Abstract:
:A 71-year-old woman and her elder sister developed ligneous conjunctivitis after ocular surgery. Laboratory tests demonstrated that the proband and her sister had 6.6% and 8.1% of plasminogen activity, and 1.2 and 1.4 mg/dl of antigen, respectively. Thus, they were diagnosed as having severe type I plasminogen deficiency, for the first time, in Japan. DNA sequencing and PCR-RFLP analyses revealed that these two cases are homozygotes of a novel A-to-G mutation at the obligatory splicing acceptor site in intron-C. Both cases were satisfactorily treated with a direct thrombin inhibitor, topical Argatroban, and topical plasma obtained from their healthy family members.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Suzuki T,Ikewaki J,Iwata H,Ohashi Y,Ichinose Adoi
10.1002/ajh.21402subject
Has Abstractpub_date
2009-06-01 00:00:00pages
363-5issue
6eissn
0361-8609issn
1096-8652journal_volume
84pub_type
杂志文章abstract::This 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...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830500307
更新日期:1995-11-01 00:00:00
abstract::The relationship between thrombocytopenia and the level of anticardiolipin antibodies (aCL) and/or the existence of lupus anticoagulant (LA) ware studied in 146 patients with systemic lupus erythematosus (SLE). These patients were divided into six groups: A, those LA positive with a high level of aCL (>10 U/ml) (10 ca...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199805)58:1<55::aid-ajh10>
更新日期:1998-05-01 00:00:00
abstract::Patients with indolent non-Hodgkin lymphoma (NHL) have multiple treatment options yet there is no consensus as to the best initial therapy. Lenalidomide, an immunomodulatory agent, has single agent activity in relapsed lymphoma. This trial was conducted to assess feasibility, efficacy, and safety of adding lenalidomid...
journal_title:American journal of hematology
pub_type: 杂志文章
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更新日期:2017-05-01 00:00:00
abstract::We report a patient with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) who presented with rapid enlargement of a cervical lymph node due to localized herpes simplex lymphadenitis, which was clinically indistinguishable from large cell (Richter's) transformation. The diagnosis was made by excisional...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10006
更新日期:2001-12-01 00:00:00
abstract::Pulmonary hypertension (PH) of sickle cell disease (SCD), as defined by a tricuspid regurgitant jet velocity by echocardiogram of >or=2.5 m/sec, occurs in approximately 1/3 of HbSS adults and is an independent risk factor for mortality. Although studies of the past few years have greatly expanded our knowledge of the ...
journal_title:American journal of hematology
pub_type: 社论
doi:10.1002/ajh.21083
更新日期:2008-01-01 00:00:00
abstract::Hemoglobin Lyon-Bron was found in two members of a family of German ascent presenting with a moderate normocytic anemia. In this alpha 2 globin variant, the N-terminal valine of the chain was replaced by an alanine. Electrospray mass spectrometry of the alpha chain showed that, as normally, the initiator methionine wa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10051
更新日期:2002-03-01 00:00:00
abstract::Secondary acute myeloid leukemia (sAML) traditionally has inferior outcomes compared to de novo AML. Allogeneic hematopoietic cell transplantation (HCT) is the sole potentially curative therapy. This study analyzes the outcomes for unmanipulated haploidentical HCT (haploHCT) for sAML using the Acute Leukemia Working P...
journal_title:American journal of hematology
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更新日期:2018-06-01 00:00:00
abstract::CD19+CD5+ lymphocytes constitute a minority of peripheral blood B cells. In view of the importance of these cells in the pathogenesis of the immunoregulation of myeloma, their incidence in another lymphoid organ was determined. CD5+ B cells were studied in 9 spleens from patients with multiple myeloma and in 10 spleen...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370306
更新日期:1991-07-01 00:00:00
abstract::The pharmacokinetics of factor VIII were studied in a series of 20 hemophilia-A patients undergoing surgery. Regardless of the type of operation, elimination of factor VIII was shown to be increased only in ten cases (50%) during the post-operative period. In this subgroup of patients, factor VIII half-life, measured ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830300305
更新日期:1989-03-01 00:00:00
abstract::Fifteen recently diagnosed patients with acute leukemias admitted for induction chemotherapy were selected for study. When thrombocytopenic (venous platelet count less than 20 X 10(9)/l) these patients received prophylactic platelet transfusions. A total of 67 platelet transfusion therapies were administered and evalu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830110305
更新日期:1981-11-01 00:00:00
abstract::Our objectives were to measure and compare plasminogen activator inhibitor levels (PAI-1) in primary adult thrombotic thrombocytopenic purpura (TTP) and in secondary TTP associated with bone marrow transplantation (BMT)-TTP. PAI-1 antigen levels were measured by an enzyme linked immunosorbent assay on platelet poor pl...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/(sici)1096-8652(199809)59:1<9::aid-ajh3>3.
更新日期:1998-09-01 00:00:00
abstract::We retrospectively measured various hemostatic markers in 240 patients with disseminated intravascular coagulation (DIC) before the onset of DIC and in 110 non-DIC patients, and examined their usefulness for the diagnosis of pre-DIC. Changes in prothrombin time ratio and fibrinogen levels were not significant before t...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199904)60:4<273::aid-ajh4>
更新日期:1999-04-01 00:00:00
abstract::Immune thrombocytopenia is frequently encountered in medical practice and is generally accepted as being caused by an IgG antibody. The capability of detecting platelet-bound IgG as a diagnostic and therapeutic modality is critical for appropriate care and management of patients with idiopathic thrombocytopenic purpur...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830090402
更新日期:1980-01-01 00:00:00
abstract::Polycythemia vera (PV) is a myeloproliferative disorder characterized by thrombotic and, less often, bleeding complications. Many mechanisms have been advanced to explain the occurrence of these complications, none of them satisfactory. We examined a cohort of 27 patients with PV, secondary erythrocytosis, and essenti...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199903)60:3<196::aid-ajh5>
更新日期:1999-03-01 00:00:00
abstract::Deformability of normal and sickle erythrocytes was measured by means of micropipette elastimetry with determination of intrinsic membrane rigidity (P) and total cell deformability (Pt). In the elastimetric technique employed, negative pressure at the pipette tip was generated and measured continuously. Membrane rigid...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830040103
更新日期:1978-01-01 00:00:00
abstract::Acquired α-thalassemia myelodysplastic syndrome (MDS) (ATMDS) is an acquired syndrome characterized by a somatic point mutation or splicing defect in the ATRX gene in patients with myeloid disorders, primarily MDS. In a large MDS patient series, the incidence of ATMDS was below 0.5%. But no large series has yet assess...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24073
更新日期:2015-08-01 00:00:00
abstract::Venous thromboembolism (VTE) recurs frequently. Greater height is associated with increased risk of incident VTE, but it is unclear whether height is related to risk of VTE recurrence. Recurrent VTE is associated with substantial morbidity and mortality, thus identifying individuals at greatest risk of experiencing a ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23199
更新日期:2012-07-01 00:00:00
abstract::Nongastric marginal zone B-cell lymphoma (NG-MZL) is a relatively uncommon indolent lymphoma. From 1990 to 2005, a total of 247 patients with histologically confirmed NG-MZL were analyzed. Ann Arbor stage I/II disease was present in 78% (167 out of 215). One hundred eighty-six patients out of two hundred eight were ca...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20874
更新日期:2007-06-01 00:00:00
abstract::Neonatal alloimmune thrombocytopenia is an uncommon but important cause of thrombocytopenia in infants. Because of the severity of the thrombocytopenia, some of these infants will have intracranial hemorrhage with resultant long-term disability. Obstetricians and neonatologists have recommended delivery by caesarean s...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280207
更新日期:1988-06-01 00:00:00
abstract::A case of red cell adenosine deaminase (ADA) overproduction associated with hereditary hemolytic anemia is reported here. This appears to be the second report. Proband is a 38-year-old Japanese male who had hemoglobin, 15.8 g/100 ml; reticulocyte count, 4.5%; serum indirect bilirubin, 4.9 mg/100 ml; 51Cr-labeled red c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830050205
更新日期:1978-01-01 00:00:00
abstract::Tumor cells of 14 cases of non-Hodgkin lymphomas and 2 cases of Hodgkin disease were tested for the presence of the transferrin receptor (CD71) by flow cytofluorimetry before 67gallium imaging. It appeared that expression of CD71 phenotype was closely related to the positivity of gallium scan before therapy. We feel t...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830360313
更新日期:1991-03-01 00:00:00
abstract::Forty-two patients with chronic lymphocytic leukemia (CLL) were studied for morphology of lymphocytes by light and electron microscopy (EM), in vitro responses of lymphocytes to a battery of physical and chemical agents, overall clinical status, immunologic status, course, and response to therapy. CLL lymphocytes coul...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830020110
更新日期:1977-01-01 00:00:00
abstract:DISEASE OVERVIEW:Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 9,000 new patients annually and representing approximately 11% of all lymphomas in the United States. DIAGNOSIS:HL is composed of two distinct disease entities; the more commonly diagnosed classical HL and the rare nodular lymph...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23348
更新日期:2012-12-01 00:00:00
abstract::Among 47 patients with thrombotic thrombocytopenic purpura (TTP), 8 patients were diagnosed to have postoperative-TTP. Two patients underwent vascular surgery, 5 patients coronary artery bypass grafts, and 1 patient resection of myocardial sarcoma. Prior to surgery, all patients except one had normal hemograms and pla...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199609)53:1<11::AID-AJH3>3
更新日期:1996-09-01 00:00:00
abstract::Systemic light-chain (AL) amyloidosis may be associated with acquired factor X (FX) deficiency and optimal management of this coagulopathy is unknown. We reviewed our experience with 60 patients with isolated FX deficiency (< or =50%) due to AL amyloidosis that underwent an invasive procedure between 1975 and 2007. Th...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21603
更新日期:2010-03-01 00:00:00
abstract::Iron refractory iron deficiency anemia (IRIDA) is a rare hereditary disease caused by mutations in TMPRSS6 gene encoding Matriptase-2, a negative regulator of hepcidin transcription. Up to now, 53 IRIDA patients from 35 families with different ethnic origins have been reported and 41 TMPRSS6 mutations have been identi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23929
更新日期:2015-04-01 00:00:00
abstract::Seventy-three patients with hereditary spherocytosis (HS) (58 nonsplenectomized, 15 splenectomized) were studied to evaluate iron status and the adequacy of iron availability for erythropoiesis. Splenectomized patients, who had hemoglobin levels in the normal or upper normal range, had higher levels of serum iron, tra...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830310202
更新日期:1989-06-01 00:00:00
abstract::The effect of several iron chelators on iron uptake and release by mouse peritoneal macrophages has been investigated. The 1,2-dimethyl (L1) and 1-ethyl-2-methyl (L1NEt) derivatives of 3-hydroxypyrid-4-one markedly enhanced iron mobilisation from macrophages pulsed with 59Fe-transferrin-antitransferrin immune complexe...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830340106
更新日期:1990-05-01 00:00:00
abstract::We report a family with a dominantly transmitted syndrome resembling Fanconi's anemia and spanning two generations. This syndrome was characterized by an ill-defined hematologic stem cell disorder, immune dysfunction, poor dentition, hyperpigmented skin, warts, and multiple second trimester spontaneous abortions and i...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830320402
更新日期:1989-12-01 00:00:00
abstract::Disseminated intravascular coagulation (DIC) is a complex acquired coagulopathy resulting from excessive thrombin formation. Abnormal tissue factor (TF) expression is a major mechanism initiating DIC in many disorders, including obstetrical complications, sepsis, cancer, and trauma. Numerous laboratory tests are avail...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/(sici)1096-8652(199809)59:1<65::aid-ajh13>
更新日期:1998-09-01 00:00:00