摘要:

Chronic granulomatous disease (CGD), an immunodeficiency syndrome characterized by extreme susceptibility to bacterial infections, is due to a defect of the respiratory burst in human phagocytes. NADPH oxidase, the enzyme that catalyzes the reduction of oxygen and the release of oxidative radicals, was studied in polymorphonuclear leucocytes (PMNs) in a family affected by an x‐linked inheritance form at high penetrance of the disease. The contents of cytochrome b, suggested as the terminal component of the oxidase electron transport chain, and FAD, the hypothetical proximal component of the chain, were determined in patients and in carriers. Cytochrome b showed the typical behaviour of x‐linked CGD: total absence in patients, intermediate values in carriers. FAD content evaluated on plasma membranes was less decreased than cytochrome b. Carriers also showed a decrease of this flavoprotein. Cytochrome b and FAD contents were compared to NBT test and superoxide production: a clear correlation was observed for the cytochrome b, but FAD plasma membrane evaluation could also be an interesting tool for the metabolic characterization of the disease in patients and in carri

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1986.tb02322.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

Hydroxocobalamin and cyanocobalamin have been compared as the ‘flushing dose’ in the Schilling test. In healthy, haematologically normal subjects excretion of the test dose was greater following a hydroxocobalamin flushing dose than following a cyanocobalamin flushing dose, and to a lesser extent this was also true in patients requiring investigation. There were occasional discrepant results, but in general it appears that, although reference values differ, hydroxocobalamin is a suitable replacement for cyanocobalamin in the Schilling t

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1986.tb02323.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

During a 4‐month period, the patients with thrombocytopenia (N = 14) at a haematological outpatient clinic were subjected to a special laboratory investigation. Evidence of pseudothrombocytopenia was found in 5 of the patients. 1 patient with a giant platelet syndrome associated with deficient platelet function was also found. It is concluded that pseudothrombocytopenia and various thrombocytopathies provide a significant confounding factor in the management of patients with alleged thrombocytopenia. It is therefore suggested that the initial diagnostic work‐up of alleged thrombocytopenia patients should include an assessment of platelet morphology in addition to an overall haemostatic test (bleeding time) and platelet function te

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1986.tb02324.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

13 previously untreated patients with poor prognosis non‐Hodgkin's lymphoma (NHL) underwent high‐dose therapy followed by autologous bone marrow transplantation (ABMT). All patients experienced a great cytoreductive effect and 9 of them reached a complete remission (mean duration 32 months). The best results were observed in patients with more limited disease and in those without symptoms. 7 patients still remain in complete unmantained remission 15–46 months from the transplant. The probability of survival is 74% at 46 months. No therapy‐related deaths were recorded. In differentiating our preliminary approach, we propose high dose therapy followed by ABMT as induction phase in patients with stage II and as consolidation after first line therapy in patients with stages III‐IV. Further studies are warranted to determine which type of lymphoma may benefit more and which conditioning regimens may improve the remis

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1986.tb02325.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

The effect of parenteral hydrocortisone on plasma lactoferrin concentration, neutrophil count and lactoferrin:neutrophil ratio was assessed in 10 volunteer subjects. Administration of a single dose of corticosteroid was followed by a significant rise in the circulating neutrophil count, a significant but proportionately smaller rise in the plasma lactoferrin concentration and a significant fall in the lactoferrin:neutrophil ratio. Acute viral infections were found to be associated with a disproportionately low plasma lactoferrin concentration relative to the circulating neutrophil count. The relatively low lactoferrin concentrations in both these situations could be of significance in regard to the propensity to bacterial infection and superinfection which these 2 groups of subjects display. Compared to patients with viral infection, those suffering from plasmodium falciparum malaria showed a significantly elevated lactoferrin:neutrophil ratio, although this ratio was not significantly different when malarial patients were compared to normal individuals. These findings suggest that the pathogenesis of relative neutropenia in viral and protozoal illnesses is fundamentally different. Finally, it was found that the temperature at which specimen collection takes place does not appear to be a significant variable determining the plasma lactoferrin concentration.

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1986.tb02326.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

Concomitant occurrence of meningeal involvement and thoracic plasmacytoma was observed in a patient with IgA lambda myeloma. Cerebrospinal fluid analysis revealed IgA‐λ paraprotein and pathological plasma cells. CT scan of the chest and lumbar myelogram excluded spinal cord compression. The patient partially responded to craniospinal irradiation but succumbed to rapidly progressive myeloma 20 weeks following diagnosis of meningeal involveme

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1986.tb02327.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY