abstract：OBJECTIVE:To investigate a family with factor VII (FVII) deficiency from Argentina. PATIENTS AND METHODS:The proposita is a 14-year-old girl who presented with a mild to moderate bleeding tendency. Menorrhagia is controlled with periodical administration of small doses of recombinant FVII concentrate. The mother of th...

journal_title：Acta haematologica

authors： Girolami A,Paoletti M,Ferrari S,Garcia D

更新日期：2021-01-01 00:00:00

abstract：:Macrothrombocytopenia (MTP) is a group of rare disorders characterized by giant platelets, thrombocytopenia, and variable association with abnormal bleeding. Inherited MTP are frequently misdiagnosed as immune thrombocytopenia. Associated second-organ manifestation can help narrow down syndromic MTPs. We describe a ca...

journal_title：Acta haematologica

authors： Karki NR,Ajebo G,Savage N,Kutlar A

更新日期：2021-01-01 00:00:00

abstract：:Treatment options are limited for patients with advanced forms of myeloproliferative neoplasms (MPN) including blast-phase disease (MPN-BP). Decitabine has frequently been deployed but its efficacy and safety profile are not well described in this population. We retrospectively reviewed 42 patients treated with decita...

journal_title：Acta haematologica

authors： Zhou S,Tremblay D,Hoffman R,Kremyanskaya M,Najfeld V,Li L,Moshier E,Mascarenhas J

更新日期：2021-01-01 00:00:00

abstract：:Chronic pain affects 50% of adults with sickle cell disease (SCD). Although inflammation is thought to contribute to the pathogenesis of chronic pain, no studies have examined the differences in circulating cytokines between patients with SCD with and without chronic pain. We performed an observational cohort study us...

journal_title：Acta haematologica

authors： Karafin MS,Simpson P,Field JJ

更新日期：2021-01-01 00:00:00

abstract：:Recently, the use of novel targeted drugs has changed the treatment paradigms in chronic lymphocytic leukemia (CLL). Among the several drugs used for the management of relapsed/refractory (R/R) CLL, Bruton tyrosine kinase inhibitors (ibrutinib and acalabrutinib), phosphatidylinositol 3-kinase inhibitors (idelalisib an...

journal_title：Acta haematologica

authors： Smolewski P,Robak T

更新日期：2020-11-25 00:00:00

abstract：:Low-risk patients with myelodysplastic syndromes (MDS) are inclined to long-term accumulation of iron in the organs due mostly to red blood cell transfusion and ineffective erythropoiesis. The effect of free toxic iron species in the liver and heart sites is well known, but recent knowledge assumes that oxidant-mediat...

journal_title：Acta haematologica

authors： Pilo F,Caocci G,Mele G,La Nasa G

更新日期：2020-10-02 00:00:00

abstract：OBJECTIVES:Reticulated platelets circulating in the blood reflect megakaryopoietic activity and platelet turnover and can be automatically and low-invasively measured as the immature platelet fraction (IPF) using a Sysmex XN hematocytometer. The present study retrospectively investigated whether or not the IPF can pred...

journal_title：Acta haematologica

authors： Takami A,Mizuno S,Nakamura A,Kanasugi J,Yamamoto H,Vu Quang L,Nakagami Y,Nakano Y,Yamada S,Matsumura S,Takasugi S,Uchino K,Horio T,Murakami S,Oohigashi Y,Nakayama T,Tani H,Enomoto M,Hanamura I

更新日期：2020-09-17 00:00:00

abstract：:Hypercalcemia is a significant feature of patients with active multiple myeloma (MM) with extensive bone disease. Among the causes of non-neoplastic hypercalcemia, primary hyperparathyroidism (PHPT) is one of the most common, leading to osteoporosis and bone fractures. Interestingly, some preclinical data indicate tha...

journal_title：Acta haematologica

authors： Notarfranchi L,Marchica V,Dalla Palma B,Pelagatti L,Burroughs-Garcia J,Pedrazzoni M,Ruffini L,Cetani F,Marcocci C,Giuliani N

更新日期：2020-09-09 00:00:00

abstract：:Acute myeloid leukemia (AML) is a stem cell malignancy that originates in the bone marrow and involves the peripheral blood. Extramedullary AML is rarer, but it is most commonly associated with the former French-American-British (FAB) subtypes M4 or M5 of AML. AML cells may also home to the central nervous system and ...

journal_title：Acta haematologica

authors： Patel SA,Bledsoe JR,Gordon J,Cerny J

更新日期：2020-08-19 00:00:00

abstract：:Eltrombopag is a thrombopoietin receptor agonist frequently used to manage immune thrombocytopenia and aplastic anemia. At the high doses used for aplastic anemia, but not the doses used for immune thrombocytopenia, eltrombopag can cause reddish-brown discoloration of plasma, which can interfere with bilirubin measure...

journal_title：Acta haematologica

authors： Al-Samkari H,Goodarzi K

更新日期：2020-07-22 00:00:00

abstract：:Treating patients with hemophilia and inhibitors is often problematic. The presence of inhibitors negatively impacts the effectiveness of treatment to achieve hemostasis especially in patients with hemophilia B, owing mainly to allergic reactions to factor IX (FIX) concentrates and the low success rate of immune toler...

journal_title：Acta haematologica

authors： Uchida E,Komori K,Kurata T,Taki M,Sakashita K

更新日期：2020-07-22 00:00:00

abstract：:Patients with HIV-associated lymphocyte-depleted Hodgkin lymphoma (HIV-HL) often present with advanced, extranodal disease and aggressive clinical features, limiting definitive therapeutic intervention. Here we report two patients with HIV-HL who presented with multi-organ dysfunction as an initial manifestation of th...

journal_title：Acta haematologica

authors： Ding J,Kumar AJ,Wilding E,Lee ST,Law JY

更新日期：2020-01-01 00:00:00

abstract：BACKGROUND:Danaparoid sodium and synthetic protease inhibitors (SPIs) have been approved for the treatment of disseminated intravascular coagulation (DIC) in Japan. OBJECTIVES:To compare the clinical results of the treatment of DIC with danaparoid or SPIs. METHODS:We retrospectively examined 188 patients with hematol...

journal_title：Acta haematologica

authors： Minakata D,Fujiwara SI,Hayakawa J,Nakasone H,Ikeda T,Kawaguchi SI,Toda Y,Ito S,Ochi SI,Nagayama T,Mashima K,Umino K,Nakano H,Yamasaki R,Morita K,Kawasaki Y,Sugimoto M,Ishihara Y,Yamamoto C,Ashizawa M,Hatano K,Sa

更新日期：2020-01-01 00:00:00

abstract：:Immunoglobulin light chain amyloidosis (AL amyloidosis) is a rare, life-threatening disease characterized by the deposition of misfolded proteins in vital organs such as the heart, the lungs, the kidneys, the peripheral nervous system, and the gastrointestinal tract. This causes a direct toxic effect, eventually leadi...

journal_title：Acta haematologica

authors： Van Doren L,Lentzsch S

更新日期：2020-01-01 00:00:00

abstract：:Treatment of chronic myeloid leukemia (CML) and Philadelphia chromosome-positive acute leukemia (Ph+ ALL) has been revolutionized with the advent of tyrosine kinase inhibitors (TKIs). Most patients with CML achieve long-term survival similar to individuals without CML due to treatment with TKIs not only in frontline b...

journal_title：Acta haematologica

authors： Saussele S,Haverkamp W,Lang F,Koschmieder S,Kiani A,Jentsch-Ullrich K,Stegelmann F,Pfeifer H,La Rosée P,Goekbuget N,Rieger C,Waller CF,Franke GN,le Coutre P,Kirchmair R,Junghanss C

更新日期：2020-01-01 00:00:00

abstract：:Thymoma is an uncommon neoplasia derived from the epithelial cells of the thymus, which leads to immune dysregulation and is associated with a series of autoimmune diseases. However, the concurrence of these disease entities is rare, and the exact mechanisms of these diseases are still unclear. We have admitted severa...

journal_title：Acta haematologica

authors： Wang W,Chen LY,Zhao W,Ren Y,Wang L,Li X,Jiang Y,Gao H

更新日期：2020-01-01 00:00:00

abstract：:Iron deficiency is the main cause of anemia in both sexes, with women being more commonly affected. Iron therapy is currently considered an effective and safe remedy to replenish the iron storages. Iron can be administrated both orally and intravenously. In particular, intravenous (IV) iron therapy is widely used when...

journal_title：Acta haematologica

authors： Di Girolamo A,Albanesi M,Loconte F,Di Bona D,Caiaffa MF,Macchia L

更新日期：2020-01-01 00:00:00

abstract：:To characterize intracellular signaling in peripheral blood (PB) cells of acute myeloid leukemia (AML) patients undergoing pretransplant conditioning with CXCR4 inhibitor plerixafor, granulocyte colony-stimulating factor (G-CSF), and busulfan plus fludarabine (Bu+Flu) chemotherapy, we profiled 153 proteins in 33 funct...

journal_title：Acta haematologica

authors： Zeng Z,Liu W,Benton CB,Konoplev S,Lu H,Wang RY,Chen J,Shpall E,Baggerly KA,Champlin R,Konopleva M

更新日期：2019-01-01 00:00:00

abstract：:Neurologic complications of allogeneic hematopoietic cell transplantation (allo-HCT) include infections, cerebrovascular events, therapy-induced neurotoxicity, recurrent malignancies, and neurologic manifestations of graft-versus-host disease (GVHD). Anti-glutamic acid decarboxylase (GAD) antibody-associated cerebella...

journal_title：Acta haematologica

authors： Shargian-Alon L,Raanani P,Rozovski U,Siegal T,Yust-Katz S,Yeshurun M

更新日期：2019-01-01 00:00:00

abstract：BACKGROUND:The incidence of venous thromboembolism (VTE) in haematological malignancies varies according to the type and grade of the disease and clinical variables, and there is a need to develop a tool to predict the occurrence of VTE in cancer patients at diagnosis to tailor prophylactic anticoagulation use during t...

journal_title：Acta haematologica

authors： Elmoamly S,Mattar M,Yacoub MF,Afif A

更新日期：2019-01-01 00:00:00

abstract：BACKGROUND/AIMS:The aim of this work was to investigate the efficacy and predictive factors of CLAG treatment in refractory or relapsed (R/R) acute myeloid leukemia (AML) patients. METHODS:Sixty-seven R/R AML patients were enrolled in this prospective cohort study and treated by a CLAG regimen: 5 mg/m2/day cladribine ...

journal_title：Acta haematologica

authors： Wang L,Xu J,Tian X,Lv T,Yuan G

更新日期：2019-01-01 00:00:00

abstract：BACKGROUND:The treatment of relapsed/refractory (R/R) peripheral T cell lymphoma (PTCL) is limited to a few agents. Romidepsin, a histone deacetylase inhibitor, was approved for PTCL treatment as a single agent in the R/R setting, yet with partial efficacy. Several attempts to combine romidepsin with other chemotherapy...

journal_title：Acta haematologica

authors： Nachmias B,Shaulov A,Lavie D,Goldschmidt N,Gural A,Saban R,Lebel E,Gatt ME

更新日期：2019-01-01 00:00:00

abstract：BACKGROUND:Argininemia is an autosomal recessive urea cycle disorder (UCD). Unlike other UCD, hyperammonemia is rarely seen. Patients usually present in childhood with neurological symptoms. Uncommon presentations like neonatal cholestasis or cirrhosis have been reported. Although transient elevations of liver transami...

journal_title：Acta haematologica

authors： Kiykim E,Zubarioglu T,Cansever MS,Celkan T,Häberle J,Aktuglu Zeybek AC

更新日期：2018-01-01 00:00:00

abstract：:The 8p11 myeloproliferative syndrome (EMS) is an aggressive neoplasm associated with chromosomal translocations involving the fibroblast growth factor receptor 1 (FGFR1) tyrosine kinase gene on chromosome 8p11-12. A new case of a 9-year-old boy with leukocytosis, eosinophilia, and general lymphadenopathy is reported i...

journal_title：Acta haematologica

authors： Lv H,Hu S,Lu J,Zhai Q,Zhai Z,Du Z,Sun Y,Li J,He H,Wang Y

更新日期：2018-01-01 00:00:00

abstract：:With the widespread use of genetic diagnostic technologies, many novel mutations have been identified in hereditary spherocytosis (HS)-related genes, including SPTA1, SPTB, ANK1, SLC4A1, and EPB42. However, mutations in HS-related genes are dispersed and nonspecific in the diagnosis of some HS patients, indicating sig...

journal_title：Acta haematologica

authors： He BJ,Liao L,Deng ZF,Tao YF,Xu YC,Lin FQ

更新日期：2018-01-01 00:00:00

abstract：BACKGROUND:Thalassemia is a group of hereditary hemoglobinopathies caused by decreased or absent synthesis of α and/or β globin chains. Studies have shown that hypercoagulability and thrombosis are common clinical symptoms in β-thalassemia, especially β-thalassemia intermedia, but little is known about in α-thalassemia...

journal_title：Acta haematologica

authors： Chansai S,Fucharoen S,Fucharoen G,Jetsrisuparb A,Chumpia W

更新日期：2018-01-01 00:00:00

abstract：OBJECTIVE:Adherence to tyrosine kinase inhibitor treatment is a significant factor in the achievement of a good clinical response in chronic myeloid leukemia (CML). The aim of this retrospective study is to investigate 1- and 2-year medication adherence to imatinib treatment, linking adherence rates with the clinical o...

journal_title：Acta haematologica

authors： Santoleri F,Lasala R,Ranucci E,La Barba G,Di Lorenzo R,Vetrò A,Di Bartolomeo P,Costantini A

更新日期：2016-01-01 00:00:00

abstract：:Non-transfusion-dependent thalassemia (NTDT) is associated with various forms of thalassemia and genetic modifiers. We report the molecular basis of NTDT in hemoglobin (Hb) E-β-thalassemia disease. This study was done in 73 adult patients encountered at the prenatal diagnosis center of Khon Kaen University, Northeast ...

journal_title：Acta haematologica

authors： Yamsri S,Pakdee N,Fucharoen G,Sanchaisuriya K,Fucharoen S

更新日期：2016-01-01 00:00:00

abstract：:The value of clearance of peripheral blood blasts (PBB) as a predictor of outcomes in acute myeloid leukemia (AML) is controversial. To investigate the prognostic significance of the time to clearance of PBB after induction in Chinese patients with AML, a retrospective analysis of 146 patients with newly diagnosed AML...

journal_title：Acta haematologica

authors： Li X,Zhu H,Zhang Y,Zhao W,Mi J,Hu J,Li J

更新日期：2016-01-01 00:00:00

abstract：BACKGROUND:Posterior reversible encephalopathy syndrome (PRES) is a rare but serious complication after allogeneic hematopoietic stem cell transplantation (alloHSCT). Among others, calcineurin inhibitors (CNI) for prophylaxis of graft-versus-host disease (GvHD) may promote the development of PRES, but the pathomechanis...

journal_title：Acta haematologica

authors： Schmidt V,Prell T,Treschl A,Klink A,Hochhaus A,Sayer HG

更新日期：2016-01-01 00:00:00

abstract：:During a screening for hemoglobinopathies, we found a carrier of the Sardinian δβ-thalassemia condition. The proband's hematology and hemoglobin (Hb) profile agreed with those of the other carriers previously identified during our diagnostic program except for the fetal Hb (HbF) composition, which consisted of both α2...

journal_title：Acta haematologica

authors： Trova S,Mereu P,Cocco E,Masala B,Manca L,Pirastru M

更新日期：2016-01-01 00:00:00

abstract：BACKGROUND:This study aimed to compare the curative effects of the combination therapy of all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO, As₂O₃) with ATRA monotherapy on newly diagnosed acute promyelocytic leukemia (APL). METHODS:The studies were retrieved from PubMed, EMBASE, Cochrane Library, ChinaInfo and...

journal_title：Acta haematologica

authors： Ma H,Yang J

更新日期：2015-01-01 00:00:00

abstract：:The management of severe late-onset hemorrhagic cystitis (LO-HC) after allogeneic hematopoietic stem cell transplantation (HSCT) is still challenging. Because mesenchymal stromal cells (MSCs) possess anti-inflammatory and tissue repair-promoting properties, we retrospectively analyzed the efficacy and safety of MSC in...

journal_title：Acta haematologica

authors： Wang Y,Chen F,Gu B,Chen G,Chang H,Wu D

更新日期：2015-01-01 00:00:00

abstract：BACKGROUND:Not all patients with diffuse large B-cell lymphoma (DLBCL) are candidates for aggressive regimens. (90)Y ibritumomab tiuxetan ((90)Y-IT), an anti-CD20 radionuclide-conjugated antibody, has demonstrated clinical efficacy in DLBCL with a favorable toxicity profile. METHODS:This phase II trial investigated th...

journal_title：Acta haematologica

authors： Arnason JE,Luptakova K,Rosenblatt J,Tzachanis D,Avigan D,Zwicker JI,Levine J,Kim M,Parker JA,Grant B,Joyce RM

更新日期：2015-01-01 00:00:00

abstract：BACKGROUND/AIMS:Autophagy is crucial for the survival and function of plasma cells including protection from toxic misfolded immunoglobulin and proper energy metabolism. Multiple myeloma (MM) is an indolent but eventually fatal neoplasm of plasma cells. Autophagy may play a critical role in the survival of MM cells and...

journal_title：Acta haematologica

authors： Jung G,Roh J,Lee H,Gil M,Yoon DH,Suh C,Jang S,Park CJ,Huh J,Park CS

更新日期：2015-01-01 00:00:00

abstract：:Janus kinases are critical components of signaling pathways that regulate hematopoiesis. Mutations of the non-receptor tyrosine kinase JAK2 are found in many BCR-ABL-negative myeloproliferative neoplasms. Preclinical results support that JAK2 inhibitors could show efficacy in treating chronic myeloproliferative neopla...

journal_title：Acta haematologica

authors： Ringel F,Kaeda J,Schwarz M,Oberender C,Grille P,Dörken B,Marque F,Manley PW,Radimerski T,le Coutre P

更新日期：2014-01-01 00:00:00

abstract：:Posttransplant lymphoproliferative disorders (PTLD) are a potentially life-threatening complication of immunosuppression in transplant recipients. The majority of cases are Epstein-Barr virus-associated lesions of B cell origin. T cell PTLD is rare, particularly in pediatric patients. We present an unusual case of mon...

journal_title：Acta haematologica

authors： Kraus TS,Twist CJ,Tan BT

更新日期：2014-01-01 00:00:00

abstract：:The increased longevity of patients with thalassemia and sickle cell disease (SCD) introduces new clinical challenges due to the accumulation of disease-related morbidity, psychosocial issues and health care adjustments. Patients with thalassemia major now live into adulthood without suffering heart failure but must c...

journal_title：Acta haematologica

authors： Yacobovich J,Tamary H

更新日期：2014-01-01 00:00:00

abstract：BACKGROUND:In 2003, oral fludarabine was introduced in the USA for the treatment of patients with hematologic malignancies as an alternative to its intravenous (i.v.) formulation; in 2008, it was introduced in México while the i.v. formulation was withdrawn. Accordingly, i.v. fludarabine had to be replaced by oral flud...

journal_title：Acta haematologica

authors： Velázquez-Sánchez-de-Cima S,Zamora-Ortiz G,Hernández-Reyes J,Rosales-Durón AD,González-Ramírez MP,Martagón-Herrera NA,Ruiz-Delgado GJ,Ruiz-Argüelles GJ

更新日期：2014-01-01 00:00:00

abstract：:Chronic graft-versus-host disease (GVHD) is a severe complication of allogeneic stem cell transplantation, with a substantial impact on the quality of life and survival, still lacking with regard to an optimal therapeutic strategy. Corticosteroids are considered the standard of care for first-line treatment of chronic...

journal_title：Acta haematologica

authors： Michael M,Shimoni A,Nagler A

更新日期：2013-01-01 00:00:00