Abstract:
:Although treatment with plasma exchange increased the survival of patients with thrombotic thrombocytopenia purpura to 80% in the 1980s, no further increase of survival occurred over the next 20 years. However, more consistent use of adjuvant treatment with corticosteroids and rituximab in recent years has begun to further increase survival as well as decrease the frequency of relapse. With adjuvant treatment, durable remissions can be achieved more quickly, requiring fewer days of plasma exchange. Fewer days of plasma exchange have resulted in fewer complications, such as central venous catheter-related systemic infections. Future potential options for adjuvant treatment, recombinant ADAMTS13 to correct severe ADAMTS13 deficiency, and agents to block von Willebrand factor-mediated platelet thrombosis are being investigated.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
George JNdoi
10.1002/ajh.23126subject
Has Abstractpub_date
2012-05-01 00:00:00pages
S88-91eissn
0361-8609issn
1096-8652journal_volume
87 Suppl 1pub_type
杂志文章,评审abstract::An 18-year-old girl with a preoperative platelet count of 216,000/cmm received 3 units of stored plasma at the time of surgery. Within 6 hours her platelet count had fallen to 5,000/mm3 and hemorrhagic manifestations appeared. One of the plasma donors was found to be P1A1 negative with an anti P1A1 antibody. The recip...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830250213
更新日期:1987-06-01 00:00:00
abstract::The hereditary persistence of fetal hemoglobin (HPFH)-6 is sporadically found in Thailand whereas the deletion-inversion type (G)gamma((A)gamma delta beta)(0)-thalassemia is described among Indians. We report a hitherto un-described case in which these two defects co-segregate. He was a 3-year-old Thai boy who had a f...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10202
更新日期:2002-10-01 00:00:00
abstract::Single agents have demonstrated activity in relapsed and refractory (R/R) peripheral T-cell lymphoma (PTCL). Their benefit relative to combination chemotherapy remains undefined. Patients with histologically confirmed PTCL were enrolled in the Comprehensive Oncology Measures for Peripheral T-cell Lymphoma Treatment (C...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.25463
更新日期:2019-06-01 00:00:00
abstract::Phosphatidylinositol-3-kinase (PI3K) signaling plays a crucial role in oncogene-mediated tumor growth and proliferation. Buparlisib (BKM120) is an oral pan-class I PI3K inhibitor. This phase I study was conducted to determine the dose limiting toxicity (DLT) and maximum tolerated dose (MTD) of BKM120 in patients (pts)...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24568
更新日期:2017-01-01 00:00:00
abstract::The presence of teardrop-shaped red cells in peripheral blood has traditionally been felt to reflect altered marrow architecture, namely myelofibrosis. We evaluated two patients with splenomegaly, moderately severe hemolytic anemia due to warm-reactive IgG anti-red cell autoantibody, and bone marrow erythroid hyperpla...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830210410
更新日期:1986-04-01 00:00:00
abstract::New treatments have transformed multiple myeloma into a chronic disease. Hence, optimal management of treatment and disease-related complications remains a critical component of survivorship care. Survivorship care model in cancers requiring a fixed-duration therapy may not be applicable to myeloma, since patients are...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25764
更新日期:2020-06-01 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::We molecularly characterized sixty-seven cases of Hb H disease by the polymerase chain reaction. The strategy depends on amplifying the alpha-thalassemia-1 (alpha-thal-1) gene by primers flanking the breakpoint and sequence differences of the 3' end of the alpha-globin gene and the nonhomologous elements I, II, and II...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450405
更新日期:1994-04-01 00:00:00
abstract::We studied red blood cell morphology in two marathon runners before, immediately after, and 1 day after a marathon race. A discocyte-stomatocyte transformation was found by light microscopy of wet preparations and also by scanning electron microscopy, with about one-half the erythrocytes becoming cup-shaped after the ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830190213
更新日期:1985-06-01 00:00:00
abstract::We have developed a rapid approach to detect the two most common alpha-thalassemia-2 (alpha-thal-2) determinants by the polymerase chain reaction (PCR) technique, which takes a few hours to complete. Specific oligonucleotides selectively amplify appropriate segments of the chromosome with the deletion and the normal c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460309
更新日期:1994-07-01 00:00:00
abstract::We have generated a murine hybridoma that secretes a monoclonal antibody (mAb) that is highly specific for hemoglobin C (HbC) [alpha 2 beta 2 6(A3)Glu----Lys] and shows no cross reactivity with HbA, HbA2, HbF, HbS, HbE, or Hb O-Arab. Using this antibody, we developed a simple and rapid enzyme linked immunosorbent assa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830330308
更新日期:1990-03-01 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::We measured the plasma level of fibrinogen in 560 patients with disseminated intravascular coagulation (DIC) and evaluated its relationship with outcome and with other hemostatic markers. Forty-seven percent of patients had >200 mg/dL of plasma fibrinogen and 24% had <100 mg/dl of plasma fibrinogen, suggesting that pl...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10249
更新日期:2003-01-01 00:00:00
abstract::Between 1980 and 1988, 126 patients with leukemia were treated with piperazinedione and fractionated total body irradiation (TBI) followed by allogeneic bone marrow transplantation from HLA matched siblings. Sixty-one patients had acute myelogenous leukemia, 46 acute lymphoblastic leukemia, and 19 chronic myelogenous ...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.2830460205
更新日期:1994-06-01 00:00:00
abstract::A single point mutation of the factor V (FV) gene, leading to the substitution Arg506Gln in the FV molecule (FV-Leiden) and hence resistance to its breakdown by activated protein C (APC), is the most prevalent risk factor for venous thrombosis in the Caucasians. A ratio determined by activated partial thromboplastin t...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199703)54:3<214::aid-ajh7>
更新日期:1997-03-01 00:00:00
abstract::A 67-year-old woman was admitted to our hospital because of lymphadenopathy and lymphocytosis. Monoclonal integration of HTLV-I provirus DNA was detected, and a diagnosis of adult T-cell leukemia (ATL) was made. Flow cytometry revealed that the ATL cells expressed CD20 as well as T-cell-associated antigens, and expres...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200101)66:1<39::AID-AJH1005>3.0.
更新日期:2001-01-01 00:00:00
abstract::As the number of anticoagulant drugs increases and new ones are brought to market, the utility of the routine screening coagulation tests of today--namely the prothrombin time and activated partial thromboplastin time--will be significantly reduced in many clinical situations. Although the new anticoagulants are desig...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22222
更新日期:2012-02-01 00:00:00
abstract::Platelet antibodies are detectable in only about 50% of patients with chronic autoimmune thrombocytopenia (AITP). We determined platelet antibodies against GPIa/IIa, GPIb/IX, GPIIb/IIIa, and GPV and reticulated platelets in three female patients with AITP, before and after immunoadsorption treatment. None of the three...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10312
更新日期:2003-05-01 00:00:00
abstract::The role of leukapheresis was explored in the management of anaemia and thrombocytopenia complicating the accumulation of lymphoid cells in patients with chronic lymphocytic leukaemia and lymphoma. The aim of this study was to determine the efficiency of this procedure in correcting these complicating features and to ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830060411
更新日期:1979-01-01 00:00:00
abstract::Hepcidin is a small defensin-like peptide whose production by hepatocytes is modulated in response to anemia, hypoxia, or inflammation. Hepcidin could also act as an indicator of functional iron deficiency in these patients. Cross-sectional study was performed to assess hepcidin correlations with renal function, iron ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20657
更新日期:2006-11-01 00:00:00
abstract::An infectious episode is the presenting manifestation of hairy cell leukemia (HCL) in approximately 30% of cases. Most often this is bacterial and only rare cases of opportunistic fungal infection are described. We report a patient who presented with sporotrichal involvement of multiple cutaneous sites and lymph nodes...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460214
更新日期:1994-06-01 00:00:00
abstract::Identification of genetic causes of neutropenia informs precision medicine approaches to medical management and treatment. Accurate diagnosis of genetic neutropenia disorders informs treatment options, enables risk stratification, cancer surveillance, and attention to associated medical complications. The rapidly expa...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25374
更新日期:2019-03-01 00:00:00
abstract::Six patients with thrombotic microangiopathy associated with drug therapy had serial analyses of von Willebrand factor (vWF) multimeric patterns in their EDTA-plasma samples by sodium dodecyl sulfate-1% agarose gel electrophoresis and autoradiography. In the plasma of five patients (one with chronic myelogenous leukem...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830420306
更新日期:1993-03-01 00:00:00
abstract::Celecoxib, a specific cyclooxygenase-2 (Cox-2) inhibitor, has been shown to possess antitumor activity in a variety of cancer cells. However, the antitumor activity of celecoxib in hematopoietic tumors, especially in chronic myeloid leukemia (CML), has not been well established. This study was designed to investigate ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20542
更新日期:2006-04-01 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::The acquired JAK2 V617F mutation is common in patients with myeloproliferative neoplasms. We previously showed that JAK2 V617F is also found in coronary patients, most of them affected by coronary atherosclerosis. Peripheral arterial disease (PAD) is another important manifestation of atherosclerosis. However, prevale...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23874
更新日期:2015-01-01 00:00:00
abstract:DISEASE OVERVIEW:Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). DIAGNOSIS:The diagnosis of MF or SS requires the integration of clinical and histopathologic data. ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23756
更新日期:2014-08-01 00:00:00
abstract::Selective labeling of phagocytes in human blood can be achieved by incubation of unseparated, heparinized blood with 99mTc-sulfur colloid. We have developed a method for quantitation of granulocyte chemotaxis using granulocytes labeled by this technique. The method was found to be more accurate and less tedious to per...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830030402
更新日期:1977-01-01 00:00:00
abstract::The activation pathways for the generation of enzymes involved in blood clotting, clot lysis, complement activation, and kinin generation are briefly reviewed. The interrelationship of the four systems is illustrated by the multiple functions of four key enzymes: Factor XIIa, kallikrein, plasmin, and C1 esterase. The ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830040412
更新日期:1978-01-01 00:00:00
abstract::Polycythemia vera (PCV) and multiple myeloma are both clonal disorders of hematopoietic stem cells. The simultaneous occurrence of these diseases in an individual patient is rare. A case of synchronous PCV and smoldering myeloma is presented and the literature is reviewed. The issues of clinical importance in this unu...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830440311
更新日期:1993-11-01 00:00:00