their incorporation into primary therapy should be studied. The use of heparin with platelet and FFP transfusions every 12 h reduces the risk of hemorrhage during induction

ISSN:0001-5792    

DOI:10.1159/000205321

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Serum beta-2-microglobulin (β2M) has been suggested as the most powerful prognostic factor in multiple myeloma (MM). This paper investigates its ability to detect remission and relapse in individual patients. A correlation analysis was carried out between β2M and M component determinations, at diagnosis and monthly during follow-up in 21 consecutive MM patients with normal renal function. A statistically significant correlation was observed in 52.4% only. The lack of correlation in the remaining cases was due to low β2M production at diagnosis, or independent fluctuation of these 2 parameters. Serum β2M proved a much less reliable parameter for the detection of tumour variations than simpler M-component determinat

ISSN:0001-5792    

DOI:10.1159/000205322

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Novel erythrocytic inclusion bodies, characterized by strong β-glucuronidase activity, are described. Their physicochemical properties differ substantially from those of known inclusion bodies and they are mainly observed in patients with liver damage. Since these inclusions occur almost exclusively in erythrocytes, the intraerythrocytic β-glucuronidase must originate from an extraerythrocytic source, perhaps entering erythrocytes via a receptor-mediated endocytotic pathwa

ISSN:0001-5792    

DOI:10.1159/000205360

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Stromal cells obtained from bone marrow of Wistar rats were cultured in liquid medium. The cultures were divided into control and experimental groups. After 24 h of culture, the medium was removed and replaced by a new one containing 10-6 and 10-9M 4β-phorbol or phorbol 12,13-diacetate ester in the experimental cultures, or phorbol solvent alone in control cultures. After 7 days, the cultures were stained with Wright’s stain or were subjected to cytochemical procedures for demonstration of nonspecific esterase, alkaline and acid phosphatases and diaphorase activities. The obtained results showed that phorbols stimulated proliferation: fibroblast colonies, fibroblasts, macrophages, immature reticular cells. The number of adipocytes was markedly lower in the experimental cultures. Phorbols stimulated activity of the investigated enzym

ISSN:0001-5792    

DOI:10.1159/000205361

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Stability and expression of the bacterial neomycin resistance gene (neor) transferred to human continuous marrow cultures by a retroviral vector [pZIP-NeoSV(X)] was evaluated over 4 weeks. Following infection of long-term human marrow cultures with pZIP-NeoSV(X), 10-15% of the stromal cells demonstrated high replating efficiency in a dose of the neomycin analogue G418 that was toxic to stromal cells from uninfected cultures. In contrast, G418 resistance was detected in ≤1% of GM-CFUc and CFU-GEMM derived from the same virus-infected compared to control cultures. Infection of human CFU-GEMM enriched 100× by monoclonal antibody selection with pZIP-NeoSV(X) did not increase the percentage of neor progenitors. Marrow cells from cultures infected with pZIP-NeoSV(X) and a replication competent amphotropic virus transferred the vector and G418 resistance to HeLa cells at a frequency of 1/105 for nonadherent and 1/104 for adherent cells. Two established human hematopoietic (HL60 and K562) and one stromal cell line (KM 101) stably expressed the neor gene. Thus, a higher efficiency of infection and expression of a gene transferred by pZIP-NeoSV(X) to permanent human hematopoietic tumor cell lines and fresh marrow stromal cells contrasts with a lower level of expression in fresh CSF-dependent human hematopoietic stem cel

ISSN:0001-5792    

DOI:10.1159/000205362

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

A case of dapsone-induced severe haemolytic anaemia and agranulocytosis is described. A possible common pathogenic mechanism for the simultaneous occurrence of these side effects of dapsone therapy in a patient with normal glucose-6-phosphate-dehydrogenase activity is proposed.

ISSN:0001-5792    

DOI:10.1159/000205363

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

An unique case of primary acquired sideroblastic anaemia (PAS A) with hyperactive thrombopoiesis is described. Chromosome study revealed that the bone marrow cells of the affected patient contained t(l;3)(p36;q21). A prominent feature was marked megakaryocytic hyperplasia with dysmegakaryocytopoiesis. The platelet count temporarily exceeded 500 × 109/1. In addition, we studied the cellular distribution of the unusual chromosome abnormality in this case. Few erythroid colonies (CFU-E and BFU-E) were observed and normal numbers of CFU-GM were formed. Some metaphases containing the clonal karyotypic abnormality were found in the granulocyte-macrophage colonies. However, no abnormal metaphases were apparent in preparations of T and B lymphocytes. The findings in the present case suggest that the long arm of chromosome 3 may contain a region involved in the regulation of megakaryopoiesis. Furthermore, our results strongly indicate that the target cell for chromosomal change is not a pluripotent stem cell (common to lymphoid and myeloid cells) but a progenitor cell common to all myeloid lineages

ISSN:0001-5792    

DOI:10.1159/000205364

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

We report a male patient in whom a diagnosis of essential thrombocythaemia was made at the age of 25. The clinical course was characterised by recurrent thrombotic episodes during the first few years of the disease, followed by a relatively benign course. He was treated with 32P, nitrogen mustard and plateletpheresis. A transformation into myelofibrosis with myeloid metaplasia was revealed 20 years after ET diagnosis. The length of the disease and the 32P therapy are discussed as factors favouring this metamorphosis.

ISSN:0001-5792    

DOI:10.1159/000205365

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

A young woman with recurrent thrombophlebitis following splenectomy for immune thrombocytopenic purpura and two cesarian sections was found to carry a protein C deficiency. This pathology should be considered in any patient with recurrent thromboembolism even when predisposing factors such as pregnancy and postoperative state may by themselves be responsible for such manifestations.

ISSN:0001-5792    

DOI:10.1159/000205366

出版商:S. Karger AG    

年代:1989

数据来源: Karger