Editorials and Perspectives:

Mark T. Gladwin, Gregory J. Kato

Hemolysis-associated hypercoagulability in sickle cell disease: the plot (and blood) thickens!
Haematologica 2008 93: 1-3. [Full Text] [PDF]  

Thrombophilia and hemostatic activation common to most hemolytic conditions are likely explained by the very feature they all share, intravascular hemolysis. See related article on page 20.

Luca Malcovati, Mario Cazzola

Myelodysplastic/myeloproliferative disorders
Haematologica 2008 93: 4-6. [Full Text] [PDF]  

Myelodysplastic/myeloproliferative disorders are defined as myeloid disorders that have both dysplastic and proliferative features at the time of initial presentation. Mutations of JAK2 and MPL have been reported with the prototype of these disorders, refractory anemia with ringed sideroblasts associated with marked thrombocytosis. See related article on page 34

Ayalew Tefferi, Srdan Verstovsek, Animesh Pardanani

How we diagnose and treat WHO-defined systemic mastocytosis in adults
Haematologica 2008 93: 6-9. [Full Text] [PDF]  

Current diagnosis and treatment of systemic mastocytosis are challenging for clinicians. This perspective summarizes the diagnostic approach and the treatment strategy adopted at the Mayo Clinic, Rochester, MN, and the M.D. Anderson Cancer Center, Houston, TX. See related article on page 49.

Jessica Hochberg, Mitchell S. Cairo

Tumor lysis syndrome: current perspective
Haematologica 2008 93: 9-13. [Full Text] [PDF]  

Tumor lysis syndrome is characterized by a group of metabolic derangements caused by the rapid lysis of malignant cells. This condition can lead to acute renal failure and even death. The early recognition and treatment of metabolic abnormalities usually prevents the life threatening complications associated with tumor lysis syndrome. See related article on page 67.

Alexander W. Hauswirth, Ulrich Jäger

Impact of cytogenetic and molecular prognostic markers on the clinical management of chronic lymphocytic leukemia
Haematologica 2008 93: 14-19. [Full Text] [PDF]  

Genetic markers have contributed important insights into the biology of chronic lymphocytic leukemia. The available evidence suggests that these markers will increasingly influence treatment decisions for tailored therapy. See related article on page 75.

Original Articles:

Red Cell Disorders:

Kenneth I. Ataga, Charity G. Moore, Cheryl A. Hillery, Susan Jones, Herbert C. Whinna, Dell Strayhorn, Cathy Sohier, Alan Hinderliter, Leslie V. Parise, Eugene P. Orringer

Coagulation activation and inflammation in sickle cell disease-associated pulmonary hypertension
Haematologica 2008 93: 20-26. [Abstract] [Full Text] [PDF]  

Patients with sickle cell disease-associated pulmonary hypertension have increased endothelial dysfunction, coagulation activation and inflammation compared with patients without pulmonary hypertension. Endothelial dysfunction and coagulation activation appear to be the result of chronic hemolysis. See related perspective on page 1.

Bone Marrow Failure:

Makoto Hirokawa, Ken-ichi Sawada, Naohito Fujishima, Shinji Nakao, Akio Urabe, Kazuo Dan, Shin Fujisawa, Yuji Yonemura, Fumio Kawano, Mitsuhiro Omine, Keiya Ozawa for the PRCA Collaborative Study Group

Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group
Haematologica 2008 93: 27-33. [Abstract] [Full Text] [PDF]  

This nationwide survey in Japan indicates that thymoma-associated pure red cell aplasia responds in most instances to cyclosporine A, and that this treatment is also effective in preventing relapse of anemia.

Myelodysplastic/Myeloproliferative Disorders:

Annette H. Schmitt-Graeff, Soon-Siong Teo, Manfred Olschewski, Franz Schaub, Sabine Haxelmans, Andreas Kirn, Petra Reinecke, Ulrich Germing, Radek C. Skoda

JAK2^V617F mutation status identifies subtypes of refractory anemia with ringed sideroblasts associated with marked thrombocytosis
Haematologica 2008 93: 34-40. [Abstract] [Full Text] [PDF] [Schmitt-Graeff et al. - Design and Methods]  

Refractory anemia with ringed sideroblasts and marked thrombocytosis is a condition with both myelodysplastic and myeloproliferative features. This study indicates that a considerable proportion of patients with this condition carry the unique V617F mutation of JAK2, and that the mutant allele burden increases over time. See related perspective on page 4.

Myeloproliferative Disorders:

Elisabetta Antonioli, Paola Guglielmelli, Giada Poli, Costanza Bogani, Alessandro Pancrazzi, Giovanni Longo, Vanessa Ponziani, Lorenzo Tozzi, Lisa Pieri, Valeria Santini, Alberto Bosi, Alessandro M. Vannucchi for the Myeloproliferative Disorders Research Consortium (MPD-RC)

Influence of JAK2^V617F allele burden on phenotype in essential thrombocythemia
Haematologica 2008 93: 41-48. [Abstract] [Full Text] [PDF]  

Variable proportions of mutant alleles are found in patients with JAK2 (V617F)-positive myeloproliferative disorders. This study shows that this variable mutant allele burden influences the clinical phenotype of JAK2 (V617F)-positive essential thrombocythemia.

Idoya Lahortiga, Cem Akin, Jan Cools, Todd M. Wilson, Nicole Mentens, Diane C. Arthur, Irina Maric, Pierre Noel, Can Kocabas, Peter Marynen, Lawrence S. Lessin, Iwona Wlodarska, Jamie Robyn, Dean D. Metcalfe

Activity of imatinib in systemic mastocytosis with chronic basophilic leukemia and a PRKG2-PDGFRB fusion
Haematologica 2008 93: 49-56. [Abstract] [Full Text] [PDF]  

Systemic mastocytosis with an associated hematologic non-mast cell lineage disease is a rare subtype of systemic mastocytosis. This study describes a patient with systemic mastocytosis associated with chronic basophilic leukemia and a PRKG2-PDGFRB fusion gene. This patient had a complete response to imatinib mesylate. See related perspective on page 6.

Acute Myeloid Leukemia:

Enrique Colado, Stela Álvarez-Fernández, Patricia Maiso, Jesús Martín-Sánchez, Maria Belén Vidriales, Mercedes Garayoa, Enrique M. Ocio, Juan Carlos Montero, Atanasio Pandiella, Jesús F. San Miguel

The effect of the proteasome inhibitor bortezomib on acute myeloid leukemia cells and drug resistance associated with the CD34⁺ immature phenotype
Haematologica 2008 93: 57-66. [Abstract] [Full Text] [PDF]  

This study shows that bortezomib induces apoptosis of acute myeloid leukemia cells in vitro. Ad hoc clinical trials might investigate the efficacy of this drug in patients with acute myeloid leukemia.

Pau Montesinos, Ignacio Lorenzo, Guillermo Martín, Jaime Sanz, Maria Luz Pérez-Sirvent, David Martínez, Guillermo Ortí, Lorenzo Algarra, Jesus Martínez, Federico Moscardó, Javier de la Rubia, Isidro Jarque, Guillermo Sanz, Miguel A. Sanz

Tumor lysis syndrome in patients with acute myeloid leukemia: identification of risk factors and development of a predictive model
Haematologica 2008 93: 67-74. [Abstract] [Full Text] [PDF]  

Tumor lysis syndrome may represent a life-threatening complication during induction chemotherapy of acute myeloid leukemia. This study shows that pretreatment elevated serum lactate dehydrogenase, increased serum creatinine, high uric acid, and markedly elevated white blood cell counts represent independent risk factors for this complication. See related perspective on page 9.

Chronic Lymphocytic Leukemia:

Eva Giné, Marta Crespo, Ana Muntañola, Eva Calpe, Maria Joao Baptista, Neus Villamor, Emili Montserrat, Francesc Bosch

Induction of histone H1.2 cytosolic release in chronic lymphocytic leukemia cells after genotoxic and non-genotoxic treatment
Haematologica 2008 93: 75-82. [Abstract] [Full Text] [PDF]  

Nuclear histone H1.2 plays an important role in transmitting apoptotic signals from the nucleus to the mitochondria following DNA double-strand breaks. This study shows that histone H1.2 is released to the cytoplasm of primary neoplastic cells from patients with chronic lymphocytic leukemia following different treatment modalities. See related perspective on page 14.

Hemostasis:

Suryasarathi Dasgupta, Ana Maria Navarrete, Sebastien André, Bharath Wootla, Sandrine Delignat, Yohann Repessé, Jagadeesh Bayry, Antonino Nicoletti, Evgueni L. Saenko, Roseline d’Oiron, Marc Jacquemin, Jean-Marie Saint-Remy, Srini V. Kaveri, Sebastien Lacroix-Desmazes

Factor VIII bypasses CD91/LRP for endocytosis by dendritic cells leading to T-cell activation
Haematologica 2008 93: 83-89. [Abstract] [Full Text] [PDF] [Dasgupta et al. Additional Figure]  

Alloimmunization against exogenous factor VIII represents the major hurdle of hemophilia A treatment. This study shows that CD91 and other members of the LDL receptor family are not involved in factor VIII internalization by monocyte-derived dendritic cells.

Progess in Hematology:

Disorders of Iron Metabolism:

Erwin H.J.M. Kemna, Harold Tjalsma, Hans L. Willems, Dorine W. Swinkels

Hepcidin: from discovery to differential diagnosis
Haematologica 2008 93: 90-97. [Abstract] [Full Text] [PDF] [Kemna et al. Additional Data]  

Hepcidin was discovered in 2000. This cysteine-rich peptide was named hepcidin because of its origin in the liver and its antimicrobial properties. Subsequent studies showed that hepcidin is the principal regulator of body iron homeostasis. This review article examines our current knowledge of the pathophysiology of hepcidin.

Decision Making and Problem Solving:

Platelet Disorders:

Marco Ruggeri, Stefania Fortuna, Francesco Rodeghiero

Heterogeneity of terminology and clinical definitions in adult idiopathic thrombocytopenic purpura: a critical appraisal from a systematic review of the literature
Haematologica 2008 93: 98-103. [Abstract] [Full Text] [PDF] [Ruggeri et al. Additional Tables]  

This systematic review of the literature shows that confusing terminology and an unacceptable heterogeneity of clinical definitions are used for management decisions in adult patients with idiopathic thrombocytopenic purpura. Standardization of terminology and definitions is needed.

Infectious Complications:

Morena Caira, Corrado Girmenia, Caterina Giovanna Valentini, Maurizio Sanguinetti, Alessandro Bonini, Giovanni Rossi, Luana Fianchi, Giuseppe Leone, Livio Pagano

Scedosporiosis in patients with acute leukemia: a retrospective multicenter report
Haematologica 2008 93: 104-110. [Abstract] [Full Text] [PDF]  

Scedosporiosis is a rare infectious complication in patients with acute myeloid leukemia. This report confirms that scedosporiosis is mainly a complication of acute myeloid leukemia, and that its outcome is very poor.

Brief Reports:

Red Cell Disorders:

Emilie Camberlein, Giuliana Zanninelli, Lénaïck Détivaud, Anna Rita Lizzi, Francesco Sorrentino, Stefania Vacquer, Marie-Bérengère Troadec, Emanuele Angelucci, Emmanuelle Abgueguen, Olivier Loréal, Paolo Cianciulli, Maria Eliana Lai, Pierre Brissot

Anemia in β-thalassemia patients targets hepatic hepcidin transcript levels independently of iron metabolism genes controlling hepcidin expression
Haematologica 2008 93: 111-115. [Abstract] [Full Text] [PDF]  

In thalassemia patients, hepcidin mRNA expression was inversely related to erythroid activity, indicating that erythroid marrow expansion inhibits hepatic production of this peptide.

Wing-Yan Au, Wynnie Wai-Man Lam, Winnie Chu, Sidney Tam, Wai-Keng Wong, Raymond Liang, Shau-Yin Ha

A T2* magnetic resonance imaging study of pancreatic iron overload in thalassemia major
Haematologica 2008 93: 116-119. [Abstract] [Full Text] [PDF] [Au et al. - Additional Tables]  

This study show that pancreatic iron overload cannot be adequately predicted by serum ferritin concentration, and that it correlates with cardiac iron overload.

Myeloproliferative Disorders:

Marjolein L. Donker, Jasper J. van Doormaal, Frederiek F. van Doormaal, Philip M. Kluin, Eveline van der Veer, Jan G.R. de Monchy, Ido P. Kema, Hanneke C. Kluin-Nelemans

Biochemical markers predictive for bone marrow involvement in systemic mastocytosis
Haematologica 2008 93: 120-123. [Abstract] [Full Text] [PDF]  

Serum tryptase and urinary histamine metabolites are useful predictive markers for bone marrow involvement in indolent systemic mastocytosis. See related perspective on page 6.

Multiple Myeloma:

Henk M. Lokhorst, Ingo Schmidt-Wolf, Pieter Sonneveld, Bronno van der Holt, Hans Martin, Rene Barge, Uta Bertsch, Jana Schlenzka, Gerard M.J. Bos, Sandra Croockewit, Sonja Zweegman, Iris Breitkreutz, Peter Joosten, Christof Scheid, Marinus van Marwijk-Kooy, Hans-Juergen Salwender, Marinus H.J. van Oers, Ron Schaafsma, Ralph Naumann, Harm Sinnige, Igor Blau, Michel Delforge, Okke de Weerdt, Pierre Wijermans, Shulamiet Wittebol, Ulrich Duersen, Edo Vellenga, Hartmut Goldschmidt for Dutch-Belgian HOVON and German GMMG

Thalidomide in induction treatment increases the very good partial response rate before and after high-dose therapy in previously untreated multiple myeloma
Haematologica 2008 93: 124-127. [Abstract] [Full Text] [PDF] [Errata Corrige]  

Thalidomide as part of initial treatment of multiple myeloma improves pre- and post-transplant response by increasing the proportion of patients achieving a very good partial response.

Malignant Lymphomas:

Catherine Ansquer, Thomas Hervouët, Anne Devillers, Sophie de Guibert, Thomas Gastinne, Steven Le Gouill, Etienne Garin, Anne Moreau, Françoise Kraeber-Bodéré, Thierry Lamy

18-F FDG-PET in the staging of lymphocyte-predominant Hodgkin’s disease
Haematologica 2008 93: 128-131. [Abstract] [Full Text] [PDF]  

In this retrospective study, FDG-PET proved to be useful in the staging of lymphocyte-predominant Hodgkin's disease.

Chronic Lymphocytic Leukemia:

Verònica Fernàndez, Pedro Jares, Itziar Salaverria, Eva Giné, Sílvia Beà, Marta Aymerich, Dolors Colomer, Neus Villamor, Francesc Bosch, Emili Montserrat, Elias Campo

Gene expression profile and genomic changes in disease progression of early-stage chronic lymphocytic leukemia
Haematologica 2008 93: 132-136. [Abstract] [Full Text] [PDF] [Fernandez et al. - Additional Figure]  

Clinical progression of early-stage chronic lymphocytic leukemia was associated with karyotype evolution and modulation of the expression of genes involved in cell adhesion and motility. See related perspective on page 14.

Hemostasis:

Clemens L. Bockmeyer, Ralf A. Claus, Ulrich Budde, Karim Kentouche, Reinhard Schneppenheim, Wolfgang Lösche, Konrad Reinhart, Frank M. Brunkhorst

Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor
Haematologica 2008 93: 137-140. [Abstract] [Full Text] [PDF] [Bockmeyer et al. - Design and Methods and Refs.]  

The findings of this study suggest that systemic inflammation results in ADAMTS13 deficiency, and thereby activates hemostasis.

Letters to the Editor:

Red Cell Disorders:

Giuseppina Lacerra, Clelia Scarano, Gennaro Musollino, Angela Flagiello, Piero Pucci, Clementina Carestia

Hb Foggia or 117(GH5)Phe Ser : a new 2 globin allele affecting the Hb-AHSP interaction
Haematologica 2008 93: 141-142. [Abstract] [Full Text] [PDF]  

Chigozie J. Uneke, Festus E. Iyare, Patrick Oke, Dochka D. Duhlinska

Assessment of malaria in pregnancy using rapid diagnostic tests and its association with HIV infection and hematologic parameters in South-Eastern Nigeria
Haematologica 2008 93: 143-144. [Abstract] [Full Text] [PDF]  

Malignant Lymphomas:

Hongtao Ye, Ellen D. Remstein, Chris M. Bacon, Andrew G. Nicholson, Ahmet Dogan, Ming-Qing Du

Chromosomal translocations involving BCL6 in MALT lymphoma
Haematologica 2008 93: 145-146. [Abstract] [Full Text] [PDF]  

Gerald Illerhaus, Fabian Müller, Friedrich Feuerhake, Arnd-Oliver Schäfer, Christoph Ostertag, Jürgen Finke

High-dose chemotherapy and autologous stem-cell transplantation without consolidating radiotherapy as first-line treatment for primary lymphoma of the central nervous system
Haematologica 2008 93: 147-148. [Abstract] [Full Text] [PDF]  

José-Tomás Navarro, Ferran Vall-Llovera, José-Luis Mate, Mireia Morgades, Evarist Feliu, Josep-Maria Ribera

Decrease in the frequency of meningeal involvement in AIDS-related systemic lymphoma in patients receiving HAART
Haematologica 2008 93: 149-150. [Abstract] [Full Text] [PDF]  

Chronic Lymphocytic Leukemia:

Sajjan Mittal, Morgan G. Blaylock, Dominic J. Culligan, Robert N. Barker, Mark A. Vickers

A high rate of CLL phenotype lymphocytes in autoimmune hemolytic anemia and immune thrombocytopenic purpura
Haematologica 2008 93: 151-152. [Abstract] [Full Text] [PDF]  

Monoclonal Gammopathies:

Federico Sackmann, Miguel Arturo Pavlovsky, Claudia Corrado, Marco Pizzolato, Mariel Alejandre, Santiago Pavlovsky

Prognostic factors in monoclonal gammopathy of undetermined significance
Haematologica 2008 93: 153-154. [Abstract] [Full Text] [PDF]  

Platelet Disorders:

Keita Kirito, Kumi Sakoe, Daisuke Shinoda, Yoshihisa Takiyama, Kenneth Kaushansky, Norio Komatsu

A novel RUNX1 mutation in familial platelet disorder with propensity to develop myeloid malignancies
Haematologica 2008 93: 155-156. [Abstract] [Full Text] [PDF]  

Infectious Disorders:

Roy F. Chemaly, Harrys A. Torres, Ray Hachem, Dimitrios P Kontoyiannis, Amar Safdar, Issam I. Raad

Human herpesvirus-6 DNAemia in immunosuppressed adult patients with leukemia at risk for mold infection
Haematologica 2008 93: 157-158. [Abstract] [Full Text] [PDF] [Chemaly et al. - Additional Tables]  

Corrado Girmenia, Giampaolo Pizzarelli, Emanuela Pozzi, Giuseppe Cimino, Giuseppe Gentile, Pietro Martino

Improving outcomes of acute invasive Aspergillus rhinosinusitis n patients with hematologici malignancies or aplastic anemia: the role of voriconazole
Haematologica 2008 93: 159-160. [Abstract] [Full Text] [PDF]  

Online Only Articles:

A.S. Haddad, V. Subbiah, A.E. Lichtin, K.S. Theil, J.P. Maciejewski

Hypocupremia and bone marrow failure
Haematologica 2008 93: e1-e5. [Abstract] [Full Text] [PDF]  

A. Furlan, F. Pietrogrande, F. Marino, C. Menin, G. Polato, F. Vianello

Sequential development of large B cell lymphoma in a patient with peripheral T-cell lymphoma
Haematologica 2008 93: e6-e8. [Abstract] [Full Text] [PDF]  

N. Masera, C. Rescaldani, M. Azzolini, C. Vimercati, L. Tavecchia, G. Masera, V. De Molfetta, P. Arpa

Development of lens opacities with peculiar characteristics in patients affected by thalassemia major on chelating treatment with deferasirox (ICL670) at the Pediatric Clinic in Monza, Italy
Haematologica 2008 93: e9-e10. [Full Text] [PDF]  

N. Meuleman, G. Vanhaelen, T. Tondreau, P. Lewalle, J. Kwan, J. Bennani, P. Martiat, L. Lagneaux, D. 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: e11-e13. [Abstract] [Full Text] [PDF]  

C.H. ten Hove, J.M. Zijlstra-Baalbergen, E.F.I. Comans, R.M. van Elburg

An unusual hotspot in a young woman with Hodgkin’s lymphoma
Haematologica 2008 93: e14-e15. [Abstract] [Full Text] [PDF]  

A. Kalff, J. Shortt, J. Farr, R. McLennan, A. Lui, J. Scott, A. Spencer

Laboratory tumor lysis syndrome complicating LBH589 therapy in a patient with acute myeloid leukaemia
Haematologica 2008 93: e16-e17. [Abstract] [Full Text] [PDF]  

R. Bergner, M. Hoffmann, M. Uppenkamp

Reply. Kastritis E. et al. Reversibility of renal failure in newly diagnosed multiple myeloma patients treated with high dose dexamethasone containing regimes and the impact of novel agents. Haematologica 2007; 92:546–9
Haematologica 2008 93: e18-e19. [Full Text] [PDF]  

J.D. Fernández Águila, M. Cabrera Zamora, O. Álvarez Fernández, L. Prieto Jiménez, O. Mediaceja Vicente, I. Villares Álvarez

Comprehensive care for patients with sickle cell disease in Cuba
Haematologica 2008 93: e20. [Full Text] [PDF]  

D. Niino, K. Tsukasaki, K. Torii, D. Imanishi, T. Tsuchiya, Y. Onimaru, H. Tsushima, S. Yoshida, Y. Yamada, S. Kamihira, M. Tomonaga

Human Herpes virus 8-negative primary effusion lymphoma with BCL6 rearrangement in a patient with idiopathic CD4 positive T-lymphocytopenia
Haematologica 2008 93: e21-e23. [Abstract] [Full Text] [PDF]  

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