ISSN:0001-5792    

DOI:10.1159/000206577

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

A qualitative abnormality of fibrinogen was identified in a 40-year-old woman with recurrent thrombophlebitis. Fibrinogen levels were normal when determined by immunologic, gravimetric, or nephelometric methods (200–390 mg/dl), but were diminished when tested by techniques based on the thrombin time (13 mg/dl). Asymptomatic family members, including both parents of the proposita, were less severely affected (mean fibrinogen level 100 mg/dl). The rate of fibrinopeptide release from purified fibrinogen was abnormally slow, whereas purified fibrin monomers polymerized at a normal rate. The abnormal fibrinogen was found to act as a competitive inhibitor of thrombin with an inhibitor constant (Ki) of 0.2 μM. This value was the same as the Michaelis constant (Km) of the normal thrombin-fibrinogen reaction, an observation consistent with an abnormality that retards fibrinopeptide release without affecting enzyme-substrate affini

ISSN:0001-5792    

DOI:10.1159/000206578

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

Two family members (daughter and mother) with a bleeding disorder showed prolonged bleeding time and activated partial thromboplastin time associated with decreased plasma levels of factor VIII procoagulant activity, factor VIII-related antigen, and factor VIII-ristocetin cofactor activity. The ristocetin-induced platelet agglutination (RIPA) was enhanced, ADP-, collagen- and Ca ionophore-induced platelet aggregation were also increased at low concentrations of these compounds. In the mother, spontaneous platelet aggregation (SPA) was also observed. Contrary to type II B von Willebrand’s disease (vWd), pseudo-vWd and platelet-type vWd, the patients did not show any increased binding of factor VIII/vWf to platelets in the presence of ristocetin. The RIPA in normal controls were inhibited by addition of antifactor VIII antiserum to the platelet-rich plasma, but not in cases 1 and 2. In this atypical vWd, therefore, the hyperreactivity of platelet aggregation may be due to an intrinsic abnormality of the platelets, but not to any enhanced interaction between plasma factor VIII and the platelet

ISSN:0001-5792    

DOI:10.1159/000206579

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

Giant granules formation was investigated in myeloblasts of a patient with acute myelogenous leukemia by means of the combined techniques of peroxidase cytochemistry both in light and electron microscopy. Several pathologic features were noted: first an abnormal packaging of peroxidase in the peripheral area in large azurophilic granulations, second the progressive enlargement of huge vacuolar inclusions resulting from the interaction and fusion of large azurophilic granules with each other, with normal-sized primary granules and with cytoplasmic components. Microcrystalline structure could not be found in giant vacuoles nor in vacuolar inclusions resembling Auer bodies. This last finding could explain that no disseminated intravascular coagulation was observed in our patient.

ISSN:0001-5792    

DOI:10.1159/000206580

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

A patient with acute leukaemia presenting with superior vena cava syndrome due to a large mediastinal mass is reported. The presence of blast cells with Auer rods in peripheral blood and bone marrow allowed the diagnosis of acute myeloblastic leukaemia. Following intensive chemotherapy the patient achieved a complete remission, with disappearance of the signs of venous obstruction and normalization of the chest roentgenogram. Although no histopathological documentation of the mediastinal tumor was available, its myeloblastic origin was assumed in view of the clinical course.

ISSN:0001-5792    

DOI:10.1159/000206581

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

In 53 patients with aplastic anemia (AA) a number of parameters concerning immune responsiveness were analyzed. Severe monocytopenia and B-lymphocytopenia was detected in most patients, especially in those with diffuse lymphocyte infiltration in the bone marrow. T-inducer (OKT4)/T-suppressor (OKT8) ratios were normal. The mean IgG level was significantly decreased. The frequency of specific antibodies to common viruses was comparable to that of healthy donors except for cytomegalovirus to which antibodies were less frequently found. The responding capacity in MLC was normal but patients’ lymphocytes were often less stimulating than control lymphocytes. Radioresistant suppressor cells were found in 1 patient. In vitro lymphocyte reactivity to mitogens and antigens was severely impaired, related to the numbers of monocytes present. When allogeneic monocytes were added, mitogen responses could be restore

ISSN:0001-5792    

DOI:10.1159/000206582

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

Dogs were chronically irradiated (10 R/day; 0–400 R) or acutely irradiated (20 R/min; 0–400 R) with 60Co γ-rays. Sera were collected and assayed for colony-enhancing activity by a double-layer agar cloning technique. When test sera alone were incorporated in the feeder layers, no colony-forming units-granulocyte/macrophage formation occurred; the addition of peripheral blood leukocytes to such test sera-containing feeder layers resulted in formation of colonies, the number of which was directly related to the cumulative radiation dose. Serum-enhancing factor activity indirectly increased colony formation by stimulating feeder layer leukocytes to generate increased levels of colony-stimulating activity. Levels of serum-enhancing activity were higher at similar dose levels following acute gamma irradiation then following chronic irradia

ISSN:0001-5792    

DOI:10.1159/000206583

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

Erythrocyte surface sialic acid was measured in 94 full-term new-born infants and in 20 premature infants of different ages, varying from 1 to 16 post-natal days. After the first 3 days of life, the amount of sialic acid, both per cell and per gramme of haemoglobin, was rapidly reduced to 70–80% of the initial value; these lower levels were maintained on the following days. The decrease observed on the 4th day may represent an expression of the mechanism by which a large amount of fetal red blood cells is eliminated from the circulation during the same period. Therefore, the relationship between sialic acid decrement and red cell sequestration exists also in fetal erythrocytes, and this may contribute to clarify the exact role of sialic acid in red cell survival tim

ISSN:0001-5792    

DOI:10.1159/000206584

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

The coexistence of autoimmune haemolytic anaemia (direct IgG antiglobulin test) and pure bone marrow sarcoidosis is described in a 69-year-old woman. This case report seems to be the 5th in the available literature.

ISSN:0001-5792    

DOI:10.1159/000206585

出版商:S. Karger AG    

年代:1984

数据来源: Karger

摘要:

A 2.5-year-old boy with congenital pure red cell aplasia (PRCA) became unresponsive to conventional prednisone (2 mg/kg) treatment following a febrile period related to diphteria-pertussis-tetanus (DPT) immunization and required transfusions. Following an administration of high-dose intravenous methylprednisolone, his bone marrow and hematological findings were completely normalized and continued so for 18 months after initiation and 9.5 months after discontinuation of the treatment. When his anemia relapsed, following an upper respiratory infection, he was again found refractory to conventional prednisone administration but responded promptly to intravenous bolus methylprednisolone treatment.

ISSN:0001-5792    

DOI:10.1159/000206586

出版商:S. Karger AG    

年代:1984

数据来源: Karger