摘要:

AbstractIn many patients with low serum levels of vitamin B12, the absorption of the free vitamin has been normal. The present study, using a total body counter57CoB12absorption method that clearly separated those with intrinsic factor deficiency from controls, found that of 94 patients with low B12levels and intact stomachs in whom the absorption of free and bound B12was determined, 44 (47%) had normal absorption of both. However, 20 of the 94 (21%) with normal absorption of free B12had low absorption of bound B12. The remainder (32%) had low absorption of both free and bound B12. All patients with high serum gastrin levels had low bound B12absorption, but so did 21% of those patients with normal serum gastrin levels. © 1992 Wiley‐Liss, I

ISSN:0361-8609    

DOI:10.1002/ajh.2830400302

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

年代:1992

数据来源: WILEY

摘要:

AbstractPlasma cytokine levels were examined in 13 patients with thrombotic thrombocytopenic purpura (TTP). Auto‐antibodies, platelet‐associated immunoglobulin G, and platelet aggregating factor were detected in many of these patients and high‐molecular‐weight bands of von Willebrand factor multimers were reduced in 9 of 10 patients examined. Complete remission (CR) was attained in 7 of the 13 patients, but 6 died. Tumor necrosis factor (TNF), Interleukin (IL)‐1β, IL‐6, and soluble IL‐2 receptor showed marked increases at onset and decreased at CR. The prognosis tended to be poor in patients with increased IL‐6 and soluble IL‐2 receptor levels. These findings suggest that immunological mechanisms, such as the activation of macrophage, are involved in the pathogenesis of TTP and are reflected in the plasma cytokine levels. © 1

ISSN:0361-8609    

DOI:10.1002/ajh.2830400303

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

年代:1992

数据来源: WILEY

摘要:

AbstractHigh‐resolution two‐dimensional gel electrophoresis (2‐DGE) was used to analyse plasma samples and partially purified cold agglutinins (CA) obtained from two selected patients. Both presented an acute hemolytic anemia with CA of high thermal amplitude, normal immunoglobulin levels, no detectable paraproteinemia, and no clinical evidence of a malignant B‐cell disorder. The electrophoretograms of their plasma showed evident alterations of the “normal” protein profile, which were directly related to hemolysis (absence of the spots of haptoglobin and in one case of those of hemopexin), but no monoclonal gammopathy. The electrophoretograms of their purified CA revealed two clearly different spot patterns respectively corresponding to a monoclonal IgM and to polyclonal IgM. These results show that the clonality of CA associated with hemolytic anemia can be easily determined by 2‐DGE. This technique may be very useful to discriminate chronic cold agglutinin disease in the early phase from “parainfectious” CA. © 19

ISSN:0361-8609    

DOI:10.1002/ajh.2830400304

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

年代:1992

数据来源: WILEY

摘要:

AbstractThe use of ibuprofen (IBP) in hemophilic men for chronic hemophilic arthropathy is associated with transient coagulation abnormalities, but usually does not cause bleeding symptoms. However, when hemophilic men are treated with ibuprofen while also receiving zidovudine (ZDV), excess bleeding has occurred in some. In order to evaluate platelet function and pharmacokinetics of combination IBP and ZDV, we measured platelet aggregation, platelet adhesive index, bleeding time, and IBP and ZDV drug levels by high performance liquid chromatography on five patients receiving chronic oral IBP, 400 mg every 6 hr, and on ten patients receiving both IBP and ZDV, 100 or 200 mg every 4 hr five times daily. Samples were obtained at baseline (ZDV alone), acutely (IBP + ZDV acutely), and chronically (2 weeks on IBP and ZDV). Abnormal platelet aggregation with arachidonic acid occurred in four of five (80%) of those receiving IBP alone and in 7 of 10 (70%) at baseline, 9 of 10 (90%) at acute, and 8 of 10 (80%) at chronic IBP and ZDV treatment, most commonly at 2 hr following dosing, when peak IBP levels occurred, and persisting 4 hr in those on chronic dosing. Half or more of those on combination IBP + ZDV showed a lowered platelet adhesive index and/or prolonged bleeding time. Excess bleeding symptoms occurred in three on chronic combination IBP + ZDV, two with increased frequency of spontaneous hemorrhages, and one with prolonged traumatic bleeding. Bleeding tendency was unrelated to the degree of platelet function abnormality, to peak drug levels of IBP or ZDV, or degree of liver function abnormality (SGPT). The clearance of IBP alone and ZDV alone were not different from the clearance of combination IBP and ZDV. Caution is urged regarding potential enhanced bleeding tendency in hemophiliacs receiving both IBP and ZDV in combination. © 1992 Wiley‐Liss, I

ISSN:0361-8609    

DOI:10.1002/ajh.2830400305

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

年代:1992

数据来源: WILEY

摘要:

AbstractThe CALGB prospectively studied 140 adult acute lymphoblastic leukemia (ALL) patients for cytogenetic abnormalities. Seven (5%) patients with adequate cytogenetic preparations had t(8;14)(q24;q32) or t(8;22)(q24;q11). Patients were compared with non‐8q24 patients for clinical and laboratory characteristics, response to therapy, and survival. The median age of patients with translocations involving 8q24 (71% males) was 40 years. Forty‐three percent had lymphadenopathy, 29% splenomegaly, and 29% hepatomegaly. None exhibited central nervous system (CNS), skin, or gum involvement. These features did not differ significantly from non‐8q24 ALLs. Patients with 8q24 translocations had higher hemoglobins (11.5 vs. 9.8 g/dl;P= 0.04) and lower percentage of blasts in the peripheral blood (8.5% vs. 69%;P= 0.007). Although all seven were finally categorized as ALL‐L3, a marked variation in the proportion of typical L3 blasts was observed that initially resulted in the diagnoses of ALL‐L2 in three cases and prolymphocytic leukemia in one. In five of five patients, the blasts typed as B cells (Slg+ and CD19+). Complete remission rates for patients with 8q24 translocations were 43%, whereas they were 68% for non‐8q24 ALLS (P= 0.22). Furthermore, patients with 8q24 abnormalities exhibited significantly shorter survival (4.8 vs. 18.4 mo;P<0.001). We conclude that ALL with translocations of 8q24 in adults shows a mature B‐cell immunophenotype (Slg+), poor prognosis and morphology ranging from classical ALL‐L3 to ALL with a subpopulation of L3 cells. Thus, the diagnosis of ALL‐L3 should be made when blastic cells possess a mature B‐cell immunophenotype (SIg+) and an 8q24 translocation, even though the number of L3 cells is low. ©

ISSN:0361-8609    

DOI:10.1002/ajh.2830400306

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

年代:1992

数据来源: WILEY

摘要:

AbstractWe have identified a patient with von Willebrand's disease (vWD) resembling type IIB vWD, with increased ristocetin induced platelet aggregation (RIPA), the absence of the large multimers of von Willebrand factor (vWF) in plasma, and the presence of the large multimers in platelets in whom a family study indicated a probable double heterozygous inheritance pattern. The propositus was a 12‐year‐old boy with frequent epistaxis and bruising. Abnormal hemostatic findings included a prolonged bleeding time (BT), decreased levels of factor VIII coagulant activity (VIIIC), von Willebrand factor antigen (vWF:Ag), ristocetin cofactor (RCof), and an increased RIPA. In the presence of ristocetin, binding of the patient's plasma vWF to normal platelets was increased but binding of normal vWF to his platelets was normal. SDS‐agarose gel (1.5%) electrophoresis revealed that plasma vWF lacked the large multimers, and 3.0% gel electrophoresis revealed that the multimers had a 5‐band pattern similar to normal. The above findings were consistent with type IIB vWD, but 1‐deamino[8‐D‐arginine]‐vasopressin (DDAVP) infusion resulted in a shortened BT and the transient appearance of large multimers without a decrease in the platelet count. Family studies revealed that his mother has mild bleeding symptoms, decreased VIIIC, vWF:Ag, and RCof levels and normal to slightly reduced RIPA with a multimer pattern consistent with type I vWD. In contrast, the father, sister, and paternal grandfather were asymptomatic, with a slightly decreased VIIIC level but a normal BT and vWF:Ag and RCof levels. Their RIPA and vWF binding to normal platelets were increased, but unlike the propositus their plasma contained large multimers. We concluded that the propositus is a type IIB‐like variant differing from previously reported IIB variants in two ways: 1) his response to DDAVP and 2) a possible double heterozygous mode of inheritance rather than the usual dominant route. © 19

ISSN:0361-8609    

DOI:10.1002/ajh.2830400307

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

年代:1992

数据来源: WILEY

摘要:

AbstractThe present epidemiological study of the molecular characteristics of β‐thalassemia in Sicily was prompted by the disparate phenotypic expression (in clinical status and absolute HbF level) observed in two β‐thalassemic homozygotes who were also homozygous for the β‐like globin gene cluster haplotype III. We suspected that polymorphismswithinhaplotype III could be the cause for the discrepancy. Based on the association of particular conformations of the (AT)xTymotif (‐540 5′ to the β gene) with milder forms of thalassemia and sickle cell anemia, 38 homozygous β‐thalassemia patients were studied to define their haplotypes, the −158 site 5′ to theGγ gene (linked to haplotype III) and the structure of the (AT)xTymotif. We found that the patient who was phenotypically mild and homozygous for β‐thalassemia, haplotype III, and the −158 C→T mutation was homozygous for the rare (AT)9T5motif. In contrast, the patient homozygous for β‐thalassemia, haplotype III, and the —158 mutation, but exhibiting a severe clinical course, was homozygous for the (AT)7T7configuration. Others have suggested that (AT)9T5is a negative regulatory protein binding sequence, and it is a silent carrier state for β‐thalassemia. The usual configuration (AT)7T7, has considerably less affinity for regulatory protein binding, and it is the most common configuration in Sicilian β‐thalassemics (67 of the 78 chromosomes studied). Within the 38 patients studied, seven were informative because they had various combinations of the (AT)9T5and (AT)7T7motif, and the −158 C→T mutation. The results in these patients suggest thatonlytheco‐presence of the (AT)9T5configuration and a C→T change at −158.5′ to theGγ gene is associated with high HbF expression and a mild clinical phenotype. We postulate that these two regions of the β‐like globin gene cluster interact, when endowed with the proper sequences, to enhance the expres

ISSN:0361-8609    

DOI:10.1002/ajh.2830400308

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

年代:1992

数据来源: WILEY

摘要:

AbstractImmunopathic thrombocytopenic purpura (ITP) can be a life‐threatening complication of human immunodeficiency virus (HIV) infection in patients with hemophilia and can seriously compromise quality of life if not managed effectively. We report here complete response to splenectomy in four severe hemophiliacs with HIV‐associated ITP. All patients were symptomatic, had platelet counts less than 25,000/mm3, and had failed at least one non‐surgical therapy prior to splenectomy. All patients tolerated surgery well and obtained an immediate and durable complete response. In addition to our experience, a review of the literature shows that splenectomy is well tolerated and provides the most effective long‐term solution for hemophiliacs with HIV‐ITP. © 1992 Wiley

ISSN:0361-8609    

DOI:10.1002/ajh.2830400309

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

年代:1992

数据来源: WILEY

摘要:

AbstractThe use of plasma‐derived coagulation factor concentrates has been marked by the transmission of viral agents. Infusions of factor IX complex concentrates have been additionally complicated by inappropriate thrombosis. Use of these concentrates in the neonate, in those with liver disease, and in surgical patients results in increased risk for this complication. Twenty patients have been infused with a purified coagulation factor IX concentrate for fall‐off and recovery studies. A two‐compartment model indicated an initial phase half‐life of 4.06 ± 2.86 hr and a β phase half‐life of 20.0 ± 3.8 hr following the administration of AlphaNine®, Coagulation Factor IX (Human). In vivo recovery was 62.7% ± 13.8%, with an average factor IX coagulant level of 73% ± 16% at 15 min after the infusion of a mean dose of 45 U/kg. Thirteen previously transfused patients with hemophilia B underwent major orthopedic or general or dental surgery using this purified factor IX. Operative outcomes were excellent in all patients. No excessive bleeding was noted. There was no laboratory or clinical evidence for a disseminated intravascular coagulopathy. The excellent surgical outcomes observed in this multitransfused group with biochemical evidence for active liver disease demonstrates the utility and safety of a purified coagulation factor IX concentrate. © 1992

ISSN:0361-8609    

DOI:10.1002/ajh.2830400310

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

年代:1992

数据来源: WILEY

摘要:

AbstractWe present two cases of May‐Hegglin anomaly incidentally discovered in a patient and his brother during investigation of the patient for end‐stage renal failure and workup for renal transplantation. Routine laboratory tests were performed and included a basically normal clotting profile. Ultrastructural studies of the May‐Hegglin inclusions proved diagnostic, findings were compared with those of two similar granulocyte inclusion bodies, and nomenclature discrepancies that still exist in most references are again emphasized. The finding of the May‐Hegglin anomaly in our patient appears to be incidental to the underlying renal disease. A successful renal transplant has been carried out in this patient.We now report on a patient and his brother in which the MHA was discovered during workup of the patient for end‐stage renal failure and renal transplantation. No association between the underlying renal disease and the MHA could be demonstrated. © 1992 Wiley

ISSN:0361-8609    

DOI:10.1002/ajh.2830400311

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

年代:1992

数据来源: WILEY