Editorials and Perspectives:

Irma Dianzani, Fabrizio Loreni

Diamond-Blackfan anemia: a ribosomal puzzle
Haematologica 2008 93: 1601-1604. [Full Text] [PDF]  

Diamond-Blackfan anemia is the first, and so far only known, inherited disease due to a defect in a structural ribosomal protein. In this perspective article, Drs. Dianzani and Loreni examine this ribosomal puzzle. See related articles on pages 1617 and 1627.

Nina Worel, Peter Kalhs

AB0-incompatible allogeneic hematopoietic stem cell transplantation
Haematologica 2008 93: 1605-1607. [Full Text] [PDF]  

Due to the fact that the HLA system is inherited independently of the blood group system, approximately 40 to 50% of all hematopoietic stem cell transplants are performed across the AB0-blood group barrier. In this perspective article, Drs. Worel and Kahls examine the features and outcome of AB0-incompatible allogeneic hematopoietic stem cell transplantation. See related paper on page 1686.

Fulvio Porta, Franco Locatelli, Giuseppe Roberto Burgio

Hematopoietic stem cell transplantation: 40 years of continuous progress and evolution
Haematologica 2008 93: 1607-1610. [Full Text] [PDF]  

Hematopoietic stem cell transplantation has represented one of the most innovative treatments of the last decades, as well as one of the most significant medical feats of human bio-solidarity. In this perspective article, Drs. Porta, Locatelli and Burgio analyze 40 years of continuous progress and evolution in the field.

Livio Pagano, Luana Fianchi, Morena Caira

Pulmonary aspergillosis in hematologic malignancies: lights and shadows
Haematologica 2008 93: 1611-1616. [Full Text] [PDF]  

The population of patients with hematological disorders at risk for pulmonary aspergillosis is expanding. In this perspective article, Drs. Pagano, Fianchi, and Caira analyze the recent advances in this field.

Original Articles:

Michelle A. Rey, Simon P. Duffy, Jennifer K. Brown, James A. Kennedy, John E. Dick, Yigal Dror, Chetankumar S. Tailor

Enhanced alternative splicing of the FLVCR1 gene in Diamond Blackfan anemia disrupts FLVCR1 expression and function that are critical for erythropoiesis
Haematologica 2008 93: 1617-1626. Published online September 24, 2008; doi:10.3324/haematol.13359 [Abstract] [Full Text] [PDF]  

This study recapitulates the Diamond-Blackfan anemia hematologic features of reduced erythropoiesis but normal myelopoiesis by disrupting FLVCR1 in human hematopoietic stem cells. See related perspective article on page 1601.

Aurore Crétien, Corinne Hurtaud, Hélène Moniz, Alexis Proust, Isabelle Marie, Orianne Wagner-Ballon, Valérie Choesmel, Pierre-Emmanuel Gleizes, Thierry Leblanc, Jean Delaunay, Gil Tchernia, Narla Mohandas, Lydie Da Costa

Study of the effects of proteasome inhibitors on ribosomal protein S19 (RPS19) mutants, identified in patients with Diamond-Blackfan anemia
Haematologica 2008 93: 1627-1634. Published online September 2, 2008; doi:10.3324/haematol.13023 [Abstract] [Full Text] [PDF]  

The findings of this study demonstrate an important role for the proteasomal degradation pathway in regulating the levels of expression and nucleolar localization of certain mutant RPS19 proteins in Diamond-Blackfan anemia. See related perspective article on page 1601.

Nasios Fourouclas, Juan Li, Daniel C. Gilby, Peter J. Campbell, Philip A. Beer, Elaine M. Boyd, Anne C. Goodeve, David Bareford, Claire N. Harrison, John T. Reilly, Anthony R. Green, Anthony J. Bench

Methylation of the suppressor of cytokine signaling 3 gene (SOCS3) in myeloproliferative disorders
Haematologica 2008 93: 1635-1644. Published online September 24, 2008; doi:10.3324/haematol.13043 [Abstract] [Full Text] [PDF] [Fouroclas et al. Supplementary appendix]  

Suppressor of cytokine signaling 3 (SOCS3) is negative regulator of the JAK/STAT pathway. Methylation of this gene might, therefore, contribute to the pathogenesis of myeloproliferative disorders. In this study, SOCS3 promoter methylation was detected in about one third of patients with idiopathic myelofibrosis suggesting a possible role for SOCS3 methylation in this disorder.

Francesco Passamonti, Elisa Rumi, Luca Arcaini, Emanuela Boveri, Chiara Elena, Daniela Pietra, Sabrina Boggi, Cesare Astori, Paolo Bernasconi, Marzia Varettoni, Ercole Brusamolino, Cristiana Pascutto, Mario Lazzarino

Prognostic factors for thrombosis, myelofibrosis, and leukemia in essential thrombocythemia: a study of 605 patients
Haematologica 2008 93: 1645-1651. Published online September 11, 2008; doi:10.3324/haematol.13346 [Abstract] [Full Text] [PDF]  

The findings from this study on a large series of patients treated according to current clinical practice provide reassurance that essential thrombocythemia is an indolent disorder and affected patients have a long survival.

Oliver Teuffel, Martin Stanulla, Gunnar Cario, Wolf D. Ludwig, Silja Rottgers, Beat W. Schafer, Martin Zimmermann, Martin Schrappe, Felix K. Niggli

Anemia and survival in childhood acute lymphoblastic leukemia
Haematologica 2008 93: 1652-1657. Published online September 24, 2008; doi:10.3324/haematol.13156 [Abstract] [Full Text] [PDF]  

The inverse relationship between severity of anemia and survival found within specific subgroups of patients with childhood acute lymphoblastic leukemia suggests that very low hemoglobin levels at diagnosis are associated with more advanced disease in these subgroups.

Paola Ballerini, Judith Landman-Parker, Jean Michel Cayuela, Vahid Asnafi, Myriam Labopin, Virginie Gandemer, Yves Perel, Gérard Michel, Thierry Leblanc, Claudine Schmitt, Sylvie Fasola, Anne Hagemejier, François Sigaux, Marie Françoise Auclerc, Luc Douay, Guy Leverger, André Baruchel

Impact of genotype on survival of children with T-cell acute lymphoblastic leukemia treated according to the French protocol FRALLE-93: the effect of TLX3/HOX11L2 gene expression on outcome
Haematologica 2008 93: 1658-1665. Published online October 2, 2008; doi:10.3324/haematol.13291 [Abstract] [Full Text] [PDF] [Ballerini et al. - Supplementary Appendix]  

The findings of this study suggest that TLX3 gene expression is an independent risk factor predicting poor survival in childhood T-cell acute lymphoblastic leukemia.

Julie A. Britton, Aneire E. Khan, Sabine Rohrmann, Nikolaus Becker, Jakob Linseisen, Alexandra Nieters, Rudolf Kaaks, Anne Tjønneland, Jytte Halkjær, Marianne Tang Severinsen, Kim Overvad, Tobias Pischon, Heiner Boeing, Antonia Trichopoulou, Victoria Kalapothaki, Dimitrios Trichopoulos, Amalia Mattiello, Giovanna Tagliabue, Carlotta Sacerdote, Petra H.M. Peeters, H. Bas Bueno-de-Mesquita, Eva Ardanaz, Carmen Navarro, Paula Jakszyn, Jone M. Altzibar, Göran Hallmans, Beatrice Malmer, Göran Berglund, Jonas Manjer, Naomi Allen, Timothy Key, Sheila Bingham, Hervé Besson, Pietro Ferrari, Mazda Jenab, Paolo Boffetta, Paolo Vineis, Elio Riboli

Anthropometric characteristics and non-Hodgkin’s lymphoma and multiple myeloma risk in the European Prospective Investigation into Cancer and Nutrition (EPIC)
Haematologica 2008 93: 1666-1677. Published online October 2, 2008; doi:10.3324/haematol.13078 [Abstract] [Full Text] [PDF]  

The incidences of non-Hodgkin’s lymphoma and multiple myeloma are increasing steadily. It has been hypothesized that this may be due, in part, to the parallel rising prevalence of obesity. This European Prospective Investigation into Cancer and Nutrition (EPIC) study supports an association between height and overall non-Hodgkin’s lymphoma and multiple myeloma among women.

Isabella Garagiola, Carla Valsecchi, Silvia Lavoretano, Hale Oren, Martina Bohm, Flora Peyvandi

Nonsense-mediated mRNA decay in the ADAMTS13 gene caused by a 29-nucleotide deletion
Haematologica 2008 93: 1678-1685. Published online October 2, 2008; doi:10.3324/haematol.13102 [Abstract] [Full Text] [PDF] [Garagiola et al. - Supplementary Appendix]  

This study demonstrates that two cases of severe ADAMTS13 deficiency are mechanistically caused by the association of two different gene defects acting at two different levels.

Fumihiko Kimura, Ken Sato, Shinichi Kobayashi, Takashi Ikeda, Hiroshi Sao, Shinichiro Okamoto, Koichi Miyamura, Shinichiro Mori, Hideki Akiyama, Makoto Hirokawa, Hitoshi Ohto, Hiroshi Ashida, Kazuo Motoyoshi for The Japan Marrow Donor Program

Impact of AB0-blood group incompatibility on the outcome of recipients of bone marrow transplants from unrelated donors in the Japan Marrow Donor Program
Haematologica 2008 93: 1686-1693. Published online October 2, 2008; doi:10.3324/haematol.12933 [Abstract] [Full Text] [PDF]  

The findings of this study indicate that major and minor AB0 incompatibility have specific effects on transplant-related mortality and acute graft-versus-host disease in recipients of bone marrow transplants from unrelated donors. See related perspective article on page 1605.

Simone Cesaro, Franco Locatelli, Edoardo Lanino, Fulvio Porta, Lucia Di Maio, Chiara Messina, Arcangelo Prete, Mimmo Ripaldi, Natasha Maximova, Giovanna Giorgiani, Roberto Rondelli, Maurizio Aricò, Franca Fagioli

Hematopoietic stem cell transplantation for hemophagocytic lymphohistiocytosis: a retrospective analysis of data from the Italian Association of Pediatric Hematology Oncology (AIEOP)
Haematologica 2008 93: 1694-1701. Published online September 2, 2008; doi:10.3324/haematol.13142 [Abstract] [Full Text] [PDF]  

This study confirms that hematopoietic stem cell transplantation represents a curative treatment for a large proportion of patients with hemophagocytic lymphohistiocytosis, irrespective of the underlying genetic defect.

Progress in Hematology:

Rick Kapur, Saskia Ebeling, Anton Hagenbeek

B-cell involvement in chronic graft-versus-host disease
Haematologica 2008 93: 1702-1711. Published online August 25, 2008; doi:10.3324/haematol.13311 [Abstract] [Full Text] [PDF]  

This article addresses the pathogenesis of chronic graft-versus-host disease, which is not yet completely understood, with a special focus on the possible role of B cells.

Decision Making and Problem Solving:

Ghulam J. Mufti, John M. Bennett, Jean Goasguen, Barbara J. Bain, Irith Baumann, Richard Brunning, Mario Cazzola, Pierre Fenaux, Ulrich Germing, Eva Hellström-Lindberg, Itsuro Jinnai, Atsushi Manabe, Akira Matsuda, Charlotte M. Niemeyer, Guillermo Sanz, Masao Tomonaga, Teresa Vallespi, Ayami Yoshimi

Diagnosis and classification of myelodysplastic syndrome: International Working Group on Morphology of myelodysplastic syndrome (IWGM-MDS) consensus proposals for the definition and enumeration of myeloblasts and ring sideroblasts
Haematologica 2008 93: 1712-1717. Published online October 6, 2008; doi:10.3324/haematol.13405 [Abstract] [Full Text] [PDF]  

This article details the proposals of the IWGM-MDS for the definition of myeloblasts, promyelocytes and ring side-roblasts in patients with myelodysplastic syndrome.

Brief Reports:

Rama Krishna Kancha, Nikolas von Bubnoff, Cornelius Miething, Christian Peschel, Katharina S. Götze, Justus Duyster

Imatinib and leptomycin B are effective in overcoming imatinib-resistance due to Bcr-Abl amplification and clonal evolution but not due to Bcr-Abl kinase domain mutation
Haematologica 2008 93: 1718-1722. Published online August 25, 2008; doi:10.3324/haematol.13207 [Abstract] [Full Text] [PDF]  

The findings of this study indicate that the combination of imatinib and leptomycin B effectively induces cell death in imatinib-resistant Ba/F3 cells which display Bcr-Abl amplification or signs of clonal evolution.

François Girodon, Céline Schaeffer, Cédric Cleyrat, Morgane Mounier, Ingrid Lafont, Frédéric Dos Santos, Aurélie Vidal, Marc Maynadié, Sylvie Hermouet

Frequent reduction or absence of detection of the JAK2-mutated clone in JAK2V617F-positive patients within the first years of hydroxyurea therapy
Haematologica 2008 93: 1723-1727. Published online August 25, 2008; doi:10.3324/haematol.13081 [Abstract] [Full Text] [PDF]  

In this study, hydroxyurea treatment reduced the percentage of JAK2V617F mutant alleles by >30% in 13/25 patients with essential thrombocythemia or polycythemia vera within the first year after diagnosis.

Tobias Berg, Manfred Fliegauf, Jan Burger, Martin S. Staege, Shaohua Liu, Natalia Martinez, Olaf Heidenreich, Stefan Burdach, Torsten Haferlach, Milton H. Werner, Michael Lübbert

Transcriptional upregulation of p21/WAF/Cip1 in myeloid leukemic blasts expressing AML1-ETO
Haematologica 2008 93: 1728-1733. Published online September 11, 2008; doi:10.3324/haematol.13044 [Abstract] [Full Text] [PDF] [Berg et al. - Supplementary Data]  

The increased expression of p21/WAF/Cip1 in primary leukemic blasts suggests that elevated p21/WAF/Cip1 levels may contribute to specific features observed in AML1-ETO positive leukemia.

Hareth Nahi, Hans Hägglund, Thomas Ahlgren, Per Bernell, Mats Hardling, Karin Karlsson, Vladimir Lj Lazarevic, Mats Linderholm, Bengt Smedmyr, Maria Åström, Helene Hallböök

An investigation into whether deletions in 9p reflect prognosis in adult precursor B-cell acute lymphoblastic leukemia: a multi-center study of 381 patients
Haematologica 2008 93: 1734-1738. Published online August 25, 2008; doi:10.3324/haematol.13227 [Abstract] [Full Text] [PDF]  

The findings of this study suggest that chromosomal abnormalities involving 9p may have a significant negative impact on survival in adult B-precursor acute lymphoblastic leukemia.

Guido Tricot, Bart Barlogie, Maurizio Zangari, Frits van Rhee, Antje Hoering, Jackie Szymonifka, Michele Cottler-Fox

Mobilization of peripheral blood stem cells in myeloma with either pegfilgrastim or filgrastim following chemotherapy
Haematologica 2008 93: 1739-1742. Published online August 25, 2008; doi:10.3324/haematol.13204 [Abstract] [Full Text] [PDF]  

In this study on patients with multiple myeloma, peripheral blood stem cell mobilization after chemotherapy was feasible and similarly effective with pegfilgrastim and filgrastim.

Silvia Vettore, Raffaella Scandellari, Stefano Moro, Anna Maria Lombardi, Margherita Scapin, Maria Luigia Randi, Fabrizio Fabris

Novel point mutation in a leucine-rich repeat of the GPIb chain of the platelet von Willebrand factor receptor, GPIb/IX/V, resulting in an inherited dominant form of Bernard-Soulier syndrome affecting two unrelated families: the N41H variant
Haematologica 2008 93: 1743-1747. Published online September 24, 2008; doi:10.3324/haematol.12830 [Abstract] [Full Text] [PDF] [Vettore et al. - Supplementary data]  

This reports describes a new variant of heterozygous Bernard-Soulier syndrome with autosomal dominant inheritance.

Letters to the Editor:

Paola Quarello, Emanuela Garelli, Alfredo Brusco, Adriana Carando, Patrizia Pappi, Marco Barberis, Valentina Coletti, Maria Francesca Campagnoli, Irma Dianzani, Ugo Ramenghi

Multiplex ligation-dependent probe amplification enhances molecular diagnosis of Diamond-Blackfan anemia due to RPS19 deficiency
Haematologica 2008 93: 1748-1750. Published online October 2, 2008; doi:10.3324/haematol.13423 [Full Text] [PDF]  

Julien Zuber, Kheira Beldjord, Nicole Casadevall, Eric Thervet, Christophe Legendre, Bruno Varet

Immune-mediated pure red cell aplasia in renal transplant recipients
Haematologica 2008 93: 1750-1752. Published online September 11, 2008; doi:10.3324/haematol.13477 [Full Text] [PDF]  

Whitney Tolpinrud, Yelena D. Maksimova, Bernard G. Forget, Patrick G. Gallagher

Nonsense mutations of the -spectrin gene in hereditary pyropoikilocytosis
Haematologica 2008 93: 1752-1754. Published online September 24, 2008; doi:10.3324/haematol.13639 [Full Text] [PDF]  

Caroline Lacoste, Nathalie Bonello-Palot, Katia Gonnet, Françoise Merono, Nicolas Levy, Isabelle Thuret, Catherine Badens

First case of -thalassemia in association with a βS allele: a pitfall in the neonatal screening for sickle cell disease
Haematologica 2008 93: 1754-1755. Published online August 25, 2008; doi:10.3324/haematol.13262 [Full Text] [PDF]  

Mustafa Vakur Bor, Mualla Çetin, Selin Aytaç, Çigdem Altay, Per Magne Ueland, Ebba Nexo

Long term biweekly 1 mg oral vitamin B₁₂ ensures normal hematological parameters, but does not correct all other markers of vitamin B₁₂ deficiency. A study in patients with inherited vitamin B₁₂ deficiency
Haematologica 2008 93: 1755-1758. Published online September 2, 2008; doi:10.3324/haematol.13122 [Full Text] [PDF]  

Ester M. Orlandi, Fausto Baldanti, Annalisa Citro, Lara Pochintesta, Marta Gatti, Mario Lazzarino

Monitoring for cytomegalovirus and Epstein-Barr virus infection in chronic lymphocytic leukemia patients receiving i.v. fludarabine-cyclophosphamide combination and alemtuzumab as consolidation therapy
Haematologica 2008 93: 1758-1760. Published online September 11, 2008; doi:10.3324/haematol.13265 [Full Text] [PDF]  

Erratum:

Erratum
Haematologica 2008 93: 1760. [Full Text] [PDF]  

Online Only Articles:

A. Torresin, M. Feasi, G. Cassola

Hepatitis B reactivation prophylaxis in immune-suppressed patients
Haematologica 2008 93: e65. [Full Text] [PDF]  

C. Targhetta, M.G. Cabras, E. Angelucci

Risk of HBV liver disease in isolated antiHbc patients receiving immuno-chemotherapy for non Hodgkin lymphoma
Haematologica 2008 93: e66. [Full Text] [PDF]  

A.K. Lukusa, C. Vermylen

Use of hydroxyurea from childhood to adult age in sickle cell disease: semen analysis
Haematologica 2008 93: e67. [Full Text] [PDF]  

I. Berthaut, R. Girot, J. Mandelbaum

Reply to: [Use of hydroxyurea from childhood to adult age in sickle cell disease: semen analysis. Haematologica 2008; 93:e67]
Haematologica 2008 93: e68. [Full Text] [PDF]  

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