The hope and reality of long-acting hemophilia products.

abstract：:Botrocetin, a protein isolated from the venom of the snake Bothrops jararaca, induces platelet aggregation/agglutination by von Willebrand factor (vWF) binding to the membrane glycoprotein (GP) Ib, an action resembling that of ristocetin. However, some differences in the interaction between vWF and platelet GPIb induc...

journal_title：American journal of hematology

authors： Nishio K,Fujimura Y,Niinomi K,Takahashi Y,Yoshioka A,Fukui H,Usami Y,Titani K,Ruggeri ZM,Zimmerman TS

更新日期：1990-04-01 00:00:00

abstract：:The hereditary persistence of fetal hemoglobin (HPFH)-6 is sporadically found in Thailand whereas the deletion-inversion type (G)gamma((A)gamma delta beta)(0)-thalassemia is described among Indians. We report a hitherto un-described case in which these two defects co-segregate. He was a 3-year-old Thai boy who had a f...

journal_title：American journal of hematology

authors： Fucharoen S,Pengjam Y,Surapot S,Fucharoen G,Sanchaisuriya K

更新日期：2002-10-01 00:00:00

abstract：:In thalassemia, fetal hemoglobin (HbF) augmentation with hydroxycarbamide (also known as hydroxyurea) is not always successful. The expected parallel effects on red cell (RBC) membrane deformability, cell hydration, and membrane phospholipid organization, all important for extending RBC life span and increasing Hb, ha...

journal_title：American journal of hematology

authors： Singer ST,Vichinsky EP,Larkin S,Olivieri N,Sweeters N,Kuypers FA,E\/beta Thalassemia Study Group.

更新日期：2008-11-01 00:00:00

abstract：:Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal stem-cell disorder in which blood cells lack complement inhibiting membrane proteins, and become susceptible to complement-mediated injury, leading to chronic intravascular hemolysis and pancytopenia. Glucocorticoids have been a mainstay of therapy. For patien...

journal_title：American journal of hematology

authors： Harrington WJ Sr,Kolodny L,Horstman LL,Jy W,Ahn YS

更新日期：1997-02-01 00:00:00

abstract：:We molecularly characterized sixty-seven cases of Hb H disease by the polymerase chain reaction. The strategy depends on amplifying the alpha-thalassemia-1 (alpha-thal-1) gene by primers flanking the breakpoint and sequence differences of the 3' end of the alpha-globin gene and the nonhomologous elements I, II, and II...

journal_title：American journal of hematology

authors： Liu TC,Chiou SS,Lin SF,Chen TP,Tseng WP,Chen PH,Chang JG

更新日期：1994-04-01 00:00:00

abstract：:The lupus anticoagulant may be accompanied by an acquired factor II deficiency and bleeding. We report on a patient with a lupus anticoagulant and factor II (Fll) deficiency responsive to Danazol. Acquired hypoprothrombinemia (FII) with the lupus anticoagulant (LA) may be accompanied by a hemorrhagic diathesis. A 64-y...

journal_title：American journal of hematology

authors： Williams S,Linardic C,Wilson O,Comp P,Gralnick HR

更新日期：1996-12-01 00:00:00

abstract：:A rare beta-thalassemia mutation at the splicing junction [namely, G-->C in intervening sequence (IVS) I-1] was found in a Japanese family. The proband and his mother were heterozygous for the mutation. Analysis of mRNA extracted from the reticulocyte-rich fraction obtained from the proband's mother revealed that the ...

journal_title：American journal of hematology

authors： Fujihara N,Tozuka M,Ueno I,Yamauchi K,Nakagoshi R,Ishikawa S,Hirota M,Okumura N,Ishii E,Katsuyama T

更新日期：2003-01-01 00:00:00

abstract：:Multiple myeloma (MM) and primary systemic light chain amyloidosis (AL) are both chronic plasma cell dyscrasias with different clinical expression but limited treatment options for relapsed refractory disease. We report the effect of the addition of clarithromycin on 31 MM and 17 AL with relapsed or refractory disease...

journal_title：American journal of hematology

authors： Shaulov A,Ganzel C,Benyamini N,Barshay Y,Goldschmidt N,Lavie D,Libster D,Gural A,Avni B,Gatt ME

更新日期：2017-02-01 00:00:00

abstract：:Ticlopidine-induced aplastic anemia (TIAA) is considered very uncommon. We present two new cases, and we review 55 additional cases from the literature. The first case concerns a 70-year-old man who developed severe aplastic anemia 7 weeks after treatment with 500 mg of ticlopidine daily. The patient sustained a sever...

journal_title：American journal of hematology

authors： Symeonidis A,Kouraklis-Symeonidis A,Seimeni U,Galani A,Giannakoulas N,Fragopanagou E,Tiniakou M,Matsouka P,Zoumbos N

更新日期：2002-09-01 00:00:00

abstract：:The pharmacokinetics of factor VIII were studied in a series of 20 hemophilia-A patients undergoing surgery. Regardless of the type of operation, elimination of factor VIII was shown to be increased only in ten cases (50%) during the post-operative period. In this subgroup of patients, factor VIII half-life, measured ...

journal_title：American journal of hematology

authors： Longo G,Messori A,Morfini M,Baudo F,Ciavarella N,Cinotti S,Filimberti E,Giustarini G,Molinari AC,Ferrini PR

更新日期：1989-03-01 00:00:00

abstract：:The acquired JAK2 V617F mutation is common in patients with myeloproliferative neoplasms. We previously showed that JAK2 V617F is also found in coronary patients, most of them affected by coronary atherosclerosis. Peripheral arterial disease (PAD) is another important manifestation of atherosclerosis. However, prevale...

journal_title：American journal of hematology

authors： Muendlein A,Kinz E,Gasser K,Leiherer A,Rein P,Saely CH,Grallert H,Peters A,Fraunberger P,Drexel H,Lang AH

更新日期：2015-01-01 00:00:00

abstract：:A 49-year-old Caucasian woman presented with features suggestive of thrombotic microangiopathy (TMA). She did not respond to treatment with repeated plasma exchange and corticosteroids. A bone marrow biopsy revealed presence of metastatic carcinoma. A limited autopsy revealed presence of breast cancer with rib metasta...

journal_title：American journal of hematology

authors： Werner TL,Agarwal N,Carney HM,Rodgers GM

更新日期：2007-04-01 00:00:00

abstract：:Four men were diagnosed with human immunodeficiency virus infection (AIDS) and autoimmune hemolytic anemia (HIV-AIHA) during the years 1997-2000 at Cook County Hospital, Chicago. All patients presented with the acute onset of severe hemolytic anemia, fever, and splenomegaly. The direct and indirect antiglobulin tests ...

journal_title：American journal of hematology

authors： Koduri PR,Singa P,Nikolinakos P

更新日期：2002-06-01 00:00:00

abstract：:The levels of adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), lactic dehydrogenase (LDH), and LDH isoenzyme patterns (LD1 to LD5) have been measured in lymphocyte extract from 28 patients with B-chronic lymphocytic leukemia (B-CLL). The activities of ADA, PNP, and LDH have been correlated with two mo...

journal_title：American journal of hematology

authors： Vives Corrons JL,Rozman C,Pujades MA,Colomer D,Perez Vila E,Anegon I,Gallart T,Vives Puiggrós J,Viñolas N,Montserrat E

更新日期：1988-03-01 00:00:00

abstract：:In HIV-positive patients with non-Hodgkin lymphoma (NHL), no benefit of adding rituximab to chemotherapy was seen in a randomized controlled trial (RCT). We performed a meta-analysis of prospective studies to ascertain outcomes in HIV-positive NHL patients treated with chemotherapy (chemo) versus rituximab and chemo (...

journal_title：American journal of hematology

authors： Castillo JJ,Echenique IA

更新日期：2012-03-01 00:00:00

abstract：:This analysis, using data from the bortezomib-melphalan-prednisone (VMP) arm of the Phase III VISTA study, investigated whether increased cumulative bortezomib dose could improve overall survival (OS) in transplant-ineligible patients with previously untreated multiple myeloma. Median cumulative bortezomib dose receiv...

journal_title：American journal of hematology

authors： Mateos MV,Richardson PG,Dimopoulos MA,Palumbo A,Anderson KC,Shi H,Elliott J,Dow E,van de Velde H,Niculescu L,San Miguel JF

更新日期：2015-04-01 00:00:00

abstract：:Production of abnormal hemoglobin (HbS) in sickle-cell disease (SCD) results in its polymerization in deoxygenated conditions and in sickled-RBC formation. Dense RBCs (DRBCs), defined as density >1.11 and characterized by increased rigidity are absent in normal AA subjects, but present at percentages that vary of a pa...

journal_title：American journal of hematology

authors： Di Liberto G,Kiger L,Marden MC,Boyer L,Poitrine FC,Conti M,Rakotoson MG,Habibi A,Khorgami S,Vingert B,Maitre B,Galacteros F,Pirenne F,Bartolucci P

更新日期：2016-10-01 00:00:00

abstract：:The effect of the synthetic vasopressin derivative 1-desamino-8D-arginine vasopressin (DDAVP = desmopressin) on bleeding time was studied in three patients with Hermansky Pudlak syndrome. A good response was observed in this type of storage pool disease. DDAVP might be useful in managing the bleeding disorder found in...

journal_title：American journal of hematology

authors： Wijermans PW,van Dorp DB

更新日期：1989-03-01 00:00:00

abstract：:Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 x 10(9)/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor th...

journal_title：American journal of hematology

authors： Dale DC,Cottle TE,Fier CJ,Bolyard AA,Bonilla MA,Boxer LA,Cham B,Freedman MH,Kannourakis G,Kinsey SE,Davis R,Scarlata D,Schwinzer B,Zeidler C,Welte K

更新日期：2003-02-01 00:00:00

abstract：:During the 5-year period from 1981 to 1985, we have observed 8 cases of acquired immunodeficiency syndrome (AIDS) among our 85 patients with hemophilia A. Thus, the prevalence of AIDS with hemophilia A is 9.4% in our patient population. By utilizing stored serum or plasma samples dating back to 1978, antibody against ...

journal_title：American journal of hematology

authors： Kim HC,Nahum K,Raska K Jr,Gocke DJ,Kosmin M,Karp GI,Saidi P

更新日期：1987-02-01 00:00:00

abstract：:The efficacy of the three common intra- and extragenic polymorphic sites of the factor VIII and IX genes has been examined in the Indian population, with an aim to develop a strategy that would be accurate and informative, yet economical. The approach for hemophilia A carrier detection includes tests for BclI, XbaI, a...

journal_title：American journal of hematology

authors： Shetty S,Ghosh K,Pathare A,Colah R,Badakare S,Mohanty D

更新日期：1997-04-01 00:00:00

abstract：:We analyzed the genetic defects of 21 unrelated patients with venous thrombosis in whom hereditary protein C deficiency was diagnosed. Eleven mutations were detected in 18 families, while no mutation was detectable in the other three families. Among these mutations, a common genetic mutation of protein C (PROC) gene r...

journal_title：American journal of hematology

authors： Tsay W,Shen MC

更新日期：2004-05-01 00:00:00

abstract：:Duvelisib (IPI-145), an oral, dual inhibitor of phosphoinositide-3-kinase (PI3K)-δ and -γ, was evaluated in a Phase 1 study in advanced hematologic malignancies, which included expansion cohorts in relapsed/refractory (RR) chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) and treatment-naïve (TN) CLL...

journal_title：American journal of hematology

authors： O'Brien S,Patel M,Kahl BS,Horwitz SM,Foss FM,Porcu P,Jones J,Burger J,Jain N,Allen K,Faia K,Douglas M,Stern HM,Sweeney J,Kelly P,Kelly V,Flinn I

更新日期：2018-11-01 00:00:00

abstract：:Aplastic anemia is a disorder characterized by pancytopenia and bone marrow hypocellularity. There is some evidence that aplastic anemia may be due to suppression of hematopoiesis by activated T-suppressor cells. Thus, immunosuppressive agents have been used as an alternative to bone marrow transplantation for treatme...

journal_title：American journal of hematology

authors： Dorr V,Doolittle G,Woodroof J

更新日期：1996-06-01 00:00:00

abstract：:Limited data are available regarding optimal treatment with desmopressin (DDAVP) or intermediate-purity FVIII concentrates rich in VWF (CFCs) in patients with von Willebrand disease (VWD) who undergo planned surgery. We undertook a retrospective review over 10 years (1988-1997) and identified 27 patients treated with ...

journal_title：American journal of hematology

authors： Nitu-Whalley IC,Griffioen A,Harrington C,Lee CA

更新日期：2001-04-01 00:00:00

abstract：:Acute Myeloid Leukemia (AML) and myelodysplasia (MDS) with chromosome 3q abnormalities have a dismal outcome either untreated or with conventional treatments. Azacitidine (AZA) is now considered as the standard of care in high-risk MDS and oligoblastic AML patients. The objective of this study was to evaluate the impa...

journal_title：American journal of hematology

authors： Wanquet A,Prebet T,Berthon C,Sebert M,Roux C,Kulasekararaj A,Micol JB,Esterni B,Itzykson R,Thepot S,Recher C,Delaunay J,Dreyfus F,Mufti G,Fenaux P,Vey N

更新日期：2015-10-01 00:00:00

abstract：:Since multiple myeloma (MM) is still not-curable, the management of relapse remains challenging. Given the known efficacy of alkylating agents in MM, we conducted a phase I/II study to test a new three drug combination in which Fotemustine (Muphoran), an alkylating agent of nitrosurea family, was added to bortezomib +...

journal_title：American journal of hematology

authors： Mangiacavalli S,Pochintesta L,Pascutto C,Cocito F,Pompa A,Cazzola M,Corso A

更新日期：2013-02-01 00:00:00

abstract：:Little is known about the frequency and significance of clinically unapparent or occult hemorrhage in ITP. Therefore, we prospectively explored the sites and frequency of occult bleeding in children with severe ITP at diagnosis or upon symptomatic relapse in a prospective, single-institution cohort study of patients ≤...

journal_title：American journal of hematology

authors： Flores A,Buchanan GR

更新日期：2016-03-01 00:00:00

abstract：:Lymphomatoid granulomatosis (LG) is an uncommon but potentially fatal disease. The disease primarily involves the lungs; however, skin, renal, and central nervous system (CNS) are seen in varying proportions. Neurological involvement occurs in one third of the patients, and confers a worse prognosis. The use of radiot...

journal_title：American journal of hematology

authors： Petrella TM,Walker IR,Jones GW,Leber B

更新日期：1999-12-01 00:00:00

abstract：:Complete remission can be achieved in 60-80% of adults with diffuse aggressive non-Hodgkin's lymphoma. However, 20-40% of them will subsequently relapse. Nevertheless, formal follow-up guidelines for recurrence detection have never been advocated. We analyzed the pattern of relapse in 30 patients with intermediate- an...

journal_title：American journal of hematology

authors： Elis A,Blickstein D,Klein O,Eliav-Ronen R,Manor Y,Lishner M

更新日期：2002-01-01 00:00:00