Stiff-man syndrome in a patient with Hodgkin's disease. An unusual paraneoplastic syndrome.

abstract：BACKGROUND AND OBJECTIVE:Abnormalities in the expression of cell adhesion molecules (CAM) are thought to influence the patterns of intranodal growth and hematogeneous spread of malignant cells in chronic lymphoproliferative disorders (LPD). Therefore, the characterization of CAM phenotypic profiles of the neoplastic cl...

journal_title：Haematologica

authors： Lúcio PJ,Faria MT,Pinto AM,da Silva MR,Correia Júnior ME,da Costa RJ,Parreira AB

更新日期：1998-02-01 00:00:00

abstract：:e tested whether polymorphic variations in glutathione S-transferase genes (GSTM1, GSTT1, GSTP1) and interleukin-1 (IL-1beta and IL-1RN) genes confer susceptibility to mucosa-associated lymphoid tissue lymphomas (MALT) in a Chinese population. The rates of GSTM1, GSTP1, IL-1beta and IL-1RN genotypes did not differ bet...

journal_title：Haematologica

authors： Wu MS,Shun CT,Huang SP,Cheng AL,Chen LT,Lin JT

更新日期：2004-08-01 00:00:00

abstract：:A case of Di Guglielmo's syndrome passed through the three stages of chronic erythromyelosis, erythroleukemia and acute myeloid leukemia (AML). According to the FAB classification the subsequent stages of this syndrome were refractory anemia (RA), RA with excess of blasts (RAEB), AML-M6, AML-M2 and undifferentiated AM...

journal_title：Haematologica

authors： Michiels JJ,van der Meulen J,Brederoo P

更新日期：1997-07-01 00:00:00

abstract：BACKGROUND:During the final stages of differentiation of mammalian erythroid cells, the chromatin is condensed and enucleated. We previously reported that Rac GTPases and their downstream target, mammalian homolog of Drosophila diaphanous 2 (mDia2), are required for enucleation of in vitro cultured mouse fetal liver er...

journal_title：Haematologica

authors： Ji P,Yeh V,Ramirez T,Murata-Hori M,Lodish HF

更新日期：2010-12-01 00:00:00

abstract：BACKGROUND:Polymorphic differences between donor and recipient human leukocyte antigen class I molecules can result in graft-versus-host disease due to distinct peptide presentation. As part of the peptide-loading complex, tapasin plays an important role in selecting peptides from the pool of potential ligands. Class I...

journal_title：Haematologica

authors： Badrinath S,Saunders P,Huyton T,Aufderbeck S,Hiller O,Blasczyk R,Bade-Doeding C

更新日期：2012-01-01 00:00:00

abstract：BACKGROUND AND OBJECTIVE:Idarubicin (IDA) is relatively immune to the multidrug resistance P-gp mechanism that is frequently expressed in recurrent and refractory hematologic malignancies. Owing to rapid metabolism in vivo, a continuous infusion (CI) of IDA might prolong exposure time to the parent drug rather than its...

journal_title：Haematologica

authors： Bassan R,Chiodini B,Zucchetti M,Lerede T,Cornelli PE,Cortelazzo S,Barbui T

更新日期：1998-01-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:The degree of globin chain imbalance and tissue hypoxia are important determinants of clinical severity in thalassemia syndromes. Thus phenotypic expression may be modified by interaction of alpha- and beta-thalassemia defects, level and type of hemoglobin synthesized and oxygen release to the...

journal_title：Haematologica

authors： Traeger-Synodinos J,Papassotiriou I,Vrettou C,Skarmoutsou C,Stamoulakatou A,Kanavakis E

更新日期：2001-04-01 00:00:00

abstract：:In this open label pilot safety study 80 children over 3 months old with deep venous thrombosis were treated with enoxaparin with a target 4 h anti-factor Xa activity between 0.5-0.8 IU/mL. The children were stratified to receive once daily or twice daily doses. The study end-points were post-thrombotic syndrome, re-t...

journal_title：Haematologica

authors： Schobess R,Düring C,Bidlingmaier C,Heinecke A,Merkel N,Nowak-Göttl U

更新日期：2006-12-01 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

authors： Giordano P,Sabato V,Schettini F,De Mattia D,Iolascon A

更新日期：1997-11-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:High-dose melphalan with autologous peripheral blood stem cell transplantation (ASCT) is an effective treatment for systemic primary amyloidosis. This procedure is, however, associated with substantial toxicity and mortality, particularly if the heart is involved. Refined selection of patients...

journal_title：Haematologica

authors： Perfetti V,Siena S,Palladini G,Bregni M,Di Nicola M,Obici L,Magni M,Brunetti L,Gianni AM,Merlini G

更新日期：2006-12-01 00:00:00

abstract：:Acquired aplastic anemia, the prototypical bone marrow failure disease, is characterized by pancytopenia and marrow hypoplasia. Most aplastic anemia patients respond to immunosuppressive therapy, usually with anti-thymocyte globulin and cyclosporine, but some relapse on cyclosporine withdrawal or require long-term adm...

journal_title：Haematologica

authors： Feng X,Lin Z,Sun W,Hollinger MK,Desierto MJ,Keyvanfar K,Malide D,Muranski P,Chen J,Young NS

更新日期：2017-10-01 00:00:00

abstract：:Elucidating the regulation mechanism of integrin αIIbβ3 is key to understand platelet biology and thrombotic diseases. Previous in vitro studies have implicated a role of migfilin in the support of platelet αIIbβ3 activation, however, contribution of migfilin to thrombosis and hemostasis in vivo and a detailed mechani...

journal_title：Haematologica

authors： Zhou Y,Hu M,Chen X,Wang S,Li J,Sa L,Li L,Huang J,Cheng H,Hu H

更新日期：2020-11-01 00:00:00

abstract：:We report a novel alpha2-globin gene allele with the mutation cod 117 TTC>TCC or alpha 117(GH5)Phe>Ser detected in three carriers with alpha-thalassemia phenotype. The mutated mRNA was present in the reticulocytes in the same amount as the normal one, but no chain or hemoglobin variant were detected. Most likely the a...

journal_title：Haematologica

authors： Lacerra G,Scarano C,Musollino G,Flagiello A,Pucci P,Carestia C

更新日期：2008-01-01 00:00:00

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 ...

journal_title：Haematologica

authors： Li J,Tian Z,Zheng HY,Zhang W,Duan MH,Liu YT,Cao XX,Zhou DB

更新日期：2013-03-01 00:00:00

abstract：BACKGROUND:Phosphatidylserine exposure by red blood cells is acknowledged as a signal that initiates phagocytic removal of the cells from the circulation. Several disorders and conditions are known to induce phosphatidylserine exposure. Removal of phosphatidylserine-exposing red blood cells generally occurs by macropha...

journal_title：Haematologica

authors： Fens MH,van Wijk R,Andringa G,van Rooijen KL,Dijstelbloem HM,Rasmussen JT,de Vooght KM,Schiffelers RM,Gaillard CA,van Solinge WW

更新日期：2012-04-01 00:00:00

abstract：:Clinical and hematologic characteristics of beta(β)-thalassemia are determined by several factors resulting in a wide spectrum of severity. Phenotype modulators are: HBB mutations, HBA defects and fetal hemoglobin production modulators (HBG2:g.-158C>T polymorphism, HBS1L-MYB intergenic region and the BCL11A). We chara...

journal_title：Haematologica

authors： Danjou F,Francavilla M,Anni F,Satta S,Demartis FR,Perseu L,Manca M,Sollaino MC,Manunza L,Mereu E,Marceddu G,Pissard S,Joly P,Thuret I,Origa R,Borg J,Forni GL,Piga A,Lai ME,Badens C,Moi P,Galanello R

更新日期：2015-04-01 00:00:00

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

authors： Escherich G,Tröger A,Göbel U,Graubner U,Pekrun A,Jorch N,Kaspers G,Zimmermann M,zur Stadt U,Kazemier K,Pieters R,Den Boer ML,Horstmann M,Janka GE,CoALL study group, Hamburg, Germany.

更新日期：2011-06-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:Detection of minimal residual disease (MRD) by multiparameter flow cytometry is an emerging prognostic factor in patients with acute myeloid leukemia (AML). The present analysis aimed at improving the applicability of this approach to more patients with AML. DESIGN AND METHODS:Bone marrow sam...

journal_title：Haematologica

authors： Kern W,Danhauser-Riedl S,Ratei R,Schnittger S,Schoch C,Kolb HJ,Ludwig WD,Hiddemann W,Haferlach T

更新日期：2003-06-01 00:00:00

abstract：BACKGROUND AND OBJECTIVE:A D-dimer assay can be helpful to rule out thromboembolism provided it is sensitive, reliable, fast and easy to perform. Tests based on the ELISA methodology have a high diagnostic sensitivity, and are therefore adequate for excluding deep venous thrombosis (DVT). The drawbacks are their long a...

journal_title：Haematologica

authors： Villa P,Ferrando F,Serra J,Faus H,Mira Y,Vayá A,Aznar J

更新日期：2000-05-01 00:00:00

abstract：:About 25-30% of patients with immune thrombocytopenic purpura (ITP) are refractory to corticosteroids, splenectomy and other treatments. It has been suggested that interferon-alpha 2b (IFN-alpha 2b) may be useful in the treatment of chronic refractory ITP patients. We treated 9 chronic refractory ITP patients with IFN...

journal_title：Haematologica

authors： Vianelli N,Tazzari PL,Baravelli S,Ricci F,Valdrè L,Tura S

更新日期：1998-08-01 00:00:00

abstract：:Seventy-four patients over 60 years of age with new cases of ANLL diagnosed between January, 1980 and December, 1986 were retrospectively evaluated. Twenty-nine (median age 63, range 60-70) received aggressive induction polychemotherapy: 15 achieved CR (52%), 10 were resistant (34.5%) and 4 died during induction (13.5...

journal_title：Haematologica

authors： Latagliata R,Sgadari C,Pisani F,Falconi M,Spadea A,Vegna ML,Petti MC

更新日期：1989-03-01 00:00:00

abstract：:Iron overload and hepatitis virus C infection cause liver fibrosis in thalassemics. In a monocentric retrospective analysis of liver disease in a cohort of 191 transfusion-dependent thalassemics, in 126 patients who had undergone liver biopsy (mean age 17.2 years; 58 hepatitis virus C-RNA positive and 68 hepatitis vir...

journal_title：Haematologica

authors： Di Marco V,Capra M,Gagliardotto F,Borsellino Z,Cabibi D,Barbaria F,Ferraro D,Cuccia L,Ruffo GB,Bronte F,Di Stefano R,Almasio PL,Craxì A

更新日期：2008-08-01 00:00:00

abstract：:Post-transfusion hepatitis (PTH) is a major problem in patients with acute leukemias requiring blood products during induction or consolidation therapy. In fact, PTH causes delays of chemotherapy with major violations in the timing of protocols. In order to assess the efficacy and safety of a short course of alpha-int...

journal_title：Haematologica

authors： Almasio P,Mirto S,Malleo C,Di Marco V,Caronia F,Craxi A

更新日期：1991-09-01 00:00:00

abstract：:Prostacyclin (PGI2) controls platelet activation and thrombosis through a cyclic adenosine monophosphate (cAMP) signaling cascade. However, in patients with cardiovascular diseases this protective mechanism fails for reasons that are unclear. Using both pharmacological and genetic approaches we describe a mechanism by...

journal_title：Haematologica

authors： Berger M,Raslan Z,Aburima A,Magwenzi S,Wraith KS,Spurgeon BEJ,Hindle MS,Law R,Febbraio M,Naseem KM

更新日期：2020-03-01 00:00:00

abstract：:Juvenile hemochromatosis is a severe form of hereditary iron overload. It can be caused by mutations in either hepcidin or hemojuvelin genes. In this study we identified the molecular basis of juvenile hemochromatosis in three Australian families and assessed the role of potential modifying genes in individuals carryi...

journal_title：Haematologica

authors： Wallace DF,Dixon JL,Ramm GA,Anderson GJ,Powell LW,Subramaniam N

更新日期：2005-02-01 00:00:00

abstract：BACKGROUND:There is variability in the outcome of patients with chronic lymphocytic leukemia with apparently the same stage of disease. Identifying genetic variants that influence patients' outcome and response to treatment may provide important insights into the biology of the disease. DESIGN AND METHODS:We investiga...

journal_title：Haematologica

authors： Wade R,Di Bernardo MC,Richards S,Rossi D,Crowther-Swanepoel D,Gaidano G,Oscier DG,Catovsky D,Houlston RS

更新日期：2011-10-01 00:00:00

abstract：:We report on a case of acute myeloid leukemia in a 17-year old boy affected by Shwachman Diamond syndrome (SDS). Conventional cytogenetics at diagnosis revealed an abnormal clone with complex karyotypic changes including typical myeloid aberrations, such as monosomy 5, tetrasomy of chromosome 8, trisomy 9, and deletio...

journal_title：Haematologica

authors： Spirito FR,Crescenzi B,Matteucci C,Martelli MF,Mecucci C

更新日期：2000-11-01 00:00:00

abstract：:We report the distribution and clinicopathological characteristics of malignant lymphomas in Taiwan, defined according to the WHO classification. Data including age and gender of the patients, clinical staging and disease courses were collected for 598 cases of malignant lymphomas. The results showed that the epidemio...

journal_title：Haematologica

authors： Lee MY,Tan TD,Feng AC,Liu MC

更新日期：2005-12-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:Myelodysplastic syndromes (MDS) comprise a group of heterogeneous hematologic disorders with risk of leukemic evolution (LE). The French-American-British (FAB) co-operative group classifies them into five morphologic entities and the International Prognostic Scoring System (IPSS) proposes four...

journal_title：Haematologica

authors： Belli C,Acevedo S,Bengio R,Arrossagaray G,Watman N,Rossi N,García J,Flores G,Goldztein S,Larripa I

更新日期：2002-01-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:To assess the efficacy and the toxic profile of gemcitabine, a novel pyrimidine antimetabolite active against several solid tumors, we carried out a study in heavily pretreated Hodgkin's disease (HD) patients. DESIGN AND METHODS:From May 1997 to January 1999, 14 pretreated patients (10 relaps...

journal_title：Haematologica

authors： Zinzani PL,Bendandi M,Stefoni V,Albertini P,Gherlinzoni F,Tani M,Piccaluga PP,Tura S

更新日期：2000-09-01 00:00:00