摘要:

In 114 patients with systemic malignant gammopathies followed during a 10-year period in the hematology clinic of the Rambam Medical Center, 23 episodes of CNS involvement were recorded. 19 cases with spinal cord compression and 4 cases of intracranial plasmacytomas. Early diagnosis followed by prompt laminectomy and subsequent radiotherapy resulted in complete recovery in 50% and partial improvement in 30% of the patients with spinal cord involvement. In good responders. overall survival was not affected by the neurological complication. In the 4 patients with intracranial involvement, external plasmacytomas of the skull preceded the neurological symptoms. 3 of them were diagnosed late, which led to delay and failure of accurate treatment. Only 1 patient was immediately operated and continued to be well for the past 2 years.

ISSN:0001-5792    

DOI:10.1159/000206956

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

The phagocytic activity of peripheral blood leukocytes was assayed by means of oxygen consumption in 28 patients with systemic lupus erythematosus (SLE) and 22 normal individuals. The method in which zymosan was added to a small volume of whole blood enabled us to assay quantitatively and rapidly both the phagocytosis-connected oxygen consumption and the opsonizing time. The degree of oxygen consumption by phagocytes from SLE patients was not significantly different from that in normals. Opsonizing time of zymosan by autologous serum in SLE patients did not differ from that in normals either. It should be emphasized, however, that the opsonizing time of zymosan in SLE patients was inversely correlated with serum complement level, especially C3 level (coefficient of correlation = –0.76, p < 0.001). This raises the possibility that the phagocytosis may be delayed in SLE patients with low titers of C

ISSN:0001-5792    

DOI:10.1159/000206957

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

Patients with sickle cell anaemia (SCA) and individuals who have undergone splenectomy have an increased susceptibility to bacterial infections. Reports of variable capacity of reduction of nitroblue tetrazolium (NBT) by neutrophils from patients with SCA suggest that there may be a polymorphonuclear dysfunction in this disease. In order to assess the role of the spleen in this neutrophil abnormality we carried out the NBT test on neutrophils from normal or splenectomized individuals and from patients with SCA, sickle cell trait and sickle-cell-β-thalassaemia (S-thal). Decreased NBT scores were observed in SCA and in splenectomized control individuals. In the S-thal group, most of the non-splenectomized patients had normal or near normal NBT scores while all splenectomized patients exhibited decreased values. These results indicate a possible contribution of the spleen in the pathogenesis of this neutrophil dysfunction. The mechanism of this participation is unknown

ISSN:0001-5792    

DOI:10.1159/000206958

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

Mitochondrial DNA (mt DNA) molecules, isolated from normal human lymphocytes, lymphocytes stimulated with phytohemaglutinin (72 h) and a cultured Burkitt’s lymphoma cell line, were examined by electron microscopy. Only monomeric forms of mt DNA were found in normal human lymphocytes, whereas in the other cell types, catenated forms were also observed. Unicircular complex forms were apparently absent in all the cell types studied, suggesting that these forms are not a common malignant transformation in human cells. No clear correlation between mitochondrial modifications – as detected by stereological analysis – and the presence of catenated forms could be established. It is suggested that other mechanisms rather than replication of mt DNA would appear to be the mechanism responsible for the formation of these

ISSN:0001-5792    

DOI:10.1159/000206959

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

An immunoproliferative disorder with M component lgD/λand a strong decrease of the other Ig classes in the serum is reported. The peculiarity of this disorder consists in the shift from a predominant chronic lymphocytic leukaemic pattern, at the beginning, to a true myeloma with plasmacytic leukaemia IgD/λ several months later. The possibility of the removal, in this case, of the initial precocious block with derepression or ‘switch on’ in transformation of the malignant lymphocyte clone and, consequently, change of a type of malignant lymphoma into another more evoluted and more malignant type is disc

ISSN:0001-5792    

DOI:10.1159/000206960

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

Serum iron, transferrin saturation, serum ferritin and urinary iron excretion have been tested in a group of Italian subjects with beta-thalassemia trait. A number of these subjects, mostly men, had signs of increased iron load. Serum ferritin was the most sensitive index among those measured for detection of iron overload and was positively correlated with desferrioxamine-induced urinary iron excretion. Italian males with beta-thalassemia trait may be at high risk of developing iron overload even without prolonged iron or transfusional therapy.

ISSN:0001-5792    

DOI:10.1159/000206961

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

Subcutaneous infusions of Desferal (DF) rarely induce negative iron balance in thalassemia major patients less than 6 years old. In nonsplenectomized patients the requirements for blood transfusions increase slightly. Urinary iron excretion decreases during the first days following a blood transfusion. An average of 5.8 mg/day equivalent to 30% of the total iron excretion is eliminated with the feces after subcutaneous infusions of DF. Serum ferritin does not decrease significantly after 18–24 months of therapy. The effectiveness of long-term therapy progressively increases in the splenectomized patients, while it decreases appreciably in the course of the treatment in the nonsplenectomized one

ISSN:0001-5792    

DOI:10.1159/000206962

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

Red blood cells (RBC) from 13 patients suffering from dysmyelopoietic syndromes (DMPS) have been studied. About half of these subjects showed an abnormal Na+ and K+ leakage of their RBC, which were incubated at 37 °C for 24 h. The mean rate of the glycolytic pathway increased significantly and the glycolysis values correlated well with the Na+ gain. Moreover, 9 out of the 13 patients showed an abnormal biosynthetic ratio of haemoglobin chains in their reticulocytes. Since the multiple defects in DMPS erythrocytes do not seem clearly linked by cause-effect relationships, they probably derive from several cooperating factors in pathological erythroid precursors, leading to RBC membrane damage

ISSN:0001-5792    

DOI:10.1159/000206963

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

A new variant of G6PD associated with chronic nonspherocytic hemolytic anemia (CNSHA) in a Japanese male is described. This variant is unique in that it has a normal electrophoretic mobility, normal Km for glucose-6-phosphate and NADP, and a normal utilization of the substrate 2-deoxyglucose-6-phosphate and deamino-NADP. It also shows a decreased thermal stability and a biphasic pH curve. These characteristics distinguish this variant from previously reported variants associated with CNSHA and we have called it G6PD Kanazawa.

ISSN:0001-5792    

DOI:10.1159/000206964

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

Circulating immune complexes were investigated in sera of 13 patients with paroxysmal nocturnal hemoglobinuria. A significant inhibition was observed in the complement-dependent rosette formation (EAC) in 6 cases; 2 patients presented an increased anti-complementary activity; antilymphocyte antibodies were demonstrated in 2 patients. The levels of C3d and immunoconglutinins were also increased; there were no differences in the total hemolytic complement between patients and normal controls. There was a significant correlation between C3d and immunoconglutinins with the inhibition of the EAC rosette formation test. These results provide evidence for the presence of circulating immune complexes in paroxysmal nocturnal hemoglobinuria.

ISSN:0001-5792    

DOI:10.1159/000206965

出版商:S. Karger AG    

年代:1982

数据来源: Karger