摘要:

AbstractThe requirements for factor VIII (AHF) or factor IX (PTC) of hemophilic patients undergoing dental extractions were evaluated to determine the minimum effective regimen. Sixteen patients underwent 19 operative procedures. The mean total dose of factor VIII or IX was 45.8 U/kg for 11 procedures with preoperative replacement therapy and 34.5 U/kg for the 8 without. Four patients received no replacement therapy at all.Our results compared favorably to published studies employing factor replacement alone or in combination with antifibrinolytic agents such as epsilon‐amino‐caproic acid (EACA), with respect to blood products utilized and duration of hospitalization. However, our patients received less factor replacement than usually suggested. General anesthesia with intubation, a common recommendation, was not required in any patients.Dental extractions can be carried out in hemophiliacs using less replacement therapy than currently recommended. In some circumstances, no replacement therapy may be necessary. The reported efficacy of antifibrinolytic agents in reducing the requirement for replacement therapy is difficult to assess because of the relatively large amount of prophylactic factor replacement used in conjunction with these age

ISSN:0361-8609    

DOI:10.1002/ajh.2830160102

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

年代:1984

数据来源: WILEY

摘要:

AbstractTo study the effect of a perfluorocarbon oxygen transport emulsion (Fluosol‐DA) on reticuloendothelial system (RES) function, we measured the blood clearance of human erythrocytes transfused to rats. Compared with saline treatment, Fluosol‐DA at 30 ml/kg doses significantly increased both the percent 20‐hour blood recovery (mean 8.9% ± 2.7 SEM vs 1.3% ± 0.25 SEM) and51Cr t1/2 survival (mean 14.0 hours ± 2.7 SEM vs 3.5 hours ± 0.33 SEM) of the human red cells. This suppression of RES clearance function was transient and no longer detectable seven days after single Fluosol‐DA doses. The Fluosol‐DA‐induced RES block was about three times greater than that obtainable with 4 g/kg of a soybean oil emulsion used for clinical hyperalimentation. On the other hand, the effect of ethyl palmitate (0.5 g/kg), a potent but toxic RES blocker, was 3.5 times greater than that of Fluosol‐DA in this test system. If Fluosol‐DA also induces RES block in humans, this emulsion could be explored as a therapeutic RES blocker in certai

ISSN:0361-8609    

DOI:10.1002/ajh.2830160103

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

年代:1984

数据来源: WILEY

摘要:

AbstractAssays of binding of125iodine‐labeled (125I) human transferrin were used to study transferrin receptor sites on reticulocytes from 15 normal subjects and from 66 patients with various hematologic disorders. In normal subjects, few or no transferrin receptors were detected whereas the average number of receptors per reticulocyte varied greatly from patient to patient, ranging from 0 to 67,700 in samples, from 35 patients, on which Scatchard analysis of binding of [125I]‐transferrin was done. Marked heterogeneity in the number of reticulocyte transferrin receptors in different hematologic disorders was also found in assays with [125I]‐OKT9 (monoclonal antibody to the human transferrin receptor). The number of receptors was not correlated with either the reticulocyte count or the hemog

ISSN:0361-8609    

DOI:10.1002/ajh.2830160104

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

年代:1984

数据来源: WILEY

摘要:

AbstractRNA from bone marrow erythroblasts and peripheral blood reticulocytes of patients with heterozygous β‐thalassemia was analyzed for relative content of α and β globin messenger RNA by molecular hybrization. Erythroblasts from nonthalassemic patients exhibited approximately the same α and β globin mRNA content (β/α mRNA ratio = 0.8–1.0) as circulating reticulocytes (β/α mRNA ratio = 0.74–1.2). The mRNA ratios corresponded well to levels of globin synthesis observed in bone marrow and peripheral blood. Erythroblasts from four patients with heterozygous β‐thalassemia also exhibited approximately the same β/α mRNA ratios in bone marrow erythroblasts (0.34–0.59) as in reticulocytes (0.34–0.4): β globin mRNA was clearly deficient in bone marrow erythroblasts. Globin biosynthesis by erythroblasts of β‐thalassemia heterozygotes was balanced despite the mRNA deficiency (β/α = 0.9–1.0), suggesting that post‐translational phenoma (eg, proteolysis of free globin chains), rather than instability of β mRNA, accounts for the balanced globin chain synthesis frequently observed in bone marrow erythroblasts of

ISSN:0361-8609    

DOI:10.1002/ajh.2830160105

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

年代:1984

数据来源: WILEY

摘要:

AbstractA silent β‐chain hemoglobin variant, not detectable by starch gel or agar gel electrophoresis was found using Triton X‐100 acid‐urea polyacrylamide gel electrophoresis. The abnormal β‐X chain had a more anodic electrophoretic mobility; 38% of the total β chain was of the abnormal type. Structural analysis using high‐performance liquid chromatography and microsequencing procedures indicated a valine to isoleucine substitution at position β11(A8). This anomaly did not change the functional proper ties of the hemoglobin molecule. A mild reticulocytosis was observed in t

ISSN:0361-8609    

DOI:10.1002/ajh.2830160106

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

年代:1984

数据来源: WILEY

摘要:

AbstractConversion of pyruvate kinase (PK) isozymes during the maturation of erythroblasts in five cases of PK deficiency was compared with that in normal subjects using immunofluorescent antibody techniques. In normal erythroblasts, M2‐type PK was clearly seen at the proerythroblast stage, then markedly declined with cell maturation whereas L‐type PK continued to increase. In two patients with classical type PK deficiency, M2‐type PK was still clearly seen in orthochromatic erythroblast, whereas L‐type PK was hardly detected during maturation. In three patients with other types of PK deficiency, change of L‐type PK was similar to that of normal subjects whereas M2‐type PK was clearly seen even at the later stage of maturation as in classical‐type PK deficiency. The present studies indicate that compensatory M2‐type PK production occurs in the erythroblasts of patients wit

ISSN:0361-8609    

DOI:10.1002/ajh.2830160107

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

年代:1984

数据来源: WILEY

摘要:

AbstractEighteen patients with acute nonlymphocytic leukemia (ANLL), aged 17–73 years, were treated with high‐dose cytosine arabinoside (HD‐Ara‐C)) using 3 g/m2IV q 12 hours × 12 doses. Seven patients were treated for relapse and four (57%) obtained a complete remission with a median duration of 19.5 weeks. In nine patients, refractory to conventional chemotherapy, no complete responders were observed. Treatment failure was most commonly due to drug resistance. Two elderly patients with ANLL not previously exposed to chemotherapy died during the initial induction. Recent data on the HD‐Ara‐C experience in ANLL are presented and compared wit

ISSN:0361-8609    

DOI:10.1002/ajh.2830160108

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

年代:1984

数据来源: WILEY

摘要:

AbstractT‐cell subsets were determined by the Leu monoclonal antibodies in the peripheral blood and/or bone marrow of 52 patients with B‐cell chronic lymphocytic leukemia (B‐CLL) not on therapy at the time of study. The diagnosis of B‐CLL required that the leukemic cells expressed surface receptors for “la‐like” antigen, Fc fragment of IgG, mouse red blood cells (MRBC), C3‐coated red cells (EAC), and low density of monoclonal surface immunoglobulin. The Leu‐3a+/2a+ratio was applied to define the balance between the helper/suppressor subsets in the residual T‐lymphocytes. Most patients showed a decrease in the Leu‐3a+/2a+ratios at all stages of disease. The decrease in ratio was mainly related to a decrease in the Leu‐3a+T‐cell subset. The more advanced stages of B‐CLL were associated with lower Leu‐3a+/2a+ratio, higher total white cell and percent lymphocyte counts. There was no correlation between the proportion of EAC or MRBC rosetting cells and stages of B‐CLL. This analysis further suggests that B‐CLL is an immunosuppressed state that becomes more pronounced in the advanced stages and is characterized by a progressive decrease in

ISSN:0361-8609    

DOI:10.1002/ajh.2830160109

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

年代:1984

数据来源: WILEY

摘要:

AbstractA patient with paroxysmal nocturnal hemoglobinuria of 14 years duration presented with severe abdominal pain and fever. On admission, his hematocrit had fallen to 19% from his usual level of approximately 30%, and stools were positive for occult blood. Dilated loops of small bowel with air fluid levels were noted on radiographs, and sonography revealed free intraperitoneal fluid. Several sections of gangrenous jejunum and ileum were resected at exploratory laparotomy, and mesenteric venous thromboses were present. This is the second report of pathologically proven intestinal infarction in a patient with paroxysmal nocturnal hemoglobinuria who has survived this abdominal emergency with surgical intervention. Although abdominal pain in patients with paroxysmal nocturnal hemoglobinuria has frequently been attributed to mesenteric venous thromboses, this has rarely been documented either during life or at autopsy.

ISSN:0361-8609    

DOI:10.1002/ajh.2830160110

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

年代:1984

数据来源: WILEY

摘要:

AbstractAn 8‐month‐old male with acute monoblastic leukemia died during induction chemotherapy of severe bleeding refractory to repeated infusions of platelets and clotting factors. A heparin effect was suggested by prothrombin time (PT) of 26 seconds, partial thromboplastin time (PTT) of 94 seconds, thrombin time 240 seconds, and reptilase time 18.4 seconds, with a fibrinogen of 88 mg/dl. Both plasma mixed with the patient's urine and the patient's plasma had their thrombin times corrected toward normal by both PF4 and protamine. Synergism of of the anticoagulant with antithrombin III was demonstrated not only by enhanced inhibition of thrombin but also by an increased rate of formation of thrombin‐antithrombin III complexes in the presence of the anticoagulant, which was eliminated by preincubation with heparinase. Since the anticoagulant activity was not found in the blasts themselves, it is presumed that the anticoagulant is heparin/heparan liberated from the endothelial lining by products of the cell destruction secondary to chemoth

ISSN:0361-8609    

DOI:10.1002/ajh.2830160111

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

年代:1984

数据来源: WILEY