Peering through zebrafish to understand inherited bone marrow failure syndromes.

abstract：:Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length were identified as predictive biomarkers of immunosuppressive th...

journal_title：Haematologica

authors： Narita A,Muramatsu H,Sekiya Y,Okuno Y,Sakaguchi H,Nishio N,Yoshida N,Wang X,Xu Y,Kawashima N,Doisaki S,Hama A,Takahashi Y,Kudo K,Moritake H,Kobayashi M,Kobayashi R,Ito E,Yabe H,Ohga S,Ohara A,Kojima S,Japan Ch

更新日期：2015-12-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:Oxidant damage is an important contributor to the premature destruction of erythrocytes and anemia in thalassemias. To assess the extent of oxidant damage of circulating erythrocytes and the effects of antioxidant therapy on erythrocyte characteristics and anemia, we used a mouse model of huma...

journal_title：Haematologica

authors： de Franceschi L,Turrini F,Honczarenko M,Ayi K,Rivera A,Fleming MD,Law T,Mannu F,Kuypers FA,Bast A,van der Vijgh WJ,Brugnara C

更新日期：2004-11-01 00:00:00

abstract：:A prospective longitudinal study was conducted to determine whether single-donor fresh frozen plasma (FFP) substitution was able to influence L-asparaginase-associated hypoproteinemia. Within a 36-month period, 20 of 42 children with ALL received a total of 42 prophylactic FFP doses at a median of 10 (5-20) mliter/kg ...

journal_title：Haematologica

authors： Nowak-Göttl U,Rath B,Binder M,Hassel JU,Wolff J,Husemann S,Ritter J

更新日期：1995-09-01 00:00:00

abstract：BACKGROUND:Paroxysmal nocturnal hemoglobinuria (PNH) is associated with an increased risk of thrombosis through unknown mechanisms. DESIGN AND METHODS:We studied 23 patients with PNH, before and after five and 11 weeks of treatment with eculizumab. We examined markers of thrombin generation and reactional fibrinolysis...

journal_title：Haematologica

authors： Helley D,de Latour RP,Porcher R,Rodrigues CA,Galy-Fauroux I,Matheron J,Duval A,Schved JF,Fischer AM,Socié G,French Society of Hematology.

更新日期：2010-04-01 00:00:00

abstract：:We evaluate the long-term results of a prospective clinical study enrolling more than 100 adult patients with Burkitt lymphoma/leukemia. Depending on extent of disease, treatment consisted of six to eight rituximab infusions and four to six courses of intensive chemotherapy (attenuated in patients aged >55 years) with...

journal_title：Haematologica

authors： Intermesoli T,Rambaldi A,Rossi G,Delaini F,Romani C,Pogliani EM,Pagani C,Angelucci E,Terruzzi E,Levis A,Cassibba V,Mattei D,Gianfaldoni G,Scattolin AM,Di Bona E,Oldani E,Parolini M,Gökbuget N,Bassan R

更新日期：2013-11-01 00:00:00

abstract：:In ribosomopathies, the Diamond-Blackfan anemia (DBA) or 5q- syndrome, ribosomal protein (RP) genes are affected by mutation or deletion, resulting in bone marrow erythroid hypoplasia. Unbalanced production of ribosomal subunits leading to a limited ribosome cellular content, regulates translation at the expense of th...

journal_title：Haematologica

authors： Boussaid I,Le Goff S,Floquet C,Gautier EF,Raimbault A,Viailly PJ,Al Dulaimi D,Burroni B,Dusanter-Fourt I,Hatin I,Mayeux P,Cosson B,Fontenay M

更新日期：2020-04-23 00:00:00

abstract：BACKGROUND AND OBJECTIVES:Identification of chromosomal rearrangements is important for a precise risk-stratified diagnosis of hematologic malignancies. As the number of known translocations, specific for different types of leukemia increases, it takes ever more time and increasing amounts of patient's material to scre...

journal_title：Haematologica

authors： Nasedkina T,Domer P,Zharinov V,Hoberg J,Lysov Y,Mirzabekov A

更新日期：2002-04-01 00:00:00

abstract：:The introduction and the extended clinical use of nilotinib in the first-line treatment of chronic myeloid leukemia have been based on company-sponsored trials. Independent confirmations are extremely important. We report an investigator-sponsored study of nilotinib 300 mg twice daily in 130 chronic myeloid leukemia p...

journal_title：Haematologica

authors： Castagnetti F,Breccia M,Gugliotta G,Martino B,D'Adda M,Stagno F,Carella AM,Avanzini P,Tiribelli M,Trabacchi E,Visani G,Gobbi M,Salvucci M,Levato L,Binotto G,Capalbo SF,Bochicchio MT,Soverini S,Cavo M,Martinelli G,

更新日期：2016-10-01 00:00:00

abstract：:The total number of children with incident cancer in Italy has never been specifically estimated. Specialized population-based Childhood Cancer Registries have only been operating in Piedmont (CCRP) and in the Marche region, while general population cancer registries cover about 20% of the Italian population. The numb...

journal_title：Haematologica

authors： Baussano I,Maule MM,Dama E,Dalmasso P,Mosso ML,Galzerano M,Merletti F,Magnani C,Pastore G

更新日期：2007-09-01 00:00:00

abstract：:The use of minimal residual disease (MRD) measurement as a surrogate marker of molecular response to treatment can potentially improve the evaluation of treatment response and enable estimates of the residual leukemic cell burden during clinical remission, thereby improving the selection of therapeutic strategies and,...

journal_title：Haematologica

authors： Cazzaniga G,Biondi A

更新日期：2005-03-01 00:00:00

abstract：BACKGROUND:In normal platelets, insulin inhibits agonist-induced Ca(2+) mobilization by raising cyclic AMP. Platelet from patients with type 2 diabetes are resistant to insulin and show increased Ca(2+) mobilization, aggregation and procoagulant activity. We searched for the cause of this insulin resistance. DESIGN AN...

journal_title：Haematologica

authors： Gerrits AJ,Gitz E,Koekman CA,Visseren FL,van Haeften TW,Akkerman JW

更新日期：2012-08-01 00:00:00

abstract：:In patients with low and intermediate risk myelodysplastic syndrome and deletion 5q (del(5q)) treated with lenalidomide, monitoring of cytogenetic response is mandatory, since patients without cytogenetic response have a significantly increased risk of progression. Therefore, we have reviewed cytogenetic data of 302 p...

journal_title：Haematologica

authors： Göhring G,Giagounidis A,Büsche G,Hofmann W,Kreipe HH,Fenaux P,Hellström-Lindberg E,Schlegelberger B

更新日期：2011-02-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:CD20 has been proposed as a novel suicide gene system for the treatment of graft-versus-host disease (GVHD), a fatal complication of allogeneic bone marrow transplantation: indeed expression of the human non-immunogenic exogenous CD20 protein allows positive immunoselection of transduced cells...

journal_title：Haematologica

authors： Serafini M,Bonamino M,Golay J,Introna M

更新日期：2004-01-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:Subcutaneous Ara-C plus interferon (IFN) produces more cytogenetic responses than IFN in chronic myeloid leukemia (CML) but a greater toxicity. The objective of this study was to determine the efficacy and tolerance of IFN plus oral Ara-C ocfosfate (YNK-01) in IFN-resistant CML patients. DESI...

journal_title：Haematologica

authors： Cervantes F,Sureda A,Hernández-Boluda JC,Martino R,Brunet S,Borrego D,Antich JL,Montserrat E

更新日期：2001-12-01 00:00:00

abstract：BACKGROUND:Transplantation from an HLA-matched sibling is the treatment of choice for young patients with acquired severe aplastic anemia. For older patients, the acceptable upper age limit for transplantation as first-line treatment varies. The current analysis, therefore, sought to identify age or ages at transplanta...

journal_title：Haematologica

authors： Gupta V,Eapen M,Brazauskas R,Carreras J,Aljurf M,Gale RP,Hale GA,Ilhan O,Passweg JR,Ringdén O,Sabloff M,Schrezenmeier H,Socié G,Marsh JC

更新日期：2010-12-01 00:00:00

abstract：:Effects of angiotensin (Ang)-(1-7), an AngII metabolite, on bone marrow-derived hematopoietic cells were studied. We identified Ang-(1-7) to stimulate proliferation of human CD34(+) and mononuclear cells in vitro. Under in vivo conditions, we monitored proliferation and differentiation of human cord blood mononuclear ...

journal_title：Haematologica

authors： Heringer-Walther S,Eckert K,Schumacher SM,Uharek L,Wulf-Goldenberg A,Gembardt F,Fichtner I,Schultheiss HP,Rodgers K,Walther T

更新日期：2009-06-01 00:00:00

abstract：:Transient pancytopenia preceding childhood acute lymphoblastic leukemia (ALL) is an unfrequent but well-known event. The association of this preleukemic syndrome with hepatitis is extremely rare, with only two such cases having been published in the literature. We report the case of a 16-year-old boy who was diagnosed...

journal_title：Haematologica

authors： Rafel M,Cobo F,Cervantes F,Bosch F,Campo E,Montserrat E

更新日期：1998-06-01 00:00:00

abstract：BACKGROUND:Mutation C1149R in the von Willebrand factor (VWF) gene has been thought to cause autosomal dominant severe type 1 von Willebrand disease (VWD). DESIGN AND METHODS:Eight patients from three unrelated families with this mutation were included in the present study who had distinct VWF abnormalities, not descr...

journal_title：Haematologica

authors： Pérez-Rodríguez A,García-Rivero A,Lourés E,López-Fernández MF,Rodríguez-Trillo A,Batlle J

更新日期：2009-05-01 00:00:00

abstract：:A pilot study, testing two different chemotherapeutic schedules in CML patients, was carried out in order to verify the possibility of Ph1 clone disappearance. Both therapeutic regimens, though able to induce severe marrow aplasia, determined only a reduction of Ph1 positive metaphases in two patients, while in one ca...

journal_title：Haematologica

authors： Centurioni R,Leoni P,Russo D,Brianzoni MF,Montillo M,Baccarani M,Tura S

更新日期：1989-09-01 00:00:00

abstract：:Recent studies indicate that a subset of patients with apparently acquired aplastic anemia (AA) have mutations in genes for telomerase ribonucleoprotein complex components. We looked for mutations in telomerase RNA (TERC) and telomerase reverse transcriptase (TERT) in 96 Japanese children with acquired AA and in 76 he...

journal_title：Haematologica

authors： Liang J,Yagasaki H,Kamachi Y,Hama A,Matsumoto K,Kato K,Kudo K,Kojima S

更新日期：2006-05-01 00:00:00

abstract：:Myeloproliferative neoplasms are uncommon disorders in children, for which we have limited understanding of the pathogenesis and optimal management. JAK2 and MPL mutations, while common drivers of myeloproliferative neoplasms in adult patients, are not clearly linked to pediatric disease. Management and clinical outco...

journal_title：Haematologica

authors： Kucine N,Chastain KM,Mahler MB,Bussel JB

更新日期：2014-04-01 00:00:00

abstract：:This study reports a case of granulocytic sarcoma that developed in the epidural zone 25 days before clinical evidence of an acute promyelocytic leukemia. The case presented the diagnostic difficulties that are common to all aleukemic granulocytic sarcomas. Moreover, it highlights the very rare association between gra...

journal_title：Haematologica

authors： Tosi A,De Paoli A,Fava S,Luoni M,Sironi M,Tocci A,Assi A,Cassi E

更新日期：1995-01-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:Serum beta(2)-microglobulin (s beta(2)m) is an established prognostic factor for multiple myeloma and non-Hodgkin's lymphoma, but only limited data suggest an adverse prognostic significance for Hodgkin's lymphoma (HL). This study was undertaken to examine the impact of s beta(2)m on the progn...

journal_title：Haematologica

authors： Vassilakopoulos TP,Nadali G,Angelopoulou MK,Siakantaris MP,Dimopoulou MN,Kontopidou FN,Karkantaris C,Kokoris SI,Kyrtsonis MC,Tsaftaridis P,Pizzolo G,Pangalis GA

更新日期：2002-07-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:Dendritic cells (DC) pulsed with multiple myeloma (MM) patient-specific idiotype (Id) protein can induce MM-specific T-cell responses. DESIGN AND METHODS:We established serum-free culture conditions to generate monocyte-derived DC for clinical use to circumvent anti-xenogenic immune responses...

journal_title：Haematologica

authors： Reichardt VL,Milazzo C,Brugger W,Einsele H,Kanz L,Brossart P

更新日期：2003-10-01 00:00:00

abstract：BACKGROUND AND OBJECTIVE:Thrombopoietin has been established as the major regulator of megakaryocyte and platelet production. In this study we evaluated the effects of PEG-recombinant human megakaryocyte growth and development factor (PEG-rHuMGDF), a pegylated and truncated form of thrombopoietin, on the growth and dif...

journal_title：Haematologica

authors： Catani L,Gugliotta L,Motta M,Tazzari P,Baravelli S,Tura S

更新日期：1998-05-01 00:00:00

abstract：:Twice-weekly carfilzomib is approved at 27 and 56 mg/m2 to treat relapsed multiple myeloma patients. In the phase III study ARROW, once-weekly 70 mg/m 2 carfilzomib prolonged the median progression-free survival of relapsed multiple myeloma patients in comparison with twice-weekly 27 mg/m2 carfilzomib, without adding ...

journal_title：Haematologica

authors： Bringhen S,Mina R,Petrucci MT,Gaidano G,Ballanti S,Musto P,Offidani M,Spada S,Benevolo G,Ponticelli E,Galieni P,Cavo M,Di Toritto TC,Di Raimondo F,Montefusco V,Palumbo A,Boccadoro M,Larocca A

更新日期：2019-08-01 00:00:00

abstract：:The lupus anticoagulant (LAC) and anticardiolipin antibody (ACA) syndromes require particular therapeutic approaches: thrombotic accidents are an indication for oral anticoagulant therapy (OAT), whereas severe thrombocytopenia may require the special treatments used for immunologic thrombocytopenic purpura (ITP). We d...

journal_title：Haematologica

authors： Ballerini G,Gemmati D,Moratelli S,Morelli P,Serino ML

更新日期：1995-05-01 00:00:00

abstract：:High levels of HbF were found in patients with myelodysplastic syndrome (MDS), as well as a possible switching of the ratio of the gamma chains from the adult to the newborn type in 25% of our patients. These abnormalities in general were not present in the parents. The possibility of having thalassemia or other hemog...

journal_title：Haematologica

authors： Bourantas KL,Georgiou I,Seferiadis K

更新日期：1991-07-01 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：:As the defective genes for more and more genetic disorders become unravelled, it is clear that patients with apparently identical genotypes can have many different clinical conditions even in simple monogenic disorders. Beta thalassemia occurs when there is a deficiency in the synthesis of beta globin chains. The clin...

journal_title：Haematologica

authors： Thein SL

更新日期：2005-05-01 00:00:00