Editorials and Perspectives:

Clara Camaschella, Laura Silvestri

New and old players in the hepcidin pathway
Haematologica 2008 93: 1441-1444. [Full Text] [PDF]  

The identification of the liver peptide hepcidin at the beginning of the new millennium opened a new era in our understanding of iron metabolism. In this perspective article, Dr. Camaschella and Silvestri summarize current knowledge of the hepcidin pathway and of the regulation of the hepcidin expression. In particular, they discuss the function of matriptase-2, a transmembrane serine protease that is encoded by the TMPRSS6 gene and suppresses hepcidin production. See related paper on pages 1466, 1473 and 1550.

James N. George, Deirdra R. Terrell

Novel thrombopoietic agents: a new era for management of patients with thrombocytopenia
Haematologica 2008 93: 1445-1449. [Full Text] [PDF]  

After many decades of an orphan existence, immune thrombocytopenic purpura is a central focus for the clinical development of multiple, novel thrombopoietic agents. In this perspective article, Drs. George and Terrell describe the molecular spectrum of thrombopoietic agents that are currently under development. See related paper on page 1495.

Damiano Rondelli

Allogeneic hematopoietic stem cell transplantation for myelofibrosis
Haematologica 2008 93: 1449-1450. [Full Text] [PDF]  

Primary myelofibrosis or myelofibrosis secondary to polycythemia vera or essential thrombocythemia can be cured only by means of allogeneic hematopoietic stem cell transplantation at present. In this perspective article, Dr. Rondelli discusses which patients are the best candidates for transplantation, and the optimal transplantation procedures. See related paper on page 1514.

Daniel J. Powell, Jr, Bruce L. Levine

Adoptive T-cell therapy for malignant disorders
Haematologica 2008 93: 1452-1456. [Full Text] [PDF]  

Adoptive cell transfer using ex vivo manipulated T lymphocytes has emerged as an important advance in cancer immunotherapy, allowing for re-education and re-setting of the host immune system. In this perspective article, Drs. Powell and Levine discuss the current situation in this field. See related paper on page 1523.

Original Articles:

Hematopoietic Stem Cells:

Charalampia Kyriakou, Neil Rabin, Arnold Pizzey, Amit Nathwani, Kwee Yong

Factors that influence short-term homing of human bone marrow-derived mesenchymal stem cells in a xenogeneic animal model
Haematologica 2008 93: 1457-1465. Published online August 25, 2008; doi:10.3324/haematol.12553 [Abstract] [Full Text] [PDF] [Kyriakou - Supplementary Appendix]  

Human mesenchymal stem cells are potential agents for tissue regeneration, enhancing hematopoietic stem cell transplantation and delivering genes of therapeutic interest. This study shows that tissue homing of systemically administered mesenchymal stem cells can be increased by enforced expression of CXCR4, at least in irradiated hosts.

Iron Metabolism:

Alessia Pagani, Laura Silvestri, Antonella Nai, Clara Camaschella

Hemojuvelin N-terminal mutants reach the plasma membrane but do not activate the hepcidin response
Haematologica 2008 93: 1466-1472. [Abstract] [Full Text] [PDF]  

Hemojuvelin positively modulates the iron regulator hepcidin. Mutations of the gene encoding for hemojuvelin cause juvenile hemochromatosis, characterized by hepcidin deficiency and severe iron overload. This study shows that the delayed export and retention in the endoplasmic reticulum of some N-terminal mutants could contribute to the pathogenesis of juvenile hemochromatosis. See related perspective article on page 1441.

Disorders of Iron Metabolism:

Maria Antonietta Melis, Milena Cau, Rita Congiu, Gabriella Sole, Susanna Barella, Antonio Cao, Mark Westerman, Mario Cazzola, Renzo Galanello

A mutation in the TMPRSS6 gene, encoding a transmembrane serine protease that suppresses hepcidin production, in familial iron deficiency anemia refractory to oral iron
Haematologica 2008 93: 1473-1479. Published online July 4, 2008; doi:10.3324/haematol.13342 [Abstract] [Full Text] [PDF]  

Previous studies have described a familial syndrome characterized by iron malabsorption, hypoferremia, and microcytic anemia that did not respond to oral iron and responded only partly to parenteral iron. In this work, Melis and coworkers studied a Sardinian family with this inherited condition. They found a homozygous causal mutation in the TMPRSS6 gene in affected members, who had inappropriately elevated levels of serum and urinary hepcidin. See related perspective article on page 1441.

Acute Myeloid Leukemia:

Jing Zhang, Li-Ping Song, Ying Huang, Qian Zhao, Ke-Wen Zhao, Guo-Qiang Chen

Accumulation of hypoxia-inducible factor-1 protein and its role in the differentiation of myeloid leukemic cells induced by all-trans retinoic acid
Haematologica 2008 93: 1480-1487. Published online August 25, 2008; doi:10.3324/haematol.13096 [Abstract] [Full Text] [PDF]  

That hypoxia-inducible factor-1 (HIF-1) contributes to the differentiation of acute myeloid leukemia cells via transcriptional activity-independent mechanisms. This study shows that increased HIF-1 levels contribute to all-trans retinoic acid-induced differentiation of acute myeloid leukemia cells cells.

Acute Lymphoblastic Leukemia:

Mathilde Hunault-Berger, Patrice Chevallier, Martine Delain, Claude-Eric Bulabois, Serge Bologna, Marc Bernard, Ingrid Lafon, Jérome Cornillon, Abdallah Maakaroun, Alexandra Tizon, Bruno Padrazzi, Norbert Ifrah, Yves Gruel for the GOELAMS (Groupe Ouest-Est des Leucémies Aiguës et Maladies du Sang)

Changes in antithrombin and fibrinogen levels during induction chemotherapy with L-asparaginase in adult patients with acute lymphoblastic leukemia or lymphoblastic lymphoma. Use of supportive coagulation therapy and clinical outcome: the CAPELAL study
Haematologica 2008 93: 1488-1494. Published online August 25, 2008; doi:10.3324/haematol.12948 [Abstract] [Full Text] [PDF]  

The effects of L-asparaginase on hemostasis during induction chemotherapy of acute lymphoblastic leukemia of lymphoblastic lymphoma are less defined in adults than in children. This retrospective study suggests that antithrombin concentrates may have a beneficial effect on the outcome of adults treated for acute lymphoblastic leukemia with L-asparaginase.

Thrombopoiesis:

Wataru Nogami, Hiroshi Yoshida, Kenzo Koizumi, Hajime Yamada, Kenji Abe, Akinori Arimura, Noriko Yamane, Koji Takahashi, Akiko Yamane, Atsushi Oda, Yoshikazu Tanaka, Hiroshi Takemoto, Yasuyuki Ohnishi, Yasuo Ikeda, Yoshitaka Miyakawa

The effect of a novel, small non-peptidyl molecule butyzamide on human thrombopoietin receptor and megakaryopoiesis
Haematologica 2008 93: 1495-1504. Published online August 25, 2008; doi:10.3324/haematol.12752 [Abstract] [Full Text] [PDF]  

Thrombocytopenia is found in several conditions, and in many of them no treatment is available apart from platelet transfusion. Findings of this study suggest that butyzamide, an orally bioavailable human Mpl activator, may increase platelet production. See related perspective article on page 1445.

Disorders of Coagulation:

Claudia Dall'Osso, Ilaria Guella, Stefano Duga, Nadia Locatelli, Elvezia Maria Paraboschi, Marta Spreafico, Abdolreza Afrasiabi, Christoph Pechlaner, Flora Peyvandi, Maria Luisa Tenchini, Rosanna Asselta

Molecular characterization of three novel splicing mutations causing factor V deficiency and analysis of the F5 gene splicing pattern
Haematologica 2008 93: 1505-1513. Published online August 25, 2008; doi:10.3324/haematol.12934 [Abstract] [Full Text] [PDF]  

Factor V deficiency is a rare autosomal recessive hemorrhagic disorder, associated with bleeding manifestations of variable severity. This paper reports the functional consequences of three novel splicing mutations leading to FV deficiency.

Stem Cell Transplantation:

Francesca Patriarca, Andrea Bacigalupo, Alessandra Sperotto, Miriam Isola, Franca Soldano, Barbara Bruno, Maria Teresa van Lint, Anna Paola Iori, Stella Santarone, Ferdinando Porretto, Pietro Pioltelli, Giuseppe Visani, Pasquale Iacopino, Renato Fanin, Alberto Bosi on behalf of the GITMO

Allogeneic hematopoietic stem cell transplantation in myelofibrosis: the 20-year experience of the Gruppo Italiano Trapianto di Midollo Osseo (GITMO)
Haematologica 2008 93: 1514-1522. Published online August 25, 2008; doi:10.3324/haematol.12828 [Abstract] [Full Text] [PDF]  

In this GITMO study, Patriarca and coworkers evaluated the outcome of patients with myelofibrosis who underwent allogeneic stem cell transplantation, and the impact of prognostic factors. They conclude that the outcome of myelofibrosis patients who underwent allogeneic stem cell transplantation significantly improved after 1996 due to the reduction in transplant-related mortality. See related perspective article on page 1449.

Immunotherapy:

Roberta Zappasodi, Massimo Di Nicola, Carmelo Carlo-Stella, Roberta Mortarini, Alessandra Molla, Claudia Vegetti, Salvatore Albani, Andrea Anichini, Alessandro M. Gianni

The effect of artificial antigen-presenting cells with preclustered anti-CD28/-CD3/-LFA-1 monoclonal antibodies on the induction of ex vivo expansion of functional human antitumor T cells
Haematologica 2008 93: 1523-1534. Published online August 25, 2008; doi:10.3324/haematol.12521 [Abstract] [Full Text] [PDF] [Zappasodi et al. - Supplementary Appendix]  

The aim of this study was to engineer an artificial APC-based system with the properties of a fluid cellular membrane and the flexibility derived from an artificial structure that could be tailored to carry the desired immunostimulatory molecules. The authors conclude that their artificial antigen-presenting cells might represent an efficient tool to rapidly obtain a sufficient number of functional T cells for adoptive immunotherapy in patients with cancer. See related perspective article on page 1452.

Marieke Griffioen, H.M. Esther van Egmond, Helen Barnby-Porritt, Menno A.W.G. van der Hoorn, Renate S. Hagedoorn, Michel G.D. Kester, Nikolai Schwabe, Roel Willemze, J.H. Frederik Falkenburg, Mirjam H.M. Heemskerk

Genetic engineering of virus-specific T cells with T-cell receptors recognizing minor histocompatibility antigens for clinical application
Haematologica 2008 93: 1535-1543. Published online September 2, 2008; doi:10.3324/haematol.13067 [Abstract] [Full Text] [PDF]  

Donor lymphocyte infusion is an effective form of adoptive immunotherapy for hematologic malignancies after allogeneic stem cell transplantation, but is frequently associated with development of graft-versus-host disease. This study describes an efficient method for generating T-cell receptors-engineered virus-specific T cells, which may provide effective adoptive anti-tumor therapy after allogeneic stem cell transplantation, with a low risk of graft-versus-host disease.

Decision Making and Problem Solving:

Chronic Myeloid Leukemia:

Hermann Brenner, Adam Gondos, Dianne Pulte

Recent trends in long-term survival of patients with chronic myelocytic leukemia: disclosing the impact of advances in therapy on the population level
Haematologica 2008 93: 1544-1549. Published online July 18, 2008; doi:10.3324/haematol.13045 [Abstract] [Full Text] [PDF]  

This analysis discloses a dramatic recent increase in long-term survival of younger patients with chronic myeloid leukemia, which most likely reflects rapid dissemination of advances in therapy on the population level.

Brief Report:

Disorders of Erythropoiesis:

Junya Kanda, Chisaki Mizumoto, Hiroshi Kawabata, Hideyuki Tsuchida, Naohisa Tomosugi, Keitaro Matsuo, Takashi Uchiyama

Serum hepcidin level and erythropoietic activity after hematopoietic stem cell transplantation
Haematologica 2008 93: 1550-1554. Published online July 18, 2008; doi:10.3324/haematol.12399 [Abstract] [Full Text] [PDF]  

In this study, an inverse relationship between erythroid activity and serum hepcidin was found after hematopoietic stem cell transplantation.

Naohito Fujishima, Ken-ichi Sawada, Makoto Hirokawa, Kazuo Oshimi, Koichi Sugimoto, Akira Matsuda, Masanao Teramura, Masamitsu Karasawa, Ayako Arai, Yuji Yonemura, Shinji Nakao, Akio Urabe, Mitsuhiro Omine, Keiya Ozawa

Long-term responses and outcomes following immunosuppressive therapy in large granular lymphocyte leukemia-associated pure red cell aplasia: a Nationwide Cohort Study in Japan for the PRCA Collaborative Study Group
Haematologica 2008 93: 1555-1559. Published online July 18, 2008; doi:10.3324/haematol.12871 [Abstract] [Full Text] [PDF]  

This report describes long-term responses following immunosuppressive therapy in large granular lymphocyte leukemia-associated pure red cell aplasia.

Myeloproliferative Disorders:

Amy V. Jones, Nicholas C.P. Cross, Helen E. White, Anthony R. Green, Linda M. Scott

Rapid identification of JAK2 exon 12 mutations using high resolution melting analysis
Haematologica 2008 93: 1560-1564. Published online August 12, 2008; doi:10.3324/haematol.12883 [Abstract] [Full Text] [PDF]  

In this study, high resolution melting analysis was found to be a rapid, sensitive and high-throughput technique for screening for JAK2 exon 12 mutations.

Acute Myeloid Leukemia:

Chetsada Boonthimat, Wanna Thongnoppakhun, Chirayu U. Auewarakul

Nucleophosmin mutation in Southeast Asian acute myeloid leukemia: eight novel variants, FLT3 coexistence and prognostic impact of NPM1/FLT3 mutations
Haematologica 2008 93: 1565-1569. Published online July 18, 2008; doi:10.3324/haematol.12937 [Abstract] [Full Text] [PDF]  

This report shows that NPM1 mutations were most prevalent in Thai patients with acute myeloid leukemia with normal karyotype.

Mariel L. te Winkel, Inge M. Appel, Rob Pieters, Marry M. van den Heuvel-Eibrink

Impaired dexamethasone-related increase of anticoagulants is associated with the development of osteonecrosis in childhood acute lymphoblastic leukemia
Haematologica 2008 93: 1570-1574. Published online August 12, 2008; doi:10.3324/haematol.12956 [Abstract] [Full Text] [PDF]  

In childhood acute lymphoblastic leukemia, a hypercoagulable state may result from a lower dexamethasone-related increase of antithrombin and protein S and the subsequent decline of these anticoagulants below normal levels after introduction of asparaginase.

Lymphoproliferative Disorders:

Davide Rossi, Antonella Zucchetto, Francesca Maria Rossi, Daniela Capello, Michaela Cerri, Clara Deambrogi, Stefania Cresta, Silvia Rasi, Lorenzo De Paoli, Chiara Lobetti Bodoni, Pietro Bulian, Giovanni Del Poeta, Marco Ladetto, Valter Gattei, Gianluca Gaidano

CD49d expression is an independent risk factor of progressive disease in early stage chronic lymphocytic leukemia
Haematologica 2008 93: 1575-1579. Published online July 18, 2008; doi:10.3324/haematol.13103 [Abstract] [Full Text] [PDF] [Rossi et al. - Supplementary appendix]  

Findings of this study suggest that CD49d expression may be useful for the initial prognostic assessment of patients with chronic lymphocytic leukemia in the initial stage.

Letters to the Editor:

Hematopoietic Stem Cells:

Sake van Wageningen, Gorica Nikoloski, Gerty Vierwinden, Ruth Knops, Bert A. van der Reijden, Joop H. Jansen

The transcription factor nuclear factor Y regulates the proliferation of myeloid progenitor cells
Haematologica 2008 93: 1580-1582. Published online August 12, 2008; doi:10.3324/haematol.12425 [Full Text] [PDF]  

Disorders of Heme Synthesis:

Jonathan S. Caudill, Hamayun Imran, Julie C. Porcher, David P. Steensma

Congenital sideroblastic anemia associated with germline polymorphisms reducing expression of FECH
Haematologica 2008 93: 1582-1584. Published online August 12, 2008; doi:10.3324/haematol.12597 [Full Text] [PDF]  

Thalassemia Syndromes:

Ali Taher, Fuad El Rassi, Hussain Isma’eel, Suzane Koussa, Adlette Inati, Maria Domenica Cappellini

Correlation of liver iron concentration determined by R2 magnetic resonance imaging with serum ferritin in patients with thalassemia intermedia
Haematologica 2008 93: 1584-1586. Published online August 25, 2008; doi:10.3324/haematol.13098 [Full Text] [PDF]  

Anwar H. Nassar, Mohammed Naja, Claudia Cesaretti, Bruno Eprassi, Maria Domenica Cappellini, Ali Taher

Pregnancy outcome in patients with β-thalassemia intermedia at two tertiary care centers, in Beirut and Milan
Haematologica 2008 93: 1586-1587. Published online August 12, 2008; doi:10.3324/haematol.13152 [Full Text] [PDF]  

Ersi Voskaridou, Dimitrios Christoulas, Marialena Konstantinidou, Evangelos Tsiftsakis, Panagiotis Alexakos, Evangelos Terpos

Continuous improvement of bone mineral density two years post zoledronic acid discontinuation in patients with thalassemia-induced osteoporosis: long-term follow-up of a randomized, placebo-controlled trial
Haematologica 2008 93: 1588-1590. Published online August 12, 2008; doi:10.3324/haematol.12849 [Full Text] [PDF]  

Disorders of Bilirubin Metabolism:

François M. Petit, Marylise Hébert, Vincent Gajdos, Liliane Capel, Ridha M’Rad, Philippe Labrune

Large deletion in UGT1A1 gene encompassing the promoter and the exon 1 responsible for Crigler-Najjar type I syndrome
Haematologica 2008 93: 1590-1591. Published online August 12, 2008; doi:10.3324/haematol.13295 [Full Text] [PDF]  

Pediatric Leukemias:

Kiminori Terui, Tomohiko Sato, Shinya Sasaki, Ko Kudo, Takuya Kamio, Etsuro Ito

Two novel variants of MOZ-CBP fusion transcripts in spontaneously remitted infant leukemia with t(1;16;8)(p13;p13;p11), a new variant of t(8;16)(p11;p13)
Haematologica 2008 93: 1591-1593. Published online August 12, 2008; doi:10.3324/haematol.13020 [Full Text] [PDF]  

Myeloproliferative Disorders:

Sandeep Chunduri, Sujata Gaitonde, Stefan O. Ciurea, Ronald Hoffman, Damiano Rondelli

Pulmonary extramedullary hematopoiesis in patients with myelofibrosis undergoing allogeneic stem cell transplantation
Haematologica 2008 93: 1593-1595. Published online July 18, 2008; doi:10.3324/haematol.13203 [Full Text] [PDF]  

Acute Myeloid Leukemia:

Saman Abbas, Gabriëlle Rotmans, Bob Löwenberg, Peter J.M. Valk

Exon 8 splice site mutations in the gene encoding the E3-ligase CBL are associated with core binding factor acute myeloid leukemias
Haematologica 2008 93: 1595-1597. Published online August 12, 2008; doi:10.3324/haematol.13187 [Full Text] [PDF]  

Malignant Lymphomas:

Kathryn Scott, Peter J. Adamson, Eleanor V. Willett, Lisa J. Worrillow, James M. Allan

Genetic variation in genes expressed in the germinal center and risk of B cell lymphoma among Caucasians
Haematologica 2008 93: 1597-1600. Published online July 18, 2008; doi:10.3324/haematol.13159 [Full Text] [PDF]  

Online Only Articles:

Avshalom Zoossmann-Diskin

Factor XI mutation and the origin of Ashkenazi Jews
Haematologica 2008 93: e59. [Full Text] [PDF]  

G. Zadra, R. Asselta, M.L. Tenchini, G. Castaman, U. Seligsohn, P.M. Mannucci, S. Duga

Reply to: [Factor XI mutation and the origin of Ashkenazi Jews. Haematologica 2008; 93:e59]
Haematologica 2008 93: e60. [Full Text] [PDF]  

D.R. Choudhary, R. Naithani, M. Mahapatra, R. Kumar, P. Mishra, R. Saxena

Efficacy of cyclosporine as a single agent therapy in chronic idiopathic thrombocytopenic purpura
Haematologica 2008 93: e61-e62. [Full Text] [PDF]  

G. Emilia, M. Luppi, M. Morselli, F. Forghieri, L. Potenza, G. Torelli

Reply to: [Efficacy of cyclosporine as a single agent therapy in chronic idiopathic thrombocytopenic purpura". Haematologica 2008; 93:e61]
Haematologica 2008 93: e63. [Full Text] [PDF]  

R.S. Carel

Reply to: [Non-Hodgkin’s lymphoma and residential proximity to toxic industrial waste in Southern Israel. Haematologica 2005; 90:1709-10]
Haematologica 2008 93: e64. [Full Text] [PDF]  

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