Editorials and Perspectives:

Sílvia Beà, Elías Campo

Secondary genomic alterations in non-Hodgkin’s lymphomas: tumor-specific profiles with impact on clinical behavior
Haematologica 2008 93: 641-645. [Full Text] [PDF]  

Lymphoid neoplasms are associated with primary genetic alterations – mainly non-random reciprocal chromosomal translocations – and secondary alterations. In this perspective article, Drs. Bea and Campo discuss the importance of secondary chromosomal alterations in non-Hodgkin’s lymphomas. See related articles on pages 670, 680, and 688.

Mario Cazzola

Molecular basis of thrombocytosis
Haematologica 2008 93: 646-648. [Full Text] [PDF]  

Thrombocytosis can be classified into three major categories: hereditary or familial thrombocytosis, thrombocytosis associated with myeloproliferative and/or myelodysplastic disorders (clonal thrombocytosis), and reactive (secondary) thrombocytosis. In this perspective article, Dr. Cazzola examines the molecular basis of familial thrombocytosis. See related article on page 706.

Massimo Franchini, Pier Mannuccio Mannucci

Interactions between genotype and phenotype in bleeding and thrombosis
Haematologica 2008 93: 649-652. [Abstract] [Full Text] [PDF]  

The clinical phenotypic variability of disorders of blood coagulation is often the result of gene-gene, or gene-environment interactions. Drs. Franchini and Mannucci discuss this only partially understood subject. See related article on page 729.

Original Articles:

Chronic Myeloid Leukemia:

Miriam Puttini, Sara Redaelli, Loris Moretti, Stefania Brussolo, Rosalind H Gunby, Luca Mologni, Edoardo Marchesi, Loredana Cleris, Arianna Donella-Deana, Peter Drueckes, Elisa Sala, Vittorio Lucchini, Michael Kubbutat, Franca Formelli, Alfonso Zambon, Leonardo Scapozza, Carlo Gambacorti-Passerini

Characterization of compound 584, an Abl kinase inhibitor with lasting effects
Haematologica 2008 93: 653-661. Published online March 26, 2008; doi:10.3324/haematol.12212 [Abstract] [Full Text] [PDF] [Puttini et al. - Supplementary Data]  

Secondary resistance to imatinib is observed in patients with chronic myeloid leukemia. This study describes a novel Abl kinase inhibitor with long-lasting effects.

Acute Myeloid Leukemia:

Elisabeth Walsby, Val Walsh, Chris Pepper, Alan Burnett, Ken Mills

Effects of the aurora kinase inhibitors AZD1152-HQPA and ZM447439 on growth arrest and polyploidy in acute myeloid leukemia cell lines and primary blasts
Haematologica 2008 93: 662-669. Published online March 26, 2008; doi:10.3324/haematol.12148 [Abstract] [Full Text] [PDF] [Walsby et al. - Supplementary Data]  

Aurora kinase inhibitors are being considered for treatment of acute myeloid leukemia. Findings of this study suggest that two such inhibitors are effective apoptosis-inducing agents in primary myeloid leukemia cells.

Malignant Lymphomas:

Bibiana I. Ferreira, Juan F. García, Javier Suela, Manuela Mollejo, Francisca I. Camacho, Angel Carro, Santiago Montes, Miguel A. Piris, Juan C. Cigudosa

Comparative genome profiling across subtypes of low-grade B-cell lymphoma identifies type-specific and common aberrations that target genes with a role in B-cell neoplasia
Haematologica 2008 93: 670-679. Published online March 26, 2008; doi:10.3324/haematol.12221 [Abstract] [Full Text] [PDF] [Ferreira et al. - Supplementary data]  

Low-grade B-cell lymphomas are very heterogeneous. This study of comparative genome profiling identifies type-specific aberrations that target genes with a role in B-cell lymphoid neoplasms. See related perspective article on page 641.

Sandrine Sander, Lars Bullinger, Elke Leupolt, Axel Benner, Dirk Kienle, Tiemo Katzenberger, Jörg Kalla, German Ott, Hans Konrad Müller-Hermelink, Thomas F.E. Barth, Peter Möller, Peter Lichter, Hartmut Döhner, Stephan Stilgenbauer

Genomic aberrations in mantle cell lymphoma detected by interphase fluorescence in situ hybridization. Incidence and clinicopathological correlations
Haematologica 2008 93: 680-687. Published online March 26, 2008; doi:10.3324/haematol.12330 [Abstract] [Full Text] [PDF] [Sander et al. - Supplementary Data]  

The primary genetic alteration of mantle cell lymphoma is a t(11;14)(q13;q32). Findings of this study provide novel insights into the pathogenesis of this lymphoid neoplasm, and in particular indicate that loss of chromosome 13q14 has additional prognostic relevance. See related perspective article on page 641.

Judith Dierlamm, Eva M. Murga Penas, Stefan Bentink, Swen Wessendorf, Hilmar Berger, Michael Hummel, Wolfram Klapper, Dido Lenze, Andreas Rosenwald, Eugenia Haralambieva, German Ott, Sergio B. Cogliatti, Peter Möller, Carsten Schwaenen, Harald Stein, Markus Löffler, Rainer Spang, Lorenz Trümper, Reiner Siebert, for the Deutsche Krebshilfe Network Project "Molecular Mechanisms in Malignant Lymphomas"

Gain of chromosome region 18q21 including the MALT1 gene is associated with the activated B-cell-like gene expression subtype and increased BCL2 gene dosage and protein expression in diffuse large B-cell lymphoma
Haematologica 2008 93: 688-696. Published online March 26, 2008; doi:10.3324/haematol.12057 [Abstract] [Full Text] [PDF] [Dierlamm et al. - Supplementary Data]  

Diffuse large B-cell lymphoma is the most common lymphoma worldwide. Recent studies of gene expression profiling have identified subgroups of this lymphoid neoplasm with different prognosis. This study suggests that a gain of chromosome 18q21 including the MALT1 gene may involve an unfavorable prognosis. See related perspective article on page 641.

Lymphoproliferative Disorders:

Francesco Forconi, Elisa Sozzi, Davide Rossi, Surinder S. Sahota, Teresa Amato, Donatella Raspadori, Livio Trentin, Lorenzo Leoncini, Gianluca Gaidano, Francesco Lauria

Selective influences in the expressed immunoglobulin heavy and light chain gene repertoire in hairy cell leukemia
Haematologica 2008 93: 697-705. Published online April 2, 2008; doi:10.3324/haematol.12282 [Abstract] [Full Text] [PDF] [Forconi et al. - Supplementary Data]  

Hairy cell leukemia is a rare, chronic B-cell neoplasm characterized by leukemic hairy cells. This immunogenetic analysis of the expressed immunoglobulin heavy ad light chain gene repertoire suggests that immunoglobulin gene selection may play an important role in the pathognesis of this neoplasm.

Thrombocytosis:

Kun Liu, Robert Kralovics, Zbigniew Rudzki, Barbara Grabowska, Andreas S. Buser, Damla Olcaydu, Heinz Gisslinger, Ralph Tiedt, Patricia Frank, Krzysztof Okoñ, Anthonie P.C. van der Maas, Radek C. Skoda

A de novo splice donor mutation in the thrombopoietin gene causes hereditary thrombocythemia in a Polish family
Haematologica 2008 93: 706-714. Published online March 26, 2008; doi:10.3324/haematol.11801 [Abstract] [Full Text] [PDF] [Liu et al. - Supplementary Data]  

Familial thrombocytosis may be associated with gain-of-function mutations in the thrombopoietin (THPO) gene, or an activating germline mutation of MPL, the gene encoding thrombopoietin receptor. This study of a large family with thrombocytosis associated with a germline THPO mutations illustrates the hematologic and clinical features of this rare condition. See related article on page 646.

Disorders of Hemostasis:

Giorgia Zadra, Rosanna Asselta, Maria Luisa Tenchini, Giancarlo Castaman, Uri Seligsohn, Pier Mannuccio Mannucci, Stefano Duga

Simultaneous genotyping of coagulation factor XI type II and type III mutations by multiplex real-time polymerase chain reaction to determine their prevalence in healthy and factor XI-deficient Italians
Haematologica 2008 93: 715-721. Published online April 2, 2008; doi:10.3324/haematol.12180 [Abstract] [Full Text] [PDF]  

Factor XI deficiency is a rare autosomal recessive coagulopathy worldwide, although relatively common in Ashkenazi Jews. This study describes the presence of the F11 gene mutations in the Italian population and confirms their Jewish origin.

Maurizio Margaglione, Giancarlo Castaman, Massimo Morfini, Angiola Rocino, Elena Santagostino, Giuseppe Tagariello, Anna Rita Tagliaferri, Ezio Zanon, Maria Patrizia Bicocchi, Giuseppe Castaldo, Flora Peyvandi, Rosa Santacroce, Francesca Torricelli, Elvira Grandone, Pier Mannuccio Mannucci, the AICE-Genetics Study Group

The Italian AICE-Genetics hemophilia A database: results and correlation with clinical phenotype
Haematologica 2008 93: 722-728. Published online April 2, 2008; doi:10.3324/haematol.12427 [Abstract] [Full Text] [PDF]  

This study reports a wide spectrum of factor 8 mutations in the large Italian database. Findings of the study indicate hat the type of mutations is a strong predictor of the clinical phenotype.

Thrombosis:

Antonia Miñano, Adriana Ordóñez, Francisco España, José Ramón González-Porras, Ramón Lecumberri, Jordi Fontcuberta, Pilar Llamas, Francisco Marín, Amparo Estellés, Ignacio Alberca, Vicente Vicente, Javier Corral

AB0 blood group and risk of venous or arterial thrombosis in carriers of factor V Leiden or prothrombin G20210A polymorphisms
Haematologica 2008 93: 729-734. Published online April 2, 2008; doi:10.3324/haematol.12271 [Abstract] [Full Text] [PDF]  

Several studies have shown an effect of AB0 blood group on hemostasis. Findings of this study indicate that non-00 group increases the risk and severity of venous thrombosis in carriers of prothrombotic polysmorphisms. See related perspective article on page 649.

Stem Cell Transplantation:

Javier de la Rubia, Felipe de Arriba, Cristina Arbona, María J. Pascual, Concha Zamora, Andrés Insunza, Dorleta Martínez, Carmen Paniagua, Miguel A. Díaz, Miguel A. Sanz

Follow-up of healthy donors receiving granulocyte colony-stimulating factor for peripheral blood progenitor cell mobilization and collection. Results of the Spanish Donor Registry
Haematologica 2008 93: 735-740. Published online April 2, 2008; doi:10.3324/haematol.12285 [Abstract] [Full Text] [PDF]  

Information about the long-term follow-up and safety of granulocyte colony-stimulating factor administration to healthy donors is limited. Findings of this study indicate that clinical side effects are generally mild, and that development of secondary hematologic malignancy is unlikely.

Decision Making and Problem Solving:

Iron Overload:

Emanuele Angelucci, Giovanni Barosi, Clara Camaschella, Maria Domenica Cappellini, Mario Cazzola, Renzo Galanello, Monia Marchetti, Antonio Piga, Sante Tura

Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders
Haematologica 2008 93: 741-752. Published online April 15, 2008; doi:10.3324/haematol.12413 [Abstract] [Full Text] [PDF]  

Three iron chelators are currently available for treatment of transfusion iron overload: deferoxamine, deferiprone, and deferasirox. This article reports the Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders.

Brief Report:

Iron Metabolism:

Sijin Liu, Rajasekhar N.V.S. Suragani, Anping Han, Wanting Zhao, Nancy C. Andrews, Jane-Jane Chen

Deficiency of heme-regulated eIF2 kinase decreases hepcidin expression and splenic iron in HFE^–/– mice
Haematologica 2008 93: 753-756. Published online March 26, 2008; doi:10.3324/haematol.12175 [Abstract] [Full Text] [PDF]  

Heme-regulated eIF2 balances heme and globin production by controlling globin synthesis. Its deficiency affects the phenotype of HFE^–/– mice by further decreasing hepcidin expression.

Sickle Cell Disease:

Eduard J. van Beers, Charlotte F.J. van Tuijn, Melvin R. Mac Gillavry, Anna van der Giessen, John-John B. Schnog, Bart J. Biemond, on behalf of the CURAMA study group

Sickle cell disease-related organ damage occurs irrespective of pain rate: implications for clinical practice
Haematologica 2008 93: 757-760. Published online March 26, 2008; doi:10.3324/haematol.12152 [Abstract] [Full Text] [PDF]  

This study shows that clinically relevant forms of organ damage occur irrespective of the frequency of painful crises in adults with sickle cell disease.

Anemia of Renal Failure:

Bruce S. Spinowitz

Anemia management in patients on peritoneal dialysis: efficacy and safety of epoetin
Haematologica 2008 93: 761-764. Published online March 26, 2008; doi:10.3324/haematol.10985 [Abstract] [Full Text] [PDF]  

In this clinical trial, subcutaneously administered epoetin was effective and well tolerated for treatment of anemia in patients on peritoneal dialysis.

Chronic Myeloid Leukemia:

Paul La Rosée, Susanne Holm-Eriksen, Heiko Konig, Nicolai Härtel, Thomas Ernst, Julia Debatin, Martin C. Mueller, Philipp Erben, Anja Binckebanck, Lydia Wunderle, Yaping Shou, Margaret Dugan, Ruediger Hehlmann, Oliver G. Ottmann, Andreas Hochhaus

Phospho-CRKL monitoring for the assessment of BCR-ABL activity in imatinib-resistant chronic myeloid leukemia or Ph⁺ acute lymphoblastic leukemia patients treated with nilotinib
Haematologica 2008 93: 765-769. Published online March 26, 2008; doi:10.3324/haematol.12186 [Abstract] [Full Text] [PDF]  

Findings of this study suggest that monitoring the actual BCR-ABL inhibition in nilotinib treated patients may be useful for establishing effective dosing and for detecting resistance against the drug.

Francesca Palandri, Ilaria Iacobucci, Fausto Castagnetti, Nicoletta Testoni, Angela Poerio, Marilina Amabile, Massimo Breccia, Tamara Intermesoli, Francesco Iuliano, Giovanna Rege-Cambrin, Mario Tiribelli, Maurizio Miglino, Fabrizio Pane, Giuseppe Saglio, Giovanni Martinelli, Gianantonio Rosti, Michele Baccarani, on behalf of the GIMEMA Working Party on CML

Front-line treatment of Philadelphia positive chronic myeloid leukemia with imatinib and interferon-: 5-year outcome
Haematologica 2008 93: 770-774. Published online March 26, 2008; doi:10.3324/haematol.12265 [Abstract] [Full Text] [PDF]  

This study confirms the excellent response to imatinib front-line therapy in patients with chronic myeloid leukemia. By contrast, most patients discontinued pegylated interferon- due to its side effects.

Acute Myeloid Leukemia:

Brunangelo Falini, Maria Paola Martelli, Cristina Mecucci, Arcangelo Liso, Niccolò Bolli, Barbara Bigerna, Alessandra Pucciarini, Stefano Pileri, Giovanna Meloni, Massimo F. Martelli, Torsten Haferlach, Susanne Schnittger

Cytoplasmic mutated nucleophosmin is stable in primary leukemic cells and in a xenotransplant model of NPMc⁺ acute myeloid leukemia in SCID mice
Haematologica 2008 93: 775-779. Published online March 26, 2008; doi:10.3324/haematol.12225 [Abstract] [Full Text] [PDF]  

Findings of this study indicate that NPM1 mutations are stable in patients with acute myeloid leukemia, providing a rationale for monitoring of minimal residual disease.

Malignant Lymphomas:

Rocio Hassan, Claudete Esteves Klumb, Fabricio E. Felisbino, Deisy M. Guiretti, Lídia R. White, Claudio Gustavo Stefanoff, Mario Henrique M. Barros, Héctor N. Seuánez, Ilana R. Zalcberg

Clinical and demographic characteristics of Epstein-Barr virus-associated childhood Burkitt’s lymphoma in Southeastern Brazil: epidemiological insights from an intermediate risk region
Haematologica 2008 93: 780-783. Published online March 26, 2008; doi:10.3324/haematol.12424 [Abstract] [Full Text] [PDF]  

This report describes clinical and demographic characteristics of Epstein-Barr virus-associated childhood Burkitt’s lymphoma in Southeastern Brazil.

Letters to the Editor:

Thalassemia Syndrome:

Wing-Yan Au, Wynnie Wai-man Lam, Winnie W.C. Chu, Hui-Leung Yuen, Alvin Siu-Cheung Ling, Rever Chak-Ho Li, Helen Man-Hong Chan, Harold Kwok-Kuen Lee, Man-Fai Law, Herman Sung Yu Liu, Raymond Liang, Shau-Yin Ha

A cross-sectional magnetic resonance imaging assessment of organ specific hemosiderosis in 180 thalassemia major patients in Hong Kong
Haematologica 2008 93: 784-786. [Full Text] [PDF]  

Myeloproliferative Disorders:

Zhijian Xiao, Yue Zhang, Lin Li, Ling Nie, Lin Yang, Shicai Xu

The Janus kinase 2 (JAK2) V617F mutation in Chinese patients with chronic myeloproliferative disorders
Haematologica 2008 93: 787-788. [Full Text] [PDF]  

Malignant Lymphomas:

Mark J. Bishton, Rodney J. Hicks, David A. Westerman, Miles H. Prince, Max Wolf, John F. Seymour

A prospective study of the separate predictive capabilities of ¹⁸[F]-FDG-PET and molecular response in patients with relapsed indolent non-Hodgkin’s lymphoma following treatment with iodine-¹³¹-rituximab radio-immunotherapy
Haematologica 2008 93: 789-790. [Full Text] [PDF]  

Multiple Myeloma:

Vania T.M. Hungria, Angelo Maiolino, Gracia Martinez, Gisele W.B. Colleoni, Érika O.D.M. Coelho, Laís Rocha, Renata Nunes, Rosane Bittencourt, Luciana C.O. Oliveira, Rosa Malena O. Faria, Ricardo Pasquini, Sílvia M.M. Magalhães, Cármino A. Souza, Jorge V. Pinto Neto, Luciana Barreto, Elizabeth Andrade, Maria do Socorro O. Portella, Vanessa Bolejack, Brian G.M. Durie, on behalf of the International Myeloma Working Group Latin America

Confirmation of the utility of the International Staging System and identification of a unique pattern of disease in Brazilian patients with multiple myeloma
Haematologica 2008 93: 791-792. [Full Text] [PDF]  

Manasi Vijapurkar, Kanjaksha Ghosh, Shrimati Shetty, Mary Ann McLane, Ana Maria Moura da Silva, Diego Butera

A simple, novel and robust test to diagnose type I Glanzmann thrombasthenia
Haematologica 2008 93: 797-798. [Full Text] [PDF]  

Monoclonal Gammopathies:

Raphael Itzykson, Magali Le Garff-Tavernier, Sandrine Katsahian, Marie-Claude Diemert, Lucile Musset, Veronique Leblond

Serum-free light chain elevation is associated with a shorter time to treatment in Waldenstrom’s macroglobulinemia
Haematologica 2008 93: 793-794. [Full Text] [PDF]  

Platelet Disorders:

Eleonora Toffoletti, Francesco Zaja, Alexsia Chiarvesio, Angela Michelutti, Marta Battista, Renato Fanin

No evidences for B-cell clonality by spectratyping analysis in patients with idiopathic thrombocytopenic purpura undergoing rituximab therapy
Haematologica 2008 93: 795-796. [Full Text] [PDF]  

Hemostasis:

Clément d’Audigier, Eric Pasmant, Odile Bournier, Yves Laurian, Marie Claude Guillin, Annie Bezeaud

A natural variant with a point mutation resulting in a homozygous Arg to His substitution at position 388 in prothrombin
Haematologica 2008 93: 799-800. [Full Text] [PDF]  

Online Only Articles:

C. Bressollette-Bodin, A. Claver, D. Boutolleau, P. Chevallier, T. Guillaume, T. Gastinne, P. Moreau, J-L. Harousseau, B.M. Imbert-Marcille, S. Le Gouill

Surgical treatment of a foscavir-resistant atypical Cytomegalovirus pneumonia in an allogeneic stem cell transplant recipient
Haematologica 2008 93: e39-e41. [Full Text] [PDF]  

E.J. van Beers, H.M.H. Spronk, H. ten Cate, A.J. Duits, D.P.M. Brandjes, J.W.J. van Esser, B.J. Biemond, J.B. Schnog, on behalf of the CURAMA study Group

No association of the hypercoagulable state with sickle cell disease related pulmonary hypertension
Haematologica 2008 93: e42-e44. [Full Text] [PDF]  

V. Gérolami, G. Le Gac, L. Mercier, M. Nezri, J-L. Bergé-Lefranc, C. Férec

Early-onset haemochromatosis caused by a novel combination of TFR2 mutations(p.R396X/c.1538-2 A>G) in a woman of Italian descent
Haematologica 2008 93: e45-e46. [Full Text] [PDF]  

L. Charnas, F. Eichler, A. Kohlschuetter, J. Tolar, P.J. Orchard

Reply to: Reduced intensity conditioning haematopoietic stem cell transplantation with mesenchymal stromal cells infusion for the treatment of metachromatic leukodystrophy: a case report. Haematologica 2008; 93:e11–13
Haematologica 2008 93: e47. [Full Text] [PDF]  

Nathalie Meuleman, Tatiana Tondreau, Geneviève Vanhaelen, Laurence Lagneaux, Dominique Bron

Reduced intensity conditioning haematopoietic stem cell transplantation with mesenchymal stromal cells infusion for the treatment of metachromatic leukodystrophy: a case report
Haematologica 2008 93: e48. [Full Text] [PDF]  

To see an article, click its [Full Text] or [PDF] link. To review many abstracts, check the boxes to the left of the titles you want, and click the 'Get All Checked Abstract(s)' button. To see one abstract at a time, click its [Abstract] link.