摘要:

AbstractRecent progress in the measurements of the hemostatic markers enables us to assess the detailed profiles of hemostatic activation in various diseases. To evaluate the degree of hemostatic system activation in patients with cerebral thrombosis, detailed coagulation studies were performed in 28 patients with acute‐phase cerebral thrombosis and in 36 with chronic‐phase cerebral thrombosis, together with 6 with chronic‐phase cerebral hemorrhage and 37 age‐matched healthy volunteers. In both acute‐phase and chronic‐phase cerebral thrombosis, plasma levels of thrombin‐antithrombin III complex, plasmin‐α2‐plasmin inhibitor complex and D‐dimer were significantly higher, and antithrombin III and protein C were significantly lower than those in the normal group. Plasma fibrinogen concentration was significantly higher in chronic‐phase cerebral thrombosis than that in chronic‐phase cerebral hemorrhage. No significant difference was found in these variables between acute‐phase and chronic‐phase cerebral thrombosis. In addition, there was no difference in these parameters between chronic phase cerebral hemorrhage and normal subjects. These findings indicate that a sustained activation of coagulation and fibrinolysis is present in cerebral thrombosis, and it might contribute to the pathoge

ISSN:0361-8609    

DOI:10.1002/ajh.2830500302

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractPlatelet RNA content can be detected by flow cytometry using thiazole orange staining to identify platelets recently released into the circulation. We studied platelet RNA content and platelet function in uremic patients under different treatment regimens. Four groups were studied: (I) 15 end‐stage renal disease (ESRD) patients (10M/5F) on maintenance hemodialysis (HD); (II) 11 ESRD patients (6M/5F) on continuous ambulatory peritoneal dialysis (CAPD); (III) 8 patients with chronic renal failure managed conservatively (5M/3F); and (IV) 34 controls (20M/14F). A double color labeling technique using a phycoerythrin‐tagged antibody against glycoprotein lb (CD42b) and RNA labeling by thiazole orange was performed and read by flow cytometry. Aggregation studies were made in platelet‐rich plasma using ADP, epinephrine, collagen, arachidonic acid, and ristocetin. In group I, samples were also obtained after HD. Platelet counts did not differ among the groups. Aggregation studies showed a lower response to ADP and ristocetin in the HD patients, but not in the CAPD or in the chronic renal failure patients. The percentage of platelets with high RNA content in group I was significantly lower than in controls (3.72 ± 1.72% vs. 9.05 ± 3.53%,P<0.01), but was also lower than in the remaining groups (I vs. IIP<0.01, and I vs. IIIP<0.01). No differences were seen in platelet RNA content among groups II (8.67 ± 2.73%), III (9.14 ± 3.04%) and IV. In group I, the percentage of reticulated platelets decreased further after HD (2.14 ± 1.09%,P<0.01). Aggregation studies showed a significantly lower response to ADP and ristocetin in group I (P<0.05), but not in groups II or III in comparison with controls. Aggregation response to ADP and ristocetin decreased after HD (P<0.05). In conclusion, HD may decrease the percentage of RNA‐rich platelets through elimination of the younger and more active platelets and worsen the thrombopathy present in ure

ISSN:0361-8609    

DOI:10.1002/ajh.2830500303

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractThis study compares maximal daily doses of loperamide to escalating doses of continuous intravenous (Cl) octreotide acetate in bone marrow transplant (BMT) and leukemia patients.A total of 36 patients were enrolled in the study. Of these, all were evaluable for intention to treat, and 31 were evaluable for initial response. Based on intent to treat at the initial 48 hr, patients receiving loperamide had a higher complete response rate (86% vs. 45%,P= 0.033) than did those who received octreotide. By treatment analysis (patients who actually received the drug), patients receiving loperamide had a higher complete response rate (92% vs. 56%,P= 0.0448) than did those who received octreotide at the 150 μg dosage level. Additional octreotide patients eventually achieved a CR at a higher dosage level (78%).Loperamide at maximal doses of 4 mg po q6h is more effective than octreotide 150 μg Cl in treating diarrhea following chemotherapy in BMT and leukemia patients. Higher doses of octreotide may be required in a significant number of patients not responding to lower doses.Following chemotherapy, BMT and leukemia patients who developed ≧600 ml of stool volume in a 24‐hr period were randomized to receive loperamide 4 mg po q6h or octreotide 150 μg mixed in hyperalimentation solution or normal saline and administered Cl. Patients were assessed at 48 hr intervals for decrease in stool volume from baseline. Complete response (CR) was defined as ≧50% from baseline stool volume (BSV). Patients receiving octreotide who did not achieve a CR at 48 hr were dose escalated by doubling the dose to a maximum of 2,400 μg with evaluations at 48 hr intervals. Patients receiving loperamide who did not achieve a CR at 48 hr had treatment dis

ISSN:0361-8609    

DOI:10.1002/ajh.2830500304

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractThe thrombocytopenia in chronic liver disease (CLD) has been attributed mainly to hypersplenism, although other factors such as reduced mean life span with increased platelet turnover have also been demonstrated. Immunological abnormalities have been described in the pathogenesis and progression of CLD. In this sense, many studies have reported elevated levels of platelet associated IgG (PAIgG) in patients with CLD, and it has been suggested that PAIgG could represent true antiplatelet antibody. In this study we used a glycoprotein (GP)‐specific immunoassay (MACE) to determine whether PAIgG or circulating antiplatelet antibodies, reacted against the GPIIb/IIIa or GPIb/IX complexes, in patients with CLD. Thirty‐six patients with CLD of diverse etiology were studied (20 female, mean age 53 years, range 38–75 years). 23 out of 36 patients (64%) had anti‐GP antibodies in MACE. Particularly, 12 had anti‐GPIb, 4 anti‐GPIIb/IIIa, and 7 had both types of autoantibodies. The existence of these anti‐GP antibodies was not related with the blood platelet count or etiology of CLD.These data show that in patients with CLD of diverse origin, there is a high prevalence of autoantibodies reacting specifically with platelet membrane GP, which constitutes the first evidence of the specific nature of platelet‐bound IgG in CLD. These findings suggest that in patients with CLD, an immune mechanism may participate in inducing or aggravating the t

ISSN:0361-8609    

DOI:10.1002/ajh.2830500305

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractConcentrations of danazol in patient plasma and red blood cells (RBC) were assayed over a 6‐month period in 75 patients on danazol therapy using a high‐pressure liquid chromatography (HPLC) method more reliable than previous radioimmunoassay (RIA) methods. It was found that plasma danazol rose regularly for 15 days after the beginning of treatment, reaching a steady state plateau of 175 ± 76 ng/ml in 20 patients on normal dose, and less for lower dose schedules. After stopping danazol, concentrations declined to near zero in a similar time frame. RBC concentrations on a packed volume basis were similar to plasma levels. However, the membrane ghosts of RBC contained about 50% of the total RBC danazol, implying about 100‐fold higher concentration in membranes than in plasma. Similar distributions were obtained in vitro with both RBC and platelets, and were confirmed by 14‐C‐labeled danazol. These findings tend to support the hypothesis that the benefits of danazol in immune disorders may be attributable in part to its intercalation in the lipid bilayer of the plasma membrane, altering antigen/receptor expression to modulate immune reactions. This hypothesis was first suggested when it was observed that the RBC of patients on danazol therapy showed morphological changes and increased resistance to osmotic lysis. It was later shown that danazol in vitro reduces binding of autoantibodies, and protects against complement‐mediated lysis, suggesting direct action of danazol on the membranes. This hypothesis is discussed, and danazol's effect in protecting against complement‐mediated lysi

ISSN:0361-8609    

DOI:10.1002/ajh.2830500306

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractThis report reviews the diagnostic significance of immune markers, their relationship to patient outcome, and the therapeutic uses of monoclonal antibodies (MoAbs) in acute leukemia. Immunophenotyping allows for rapid and reproducible diagnosis in the majority of cases of acute leukemia. It is of particular importance in recognizing the major immunologic subclasses of acute lymphoblastic leukemia (ALL), and in identifying subtypes of acute myeloblastic leukemia (AML) which cannot be differentiated by morphology and cytochemistry alone, such as FAB M0 or M7. Immune marker analysis has been used to detect minimal residual disease in patients' bone marrow and CSF after treatment. However, the presence of leukemia‐associated phenotypes on small numbers of normal ceils may reduce the sensitivity of detection in some cases. The prognostic value of immune markers in AML is limited. In ALL, the prognostic significance of the different immunophenotypic subtypes has been lessened by modern treatment protocols. The relationship of mixed‐lineage or biphenotypic antigen expression to patient outcome in both AML and ALL is unclear. Therapeutic applications of MoAbs in acute leukemia include immunologic techniques for purging malignant cells from autografts prior to transplantation, T‐lymphocyte depletion from allografts as a strategy to reduce graft‐versus‐host disease, and the use of flow cytometry to monitor the timing and extent of leukapheresis in peripheral stem cell transplantation. MoAbs have also enabled the recent development of transplantation protocols using positively‐selected CD34 +

ISSN:0361-8609    

DOI:10.1002/ajh.2830500307

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractFibrinogen Richfield exemplifies a dysfibrinogen associated with a life‐long thrombotic tendency. The evaluation of this novel case indicates that, like similar thrombotic dysfibrinogenemias, the abnormal protein polymerizes abnormally and demonstrates impaired clot dissolution. A survey of other cases of dysfibrinogenemia indicates that the relatively common abnormalities of Fibrinopeptide A release are generally asymptomatic or associated with bleeding, polymerization abnormalities are likely to be asymptomatic or associated with thrombosis (or occasionally bleeding), and complex abnormalities or additional, independent hemostatic defects are rather common. Thrombin and Reptilase clotting times are not helpful in distinguishing between the subsets, but clinical history, fibrinopeptide release, and polymerization studies may be useful. Abnormalities of fibrinogen function tend to correlate with changes in molecular domains related to binding and hydrolysi

ISSN:0361-8609    

DOI:10.1002/ajh.2830500308

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractA case of CD56/NCAM+ malignant lymphoma is reported. Only a rare malignant lymphoma cell showed azurophilic granules in the cytoplasm of Giesma‐stained preparations, while electron microscopic examination revealed occasional cytoplasmic granules with paracrystalline inclusions. The most common phenotype seen in NK lymphomas, CD2+, CD3‐, CD56+, CD16‐, CD57‐, was present in the case. Cases with this phenotype have been interpreted to represent either true NK lymphoma or T‐cell lymphoma with NK expression. Genotyping, where performed, has shown TCR germline configuration. Our case showed TCRβ rearrangement indicating that the above phenotype can be associated with a peripheral T‐c

ISSN:0361-8609    

DOI:10.1002/ajh.2830500309

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractThe molecular basis for a family with Type I antithrombin deficiency has been established. Amplification and sequencing of the antithrombin gene identified two mutations: Met20Thr (2523T°C) within exon 2 and Tyr166Cys (5493A→G) within exon 3a. Further analysis indicated that the propositus was a compound heterozygote but in addition provided evidence for phase disruption during the amplification and/or cloning procedure. The Met20Thr mutation appears to be a neutral mutation with no functional consequences. In contrast, the Tyr166Cys mutation is associated with a Type I phenoty

ISSN:0361-8609    

DOI:10.1002/ajh.2830500310

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY

摘要:

AbstractThis report describes the diagnosis of acquired type I von Willebrand disease in a 30‐year‐old woman (G5P5) who presented with complaints of excessive bleeding in the postpartum period. The patient's additional complaints of fatigue, depression, and inability to lose weight resulted in laboratory testing that indicated hypothyroidism due to thyroiditis. Clinical symptoms and laboratory tests for von Willebrand disease and hypothyroidism normalized with L‐thyroxine replacement Thyroiditis resulting in symptomatic hypothyroidism occurs in 2–4 per cent of postpartum women. The possibility of underlying hypothyroidism should be considered for those patients, especially if they are parous women, who appear to have an acquired bleeding disorder suggestive of von Willebrand

ISSN:0361-8609    

DOI:10.1002/ajh.2830500311

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1995

数据来源: WILEY