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 rigidity is defined as the negative pressure, in mm H2O, required to induce a hemispherical projection of the cell surface into the micropipette, and total cell deformability as the negative pressure required to aspirate the entire cell into the pipette lumen. Membrane rgidity for oxygenated sickle discocytes was not statistically different from that of control normal discocytes, but Pt measurements were significantly higher for sickle than normal discocytes. Irreversibly sickled cells (ISCs) had markedly increased membrane rigidity and whole cell deformability when compared to control normal cells. Mildly deformed ISCs and severely deformed ISCs at ambient pO2, both showed significantly higher mean membrane rigidity values than sickle discocytes and reversibly sickled cells. Sickle and normal discocytes both showed membrane elasticity with reversion to original cell shape following release of the cell from its aspirated position at the pipette tip. ISCs, however, exhibited elastic deformation of the membrane. These studies provide further evidence of progressive alteration of the sickle cell membrane induced by the sickling-unsickling process, culminating in formation of the ISC, and suggest a role for the ISC membrane abnormality in the pathologic rheology of sickle cell disease.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Havell TC,Hillman D,Lessin LSdoi
10.1002/ajh.2830040103subject
Has Abstractpub_date
1978-01-01 00:00:00pages
9-16issue
1eissn
0361-8609issn
1096-8652journal_volume
4pub_type
杂志文章abstract::Although tyrosine kinase inhibitors have redefined the care of chronic myeloid leukemia (CML), these agents have not proved curative, likely due to resistance of the leukemia stem cells (LSC). While a number of potential therapeutic targets have emerged in CML, their expression in the LSC remains largely unknown. We t...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21915
更新日期:2011-01-01 00:00:00
abstract::The effect of heparin on platelet aggregation was systematically examined on platelets in plasma (PRP), as well as on gel-filtered, washed, and formaldehyde-fixed platelets. Results indicate that, although heparin causes a mild potentiation of platelet aggregation in the PRP systems, a significant inhibitory activity ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830170310
更新日期:1984-10-01 00:00:00
abstract:DISEASE OVERVIEW:Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, h...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25819
更新日期:2020-07-01 00:00:00
abstract::The expression of mature B-cell markers and T markers was determined in lymphocytes isolated from the peripheral blood (PBL) of 20 healthy and 51 patients with non-Hodgkin malignant lymphoma (NHL). The disease was classified as newly diagnosed, in remission, or being treated with chemotherapy and of low-, intermediate...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830260203
更新日期:1987-10-01 00:00:00
abstract::The clinical development of lenalidomide (Revlimid™), then pomalidomide (Actimid™) as members of immunomodulatory drugs (IMiDs) for the treatment of multiple myeloma (MM), exemplifies how insight into disease biology can lead to design of effective therapeutic agents. Increased experience and understanding of IMiD's d...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23234
更新日期:2012-12-01 00:00:00
abstract::Corticosteroids are essential and one of the mainstays in the treatment of acute lymphoblastic leukemia (ALL). In vitro assays show that dexamethasone(DXM) is five to six times more cytotoxic to leukemic lymphoblasts than prednisolone (PDN) [1], and the use of DXM as an alternative drug for PDN is an important issue i...
journal_title:American journal of hematology
pub_type: 信件,随机对照试验
doi:10.1002/ajh.21827
更新日期:2010-10-01 00:00:00
abstract::Over a 36-month period, 46 consecutive Mexican mestizos with a clinical marker associated with a primary hypercoagulable state were prospectively assessed by searching for the sticky platelet syndrome (SPS), the activated protein C resistance (aPCR) phenotype, coagulation protein C activity and antigen, coagulation pr...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20233
更新日期:2005-01-01 00:00:00
abstract::Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >/=18 years seen at the Adult Sickle Cell Center at Duke University Medical Center between January 2000 and April 2005 wer...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21569
更新日期:2010-01-01 00:00:00
abstract::Substantial evidence exists indicating T cell abnormalities in chronic lymphocytic leukemia (CLL). There is also evidence that the T cell is an important source of burst promoting activity (BPA) for the peripheral blood (PB) erythroid burst forming unit (BFU-e). We studied the BPA of T cells and response of BFU-e in n...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830160207
更新日期:1984-02-01 00:00:00
abstract::We report a patient with incapacitating POEMS syndrome characterized by serum monoclonal protein, polyneuropathy, organomegaly, endocrinopathy, mesangiocapillary glomerulonephritis, massive ascites formation, and pulmonary hypertension. A dramatic improvement in the clinical condition occurred after administration of ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20051
更新日期:2004-05-01 00:00:00
abstract::Evaluation of pharmacologic agents that stimulate fetal hemoglobin production has been done mainly in baboons and macaques. We investigated whether results in transgenic mice can predict the stimulation of fetal hemoglobin in primates, by testing gamma globin induction in response to a new ribonucleotide reductase inh...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450208
更新日期:1994-02-01 00:00:00
abstract::Recombinant DNA technology and protein engineering are creating hope that we can address ongoing challenges in hemophilia care such as reducing the costs of therapy, increasing the availability to the developing world, and improving the functional properties of these proteins. Technological advances to improve the hal...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23146
更新日期:2012-05-01 00:00:00
abstract::Patients presenting with Hodgkin's disease (HD) may show lung involvement characterized by contiguous spread from ipsilateral hilar lymph nodes. Lung consolidation or noncontiguous pulmonary involvement makes an alternative diagnosis more likely. This report describes a patient with HD in whom concurrent pulmonary bla...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830430211
更新日期:1993-06-01 00:00:00
abstract::Lentiviral modification combined with ex vivo erythroid differentiation was used to stably inhibit RhAG expression, a critical component of the Rh(rhesus) membrane complex defective in the Rh(null) syndrome. The cultured red cells generated recapitulate the major alterations of native Rh(null) cells regarding antigen ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23414
更新日期:2013-05-01 00:00:00
abstract::Despite advances in chemotherapeutic agents, the prognosis for some cancers remains extremely poor, suggesting the need for other treatment modalities. Immunotherapy appears an ideal approach because the mechanisms of tumor cell killing induced by tumor vaccines are different from those from chemotherapy. Various inve...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20415
更新日期:2005-09-01 00:00:00
abstract::Although infectious complications represent a relevant cause of morbidity and mortality in patients with myelofibrosis (MF), little is known about their incidence, outcome and risk factors. We retrospectively evaluated a cohort of 507 MF patients, diagnosed between 1980 and 2014 in five Italian hematology centers, to ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24572
更新日期:2017-01-01 00:00:00
abstract::Immunoglobulin light-chain amyloidosis (AL) is a rare disorder characterized by production of a monoclonal light chain. This insoluble light chain, or a fragment thereof, deposits in tissues as amyloid and results in disruption of organ function and, ultimately, in death. Although melphalan and prednisone are benefici...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/(sici)1096-8652(199906)61:2<115::aid-ajh7>
更新日期:1999-06-01 00:00:00
abstract::Antithrombin III (AT III) was isolated by two procedures using polyethylene glycol-400 (PEG) precipitation as the first stage. The PEG supernatant (PEG-sup) was applied to a heparin-affinity chromatographic system and AT III-heparin cofactor (AT III-HCF) was isolated. The PEG precipitate (PEG-ppt) was separated by a S...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830150104
更新日期:1983-08-01 00:00:00
abstract::Comparison of the chromosome findings obtained on routine examination (10-50 cells) of the marrows from patients with Ph1-positive CML with those based on a large number (110-500 cells) of metaphases in six of these patients, in whom appropriate material was available, revealed the presence of small percentages of ane...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830030202
更新日期:1977-01-01 00:00:00
abstract::Primary mediastinal large B-cell lymphoma (PMBL) is a distinct subtype of diffuse large B-cell lymphoma (DLBCL) that shows overlap with classical Hodgkin lymphoma (CHL) and a favorable prognosis compared to mediastinal gray-zone lymphoma (MGZL). We performed immunohistochemistry on initial diagnostic specimens of 49 c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24485
更新日期:2016-10-01 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::Splenectomy is a time-honoured well established approach for patients with steroid-resistant immune thrombocytopenia (ITP). However, due to the more recent availability of therapeutic options alternative to splenectomy, such as rituximab and agonists of the thrombopoietin-receptor, the choice of second-line therapy is...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23823
更新日期:2014-11-01 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::The levels of adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), lactic dehydrogenase (LDH), and LDH isoenzyme patterns (LD1 to LD5) have been measured in lymphocyte extract from 28 patients with B-chronic lymphocytic leukemia (B-CLL). The activities of ADA, PNP, and LDH have been correlated with two mo...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830270302
更新日期:1988-03-01 00:00:00
abstract::In this study we investigated the possible role of neutrophil (PMN) elastase and its natural inhibitor, alpha1-proteinase inhibitor (alpha1-PI) in the pathogenesis of the pseudoxanthoma elasticum (PXE)-like syndrome which is found in patients with homozygous beta-thalassemia. We studied 30 beta-thalassemia homozygotes...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/(sici)1096-8652(200002)63:2<63::aid-ajh1>3
更新日期:2000-02-01 00:00:00
abstract::Since multiple myeloma (MM) is still not-curable, the management of relapse remains challenging. Given the known efficacy of alkylating agents in MM, we conducted a phase I/II study to test a new three drug combination in which Fotemustine (Muphoran), an alkylating agent of nitrosurea family, was added to bortezomib +...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23358
更新日期:2013-02-01 00:00:00
abstract::Most reports of prognosis and therapy in diffuse small non-cleaved cell lymphoma (DSNCL), an aggressive high-grade non-Hodgkin's lymphoma (NHL) which appears to be of two histopathologic subtypes, have included predominantly a pediatric population and very few adults. We studied 20 newly diagnosed, previously untreate...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460408
更新日期:1994-08-01 00:00:00
abstract::A 49-year-old Caucasian woman presented with features suggestive of thrombotic microangiopathy (TMA). She did not respond to treatment with repeated plasma exchange and corticosteroids. A bone marrow biopsy revealed presence of metastatic carcinoma. A limited autopsy revealed presence of breast cancer with rib metasta...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20783
更新日期:2007-04-01 00:00:00
abstract::Indolent non-hodgkin lymphomas (iNHL) are a rare cause of monoclonal immunoglobulin deposits-related glomerulopathy (mIgGN). In patients with iNHL-related mIgGN, whether treatment should include either single or a combination of drug(s) to target the malignant clone and renal inflammation remains elusive. In this retr...
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.23798
更新日期:2014-10-01 00:00:00
abstract::Nucleotide sequence analysis of the 5' beta-globin gene flanking region has been carried out for numerous homozygous beta-thalassemia patients with different mutations and of various ethnic backgrounds. Four different rearrangements were found associated with numerous beta-thalassemia mutations. The (AT)X(T)Y repeat m...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450108
更新日期:1994-01-01 00:00:00