Abstract:
BACKGROUND:Hereditary thrombotic thrombocytopenic purpura, also called Upshaw-Schulman syndrome (USS), is a rare disease caused by genetic mutations in the ADAMTS13 gene, which severely decrease the activity of ADAMTS13, a metalloprotease that cleaves von Willebrand factor multimers (VWF). Genotypically identical patients can show great phenotypic diversity. OBJECTIVES:Comparison of selected laboratory parameters and ADAMTS13 pharmacokinetics among patients with USS was performed. PATIENTS/METHODS:Six patients with USS on prophylactic plasma therapy have been reviewed, retrospectively. Blood counts, lactate dehydrogenase (LDH), and ADAMTS13 activity at various time-points before and after different treatment cycles were evaluated. RESULTS:ADAMTS13 recovery and pharmacokinetics were affected by treatment modality, and also reflected the patients' comorbidities and their current physiological and clinical condition. CONCLUSIONS:Our present findings support a multifactorial contribution to treatment efficacy, and confirm the importance of adaptability and individualization of USS therapy. Therapeutic plasma exchange even in hereditary TTP is an option that can in some patients prolong intervals between plasma administration.
journal_name
J Clin Apherjournal_title
Journal of clinical apheresisauthors
Kovarova P,Hrdlickova R,Blahutova S,Cermakova Zdoi
10.1002/jca.21664subject
Has Abstractpub_date
2019-02-01 00:00:00pages
13-20issue
1eissn
0733-2459issn
1098-1101journal_volume
34pub_type
杂志文章abstract::Lipid-apheresis (LA) is thought to improve microcirculation. However, limited data are available on the effects on peripheral microcirculation. We investigated upper limb microcirculation of 22 patients undergoing regular LA on a weekly basis before and after LA. Using standardized semiquantitative scales, we analyzed...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.20285
更新日期:2011-01-01 00:00:00
abstract:OBJECTIVE:Effects of therapeutic plasma exchange (TPE) on immune cells and their cytokine production in MS, are unknown. Since interleukine-6 and tumor growth factor-β have critical roles in MS immunopathogenesis, the impacts of TPE on the expression of these cytokines and their receptors on the surface of CD4+ T lymph...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21755
更新日期:2020-04-01 00:00:00
abstract::Thrombopoietin (TPO) serum levels in 14 patients (9 male and 5 female, mean age 36 years, range 16 to 55 years) with breast cancer (n = 5), testicular cancer (n = 7), or lymphoma (n = 2), undergoing high dose chemotherapy with peripheral blood stem cell (PBSC) transplantation, were evaluated at the first day of the mo...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/(sici)1098-1101(1999)14:2<57::aid-jca2>3.0
更新日期:1999-01-01 00:00:00
abstract::The process of publishing original work in a peer review journal is not complete at the point where the manuscript is first submitted. The journal editors will submit the manuscript to peer review whereby outside experts are asked to vet the manuscript for scientific merit, originality and quality. Reviewers' comments...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章,评审
doi:10.1002/jca.21822
更新日期:2020-08-07 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Specialized centers claim a need for blood component production independent from the general blood transfusion services. We performed a prospective change control analysis of the transfer of platelet (PLT) production for hematological patients at the University Hospital Basel from the Departme...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21214
更新日期:2012-01-01 00:00:00
abstract:INTRODUCTION:Algorithms have been developed to predict the platelet yield after apheresis from the donor's data, as well as the effect on the blood cell count, to extract an acceptable platelet number without affecting the donor. However, the evaluation of these algorithms has not been widely reported. This study aimed...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21846
更新日期:2020-10-05 00:00:00
abstract::Folinic acid (FA) has been reported to expand the pool of peripheral blood stem cells (PBSC) after chemotherapy. We evaluated the efficacy of FA for harvesting PBSC following cytotoxic chemotherapy in 4 patients with acute leukemia. After achieving a complete remission (CR), 3 courses of chemotherapy for a consolidati...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.2920070410
更新日期:1992-01-01 00:00:00
abstract:INTRODUCTION:Limited data are available describing indications for and outcomes of therapeutic plasma exchange (TPE) in cardiac transplantation. METHODS:In a retrospective study of patients who underwent cardiac transplantation at Duke University Medical Center from 2010 to 2014, we reviewed 3 TPE treatment patterns: ...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21622
更新日期:2018-08-01 00:00:00
abstract::The cell separator MCS-3P is an apheresis system offering the flexibility to collect standardized red blood cells, plasma, and/or platelets from one donor. Two different programs were used for the red cell apheresis--RBCP (collection of one unit of red cells and two units of plasma) and RBCPS (one unit of red cells an...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.2920090308
更新日期:1994-01-01 00:00:00
abstract:BACKGROUND:Nephrogenic systemic fibrosis (NSF), also known as nephrogenic sclerosing dermopathy (NSD), is a rare progressive fibrosing disease associated with gadolinium based dyes in patients with renal disease. The exact pathophysiology is not well understood. Accepted treatments include corticosteroids, immune modul...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21253
更新日期:2013-08-01 00:00:00
abstract::Photopheresis has become a key component in the therapeutic armamentarium of cutaneous T-cell lymphoma, graft-versus-host disease following stem cell transplant, and allograft rejection of solid organs such as heart. Although it is considered a new treatment modality in its present form, the field of phototherapy date...
journal_title:Journal of clinical apheresis
pub_type: 历史文章,杂志文章,评审
doi:10.1002/jca.20089
更新日期:2006-04-01 00:00:00
abstract::For many years dextran sulfate adsorption (DSA) treatment of separated plasma has been an established technology for low-density lipoprotein (LDL)-elimination. Recently a system for the treatment of whole blood based on DSA was introduced into clinical practice. To further characterize DSA treatment of whole blood, th...
journal_title:Journal of clinical apheresis
pub_type: 临床试验,杂志文章
doi:10.1002/jca.20135
更新日期:2007-01-01 00:00:00
abstract::Mobilization regimens for CD34+ cells have generally been judged successful based on the number of cells collected without evaluating mobilization separately from collection. Using retrospective data for patients who collected CD34+ cells on Total Therapy protocols 3a/3b (VTD-PACE) and Total Therapy 4/5 using a novel ...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21313
更新日期:2014-10-01 00:00:00
abstract:BACKGROUND:Securing adequate vascular access is essential for a successful apheresis procedure. In most, peripheral access is preferred but it is not always technically possible. Ultrasound-Guided Peripheral Vascular Access (USG-PIVA) is a well-documented technique in the setting of Emergency departments. However, limi...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21440
更新日期:2016-12-01 00:00:00
abstract::Apheresis procedures in small children are technically challenging and require special planning with attention to extracorporeal volume. Discontinuous procedures such as extracorporeal photopheresis (ECP) require additional consideration. Alternative methods to perform ECP have been utilized in small children that req...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.20231
更新日期:2010-01-01 00:00:00
abstract:BACKGROUND:Dysphagia screening is oftentimes a focus of hospitalized patients, but dysphagia can also occur in outpatient settings. Dysphagia can be overlooked by nurses and clinicians, and it is therefore important to educate nurses on the importance of dysphagia screenings. METHODS:This was a randomized prospective ...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章,随机对照试验
doi:10.1002/jca.21572
更新日期:2018-02-01 00:00:00
abstract::Treatment by low density lipoprotein (LDL) apheresis using dextran sulfate columns (DSC) leads to hemostasis alterations with prolonged activated partial thromboplastin time (APTT) of more than 120 seconds. In order to explain this hypocoagulability, we studied hemostasis parameters both in patients and in the extraco...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.2920080302
更新日期:1993-01-01 00:00:00
abstract::This study was undertaken to evaluate the relationship between the time to recovery of peripheral blood counts and CD34+ cells in the peripheral blood (PB) and apheresis collections of patients undergoing intensive chemotherapy followed by rhG-CSF. Twenty-three patients with a median age of 42 years (range 17-64) with...
journal_title:Journal of clinical apheresis
pub_type: 临床试验,杂志文章
doi:10.1002/(sici)1098-1101(1998)13:1<1::aid-jca1>3.0.
更新日期:1998-01-01 00:00:00
abstract::The removal of specific antibody in experimental animals has been reported to result in a subsequent increase in antibody to levels equal to (rebound) or exceeding those existing prior to removal (overshoot). Anecdotal reports suggest that rebound antibody synthesis after plasmapheresis may occur in humans with autoim...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.2920050304
更新日期:1990-01-01 00:00:00
abstract::This is an attempt at comparing two cell separators for plateletpheresis, namely the Fresenius AS.TEC 204 and Haemonetics MCS 3p, at a tertiary care center in India. Donors who weighed between 55-75 kg, who had a hematocrit of 41-43%, and platelet counts of 250x10(3)-400x10(3)/microl were selected for the study. The c...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.20108
更新日期:2007-02-01 00:00:00
abstract::Mobilized stem cells in the peripheral blood (PB) must be efficiently harvested at the appropriate time before autologous PB stem cell (PBSC) transplantation. Enumeration of CD34+ cells in the PB before apheresis predicts the number of PBSCs that can be collected, but the cytometric techniques used are complex and exp...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.20275
更新日期:2011-01-01 00:00:00
abstract:BACKGROUND:Patients undergoing therapeutic plasma exchange (TPE) may present with risks for hemorrhage or thrombosis. Use of replacement fluids devoid of coagulation factors will decrease factor levels and platelet levels. There are no established guidelines for hemostasis management in these situations. MATERIALS AND...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21653
更新日期:2018-10-01 00:00:00
abstract::Lipoprotein apheresis is applied to deplete low density lipoprotein and other apolipoprotein B containing lipoproteins in patients with severe familial hypercholesterolemia, hypertriglyceridemia associated pancreatitis, or lipoprotein (a)-hyperlipoproteinemia. Anticoagulation of the extracorporeal circuit may influenc...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21519
更新日期:2017-12-01 00:00:00
abstract::Either bone marrow or peripheral blood may be harvested to provide hematopoietic stem cells (HSC) for autologous transplantation. Both, however, comprise heterogeneous cell populations. The HSC necessary for successful engraftment constitute a very small fraction of the cells harvested. After collection, the harvested...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章,评审
doi:10.1002/jca.2920070307
更新日期:1992-01-01 00:00:00
abstract::A "snapshot" survey of physician coverage for evaluation and supervision of therapeutic apheresis procedures shows significant variation in current clinical practice between 39 blood center-and 41 non-blood center-based programs. Whereas 56% of blood center-based programs usually require physician's "in person" evalua...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.2920100405
更新日期:1995-01-01 00:00:00
abstract:INTRODUCTION:Nanoscale particle-based drug delivery systems like long circulating liposomal doxorubicin show unique pharmacokinetic properties and improved toxicity profiles. Liposomal doxorubicin accumulates in tumor tissue due to the enhanced permeation and retention effect, but only a small fraction of a total dose ...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.20229
更新日期:2010-01-01 00:00:00
abstract::The blood flow property is one of the factors determining blood perfusion and oxygen supply. The viscosity of the blood is primarily related to the hematocrit, but also to the amount of fibrinogen and other macromolecules present in the blood. Patients with ischemic heart disease have shown a rapid and safe improvemen...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/(sici)1098-1101(1997)12:4<183::aid-jca5>3.
更新日期:1997-01-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Estimation of the total blood volume (TBV) is fundamental to the control of automated red cell exchange (RCE). Error in the TBV estimate can produce outcomes that deviate from the prescribed targets, and may endanger the patient. Both the Spectra Optia and the COBE Spectra use the Nadler formu...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21748
更新日期:2019-12-01 00:00:00
abstract:INTRODUCTION:ABO- or HLA-incompatible kidney transplantation is possible thanks to pretransplant antibody-depletion achieved by extracorporeal-treatment modalities. These methods induce depletion of some plasma proteins and may also impact on proteins involved in hemostasis. METHODS:To determine the impact of one sess...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章
doi:10.1002/jca.21825
更新日期:2020-08-17 00:00:00
abstract::Sickle cell disease (SCD) patients are prone to develop complications that include stroke, acute chest syndrome, and other crises. Some of these complications require chronic transfusion therapy or red cell exchange (RCE), either for therapeutic or prophylactic reasons. Due to a discrepancy of red cell antigens betwee...
journal_title:Journal of clinical apheresis
pub_type: 杂志文章,评审
doi:10.1002/jca.20112
更新日期:2006-12-01 00:00:00