Abstract:
:POEMS syndrome is a rare clonal plasma cell disease. Patients with POEMS syndrome are at risk of developing pulmonary hypertension, but the data on its incidence and impact on outcome are limited. We reviewed records of 154 POEMS syndrome patients with complete duplex echocardiography data for estimation of pulmonary artery systolic pressure (sPAP) at the time of diagnosis. Forty-two (27%) of 154 patients with pulmonary hypertension (estimated sPAP ≥50 mmHg) were identified. Median age was 46 years (range 31-71 years). Patients with pulmonary hypertension were more likely to have peripheral edema (P=0.04), ascites (P=0.02), pleural effusion (P=0.005), and have longer time from onset to diagnosis (P=0.004) when compared with those without pulmonary hypertension. Restrictive abnormalities and decreased diffusion capacity of carbon monoxide were observed in 83% and 96% patients with pulmonary hypertension, compared with 50% and 72% in patients without pulmonary hypertension, respectively. Reversibility of pulmonary hypertension was observed after treatment of POEMS syndrome. After median follow of 32 months, survival of patients with pulmonary hypertension was worse than those without (median overall survival 54 months vs. median not reached, P=0.021). In conclusion, pulmonary hypertension is a common feature of POEMS syndrome, and is associated with signs of extravascular volume overload. Although active treatment of POEMS syndrome can reverse pulmonary hypertension, survival of these patients is worse than those without pulmonary hypertension.
journal_name
Haematologicajournal_title
Haematologicaauthors
Li J,Tian Z,Zheng HY,Zhang W,Duan MH,Liu YT,Cao XX,Zhou DBdoi
10.3324/haematol.2012.073031subject
Has Abstractpub_date
2013-03-01 00:00:00pages
393-8issue
3eissn
0390-6078issn
1592-8721pii
haematol.2012.073031journal_volume
98pub_type
杂志文章相关文献
HAEMATOLOGICA文献大全abstract:BACKGROUND:In a study of childhood acute lymphoblastic leukemia (CoALL 06-97 study), the in vitro sensitivity of the patients' cells to prednisolone, vincristine and asparaginase was introduced as a new additional risk parameter for treatment stratification. In parallel in vivo treatment response was assessed by determ...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2010.039735
更新日期:2011-06-01 00:00:00
abstract::Hepcidin levels are high and iron absorption is limited in acute malaria. The mechanism(s) that regulate hepcidin secretion remain undefined. We have measured hepcidin concentration and cytokines in 100 Kenyan children with acute falciparum malaria and different degrees of anemia. Hepcidin was increased on admission a...
journal_title:Haematologica
pub_type: 临床试验,杂志文章
doi:10.3324/haematol.2012.065854
更新日期:2012-11-01 00:00:00
abstract:BACKGROUND:Preclinical studies have highlighted the activity of lenalidomide in mantle cell lymphoma and its anti-proliferative synergy with dexamethasone. DESIGN AND METHODS:In this prospective, multicenter, phase II study, patients with relapsed/refractory mantle cell lymphoma who were not eligible for, or had relap...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究
doi:10.3324/haematol.2011.051813
更新日期:2012-03-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Peripheral blood stem cells (PBSC) following reduced intensity conditioning (RIC) are being increasingly used for allogeneic transplantation in multiple myeloma. The purpose of this study was to compare outcome of patients transplanted with either PBSC or bone marrow (BM) following RIC or myel...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.11353
更新日期:2007-11-01 00:00:00
abstract::Thromboembolism is a serious complication of induction therapy for childhood acute lymphoblastic leukemia. We prospectively compared the efficacy and safety of antithrombotic interventions in the consecutive leukemia trials ALL-BFM 2000 and AIEOP-BFM ALL 2009. Patients with newly diagnosed acute lymphoblastic leukemia...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.3324/haematol.2018.194175
更新日期:2019-04-01 00:00:00
abstract::Tissue Factor is a cell-surface glycoprotein expressed in various cells of the vasculature and is the principal regulator of the blood coagulation cascade and hemostasis. Notably, aberrant expression of Tissue Factor is associated with cardiovascular pathologies such as atherosclerosis and thrombosis. Here, we sought ...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2017.183087
更新日期:2018-06-01 00:00:00
abstract::Previously, we found that LDL-receptor related protein-1 on macrophages mediated shear stress-dependent clearance of von Willebrand factor. In control experiments, however, we observed that von Willebrand factor also binds to macrophages independently of this receptor under static conditions, suggesting the existence ...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2017.175216
更新日期:2018-04-01 00:00:00
abstract::Peripheral blood lymphoid cell expansions with an unusual CD3+, CD4+, CD8dim+/-, CD11b+, CD57+ immunophenotype have recently been reported. They frequently have the morphology of large granular lymphocytes (LGL) and can be either monoclonal or polyclonal. Their significance is still unclear and no association with hem...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1995-03-01 00:00:00
abstract::Fibrinogen is an abundant protein synthesized in the liver, present in human blood plasma at concentrations ranging from 1.5-4 g/L in healthy individuals with a normal half-life of 3-5 days. With fibrin, produced by thrombin-mediated cleavage, fibrinogen plays important roles in many physiological processes. Indeed, t...
journal_title:Haematologica
pub_type: 杂志文章,评审
doi:10.3324/haematol.2019.236901
更新日期:2020-01-31 00:00:00
abstract:BACKGROUND:Pediatric acute myeloid leukemia is a heterogeneous disease characterized by non-random genetic aberrations related to outcome. The genetic subtype is currently detected by different diagnostic procedures which differ in success rate and/or specificity. DESIGN AND METHODS:We examined the potential of gene e...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2010.029660
更新日期:2011-02-01 00:00:00
abstract::We sought to confirm the prognostic importance of simple clinically available biomarkers of C-reactive protein, serum albumin, and ferritin prior to allogeneic hematopoietic cell transplantation. The study population consisted of 784 adults with acute myeloid leukemia in remission or myelodysplastic syndromes undergoi...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究
doi:10.3324/haematol.2016.145847
更新日期:2016-11-01 00:00:00
abstract::This study investigated the kinetics of CD3+-T cell chimerism (TCC) in 102 patients receiving reduced intensity conditioning allogeneic stem cell transplantation (RIC-allo-SCT) from an HLA-identical sibling. Patients with full donor TCC at day 30 had a higher incidence of grade 2-4 acute GVHD compared to patients in m...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.10971
更新日期:2007-07-01 00:00:00
abstract::Multicytokine therapy may be useful to counteract radiation-induced myelosuppression. We assessed the stem cell factor + glycosylated erythropoietin + pegylated granulocyte colony-stimulating factor combination (SEG) as an emergency treatment. SEG in highly irradiated monkeys efficacy appeared to be restricted to gran...
journal_title:Haematologica
pub_type: 信件
doi:10.3324/haematol.12183
更新日期:2008-03-01 00:00:00
abstract:BACKGROUND:Increased levels of microparticles exposing tissue factor circulate in the blood of patients with coronary heart disease, possibly disseminating their pro-thrombotic and pro-inflammatory potential. Because diets rich in n-3 (polyunsaturated) fatty acids have been associated with reduced incidence of coronary...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.11845
更新日期:2008-06-01 00:00:00
abstract:BACKGROUND:Chimeric oncogenes encoding constitutively active protein tyrosine kinases are associated with chronic myeloid neoplasms. TEL-PDGFRbeta (TPbeta, also called ETV6-PDGFRB) is a hybrid protein produced by the t(5;12) translocation, FIP1L1-PDGFRalpha (FPalpha) results from a deletion on chromosome 4q12 and ZNF19...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2008.001149
更新日期:2009-08-01 00:00:00
abstract:BACKGROUND:Gastrointestinal bleeding is a frequent complication of liver cirrhosis (LC) and represents an important warning sign of imminent death. Platelet dysfunction is an abnormality occurring prevalently in severe liver failure, and could well predispose to bleeding. METHODS:One hundred and two patients with live...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1994-01-01 00:00:00
abstract:BACKGROUND:Usefulness of iron chelation therapy in myelodysplastic patients is still under debate but many authors suggest its possible role in improving survival of low-risk myelodysplastic patients. Several reports have described an unexpected effect of iron chelators, such as an improvement in hemoglobin levels, in ...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2009.016824
更新日期:2010-08-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHL) showed recurrent rearrangement of the BCL6 which is gene detected in 48% of cases analyzed by interphase-fluorescent in situ hybridization (FISH). These findings point to a critical role for BCL6 in the development of this distinct Hodg...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2004-08-01 00:00:00
abstract:BACKGROUND:Most minimal residual disease-directed treatment interventions in current treatment protocols for acute lymphoblastic leukemia are based on bone marrow testing, which is a consequence of previous studies showing the superiority of bone marrow over peripheral blood as an investigational material. Those studie...
journal_title:Haematologica
pub_type: 临床试验,杂志文章,多中心研究
doi:10.3324/haematol.2011.042937
更新日期:2011-12-01 00:00:00
abstract::We conducted a phase I/II multicenter trial using 6 cycles of brentuximab vedotin (BV) in combination with rituximab, cyclophosphamide, doxorubicin, and prednisone (R-CHP) for treatment of patients with CD30-positive (+) B-cell lymphomas. Thirty-one patients were evaluable for toxicity and 29 for efficacy including 22...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2019.238675
更新日期:2020-05-15 00:00:00
abstract:BACKGROUND AND OBJECTIVE:This study was designed to evaluate the efficacy and toxicity of monthly alternating ABVD/MOPP compared to ABVD/OPP regimens in patients with advanced stage Hodgkin's disease (HD), as well as in early stage patients with systemic symptoms and/or bulky disease. DESIGN AND METHODS:218 patients w...
journal_title:Haematologica
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1998-07-01 00:00:00
abstract::Over the past 20 years we have studied 1781 patients with beta-thalassemia syndromes of which 1481 Yugoslav, 166 Bulgarian, 102 Turkish and 32 Albanian. In this paper we summarize the data on the heterogeneity and molecular basis of beta-thal, delta beta-thal and Lepore hemoglobinopathy in these four nationalities liv...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1990-09-01 00:00:00
abstract::This review deals with the chronic lymphoproliferative disease of large granular lymphocytes endowed with T and natural killer cell characteristics. The disease is sufficiently characterized to allow its distinction from other lymphoproliferative disorders of the T cell type. The heterogeneous clinical course and labo...
journal_title:Haematologica
pub_type: 杂志文章,评审
doi:
更新日期:1989-01-01 00:00:00
abstract::Eleven patients with advanced APL were treated with ATO (0.15 mg/Kg daily). Eight (73%) achieved molecular CR, but 5 relapsed, 1 died in molecular CR, 1 was lost to follow-up and 1 is still alive in CR after allogeneic transplantation. We suggest that ATO may be effective also in advanced APL, but given the short CR, ...
journal_title:Haematologica
pub_type: 信件
doi:
更新日期:2004-05-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human metabolic disorder in southern China. We investigated the incidence and distribution of mutations, the molecular pathology of affected females and the haplotype association with G6PD deficiency in patients from the Gu...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2006-10-01 00:00:00
abstract::BCL11A was the focus of recent studies on its inhibiting effect when bound onto the β-globin cluster in the mechanism of hemoglobin switching and HbF downregulation. We examined a cohort of 10 patients displaying different HbF levels and short deletions within the γβ-δ intergenic region to find a possible correlation ...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2012.061994
更新日期:2013-02-01 00:00:00
abstract::It is well known that thalassemic patients exhibit an increased frequency of thrombotic events. Most individuals with resistance to activated protein C (APCR) are the result of a point mutation replacing Arg 506 with Gln in the factor V aminoacidic sequence (factor V Leiden). Recently APCR has been shown to account fo...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1997-11-01 00:00:00
abstract::Treatment of adults with acute myeloblastic leukemia has changed substantially over the past two decades. Currently available estimates of survival do not reflect results from present state-of-the-art treatment due to a lag between the availability of new treatments and data concerning their effect on survival on the ...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.12304
更新日期:2008-04-01 00:00:00
abstract:BACKGROUND:Imatinib can induce severe hepatotoxicity, in 1-5% of CML patients, many of whom need permanent imatinib discontinuation. DESIGN AND RESULTS:We report 5 CML patients who developed grade 3-4 hepatotoxicity after 2-8 months in imatinib. Different aetiologies of liver damage were ruled out and toxicity recurre...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2006-06-01 00:00:00
abstract::Transient pancytopenia preceding childhood acute lymphoblastic leukemia (ALL) is an unfrequent but well-known event. The association of this preleukemic syndrome with hepatitis is extremely rare, with only two such cases having been published in the literature. We report the case of a 16-year-old boy who was diagnosed...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1998-06-01 00:00:00