Hermansky-Pudlak syndrome: correction of bleeding time by 1-desamino-8D-arginine vasopressin.

abstract：:In addition to transferrin, ferritin can also function as a source of iron for heme synthesis (Gelvin D, et al. Blood 1996;88:3200-3207; Meyron-Holtz EG, et al. Blood 1999;94:3205-3211). In the present study we investigate the effect of external ferritin on the proliferation and hemoglobinization of human erythroid pr...

journal_title：American journal of hematology

authors： Leimberg JM,Konijn AM,Fibach E

更新日期：2003-07-01 00:00:00

abstract：:Granulocyte macrophage-colony-stimulating factor (GM-CSF) has shown promise as a means of alleviating leukopenia associated with a wide variety of disorders. It is currently undergoing evaluation as an adjunct to bone marrow transplantation but its use in patients with metabolic disorders, such as Hurler's syndrome (H...

journal_title：American journal of hematology

authors： Lang E,Cibull ML,Gallicchio VS,Henslee-Downey PJ,Davey DD,Messino MJ,Harder EJ

更新日期：1992-12-01 00:00:00

abstract：:Alpha-hemoglobin stabilizing protein (AHSP) is a potential modifier of beta-thalassemia by virtue of its ability to detoxify excess free alpha-globin. However, examination of patients with beta-thalassemia from a few geographic regions failed to identify obvious AHSP mutations. We extended these studies by analyzing A...

journal_title：American journal of hematology

authors： dos Santos CO,Zhou S,Secolin R,Wang X,Cunha AF,Higgs DR,Kwiatkowski JL,Thein SL,Gallagher PG,Costa FF,Weiss MJ

更新日期：2008-02-01 00:00:00

abstract：:Hydroxyurea is a drug that is used to treat some patients with sickle cell disease. We have measured the deformability of sickle erythrocytes incubated in hydroxyurea in vitro and found that hydroxyurea acts to decrease the deformability of these cells. The deformability of normal erythrocytes was not significantly af...

journal_title：American journal of hematology

authors： Huang Z,Louderback JG,King SB,Ballas SK,Kim-Shapiro DB

更新日期：2001-07-01 00:00:00

abstract：:Since trisomy 12 is the most common chromosome abnormality found in CLL and MDM2 has been mapped to this chromosome, we examined the possible association of MDM2 in the pathogenesis of CLL. A rearrangement of the MDM2 gene was observed in 4 of 11 peripheral blood mononuclear cells (PBMC) from patients with CLL by Sout...

journal_title：American journal of hematology

authors： Huang YQ,Raphael B,Buchbinder A,Li JJ,Zhang WG,Friedman-Kien AE

更新日期：1994-10-01 00:00:00

abstract：:Everolimus is an oral antineoplastic agent that targets the raptor mammalian target of rapamycin (mTORC1). The phosphatidylinositol 3-kinase/mTOR signal transduction pathway has been demonstrated to be activated in tumor samples from patients with Hodgkin lymphoma (HL). The goal of this trial was to learn the antitumo...

journal_title：American journal of hematology

authors： Johnston PB,Inwards DJ,Colgan JP,Laplant BR,Kabat BF,Habermann TM,Micallef IN,Porrata LF,Ansell SM,Reeder CB,Roy V,Witzig TE

更新日期：2010-05-01 00:00:00

abstract：:The Muir-Torre syndrome (MTS) is defined as the concurrent or sequential discovery of at least one sebaceous gland tumor and a minimum of one internal malignancy. A man with Hodgkin's lymphoma who subsequently developed an ocular sebaceous carcinoma in situ is described and the world literature of patients with the MT...

journal_title：American journal of hematology

authors： Cohen PR

更新日期：1992-05-01 00:00:00

abstract：:Glomerular hyperfiltration and microalbuminuria/proteinuria are early manifestations of sickle nephropathy. The effects of hydroxyurea therapy on these renal manifestations of sickle cell anemia (SCA) are not well defined. Our objective was to investigate the effects of hydroxyurea on glomerular filtration rate (GFR) ...

journal_title：American journal of hematology

authors： Aygun B,Mortier NA,Smeltzer MP,Shulkin BL,Hankins JS,Ware RE

更新日期：2013-02-01 00:00:00

abstract：:Fibrillar granules (FGs) represent neutrophilic primary granules containing clustered filaments. We investigated neutrophils in the bone marrow obtained from 17 patients with chronic myeloproliferative disorders (CMPD) by electron microscopy. FG-positive neutrophils were seen in 15 of the 17 CMPDs with varying frequen...

journal_title：American journal of hematology

authors： Takemori N,Hirai K,Onodera R,Saito N,Miyokawa N

更新日期：1994-11-01 00:00:00

abstract：:In this study, we further established the role of interleukin-1 alpha (IL-1 alpha), interleukin-1 beta, tumor necrosis factor-alpha (TNF-alpha), and interferon-alpha (IFN-alpha) as regulators of proliferation of acute myeloid leukemia (AML) cells. AML cells from 8 of 15 patients incorporated high levels of 3H-thymidin...

journal_title：American journal of hematology

authors： Carter A,Silvian-Draxler I,Tatarsky I

更新日期：1992-08-01 00:00:00

abstract：:We report the clinical, molecular, and immunohistological findings of 20 Chinese patients with mantle cell lymphoma diagnosed over a 10-year period. The disease affected mainly elderly patients (median age, 65.5 years) with a male predominance (M/F, 3/1). Eighty percent presented with advanced stage III/IV disease but...

journal_title：American journal of hematology

authors： Chim CS,Chan AC,Choo CK,Kwong YL,Lie AK,Liang R

更新日期：1998-12-01 00:00:00

abstract：DISEASE OVERVIEW:Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, and lymphadenopathy. DIAGNOSIS:Presence of IgM monoclonal protein associated with 10% clonal lymphoplasmacytic cell...

journal_title：American journal of hematology

authors： Gertz MA

更新日期：2011-05-01 00:00:00

abstract：:While surgery is commonly required for complications related to hemoglobin SC (HbSC) disease, little is known about the perioperative complications or the indications for preoperative transfusion in this group. We describe the patient characteristics, preoperative transfusion regimens, and outcome in 92 patients with ...

journal_title：American journal of hematology

authors： Neumayr L,Koshy M,Haberkern C,Earles AN,Bellevue R,Hassell K,Miller S,Black D,Vichinsky E

更新日期：1998-02-01 00:00:00

abstract：:Although treatment with plasma exchange increased the survival of patients with thrombotic thrombocytopenia purpura to 80% in the 1980s, no further increase of survival occurred over the next 20 years. However, more consistent use of adjuvant treatment with corticosteroids and rituximab in recent years has begun to fu...

journal_title：American journal of hematology

authors： George JN

更新日期：2012-05-01 00:00:00

abstract：:In patients with acute myelogenous leukemia (AML), 34 out of 35 patients (97%) had cytochemically abnormal polymorphonuclear neutrophils (PMN). Some interesting regularity was noted in the appearance of cytochemical abnormalities of the PMNs. When peroxidase (PO) activity was low, both alkaline phosphatase (AP) and ch...

journal_title：American journal of hematology

authors： Suda T,Onai T,Maekawa T

更新日期：1983-08-01 00:00:00

abstract：:Between 1980 and 1988, 126 patients with leukemia were treated with piperazinedione and fractionated total body irradiation (TBI) followed by allogeneic bone marrow transplantation from HLA matched siblings. Sixty-one patients had acute myelogenous leukemia, 46 acute lymphoblastic leukemia, and 19 chronic myelogenous ...

journal_title：American journal of hematology

authors： Dimopoulos MA,Yau JC,Huan SD,Jagannath S,Spitzer G,Spinolo JA,Zagars GK,LeMaistre CF,Dicke KA,Zander AR

更新日期：1994-06-01 00:00:00

abstract：:The relationship between thrombocytopenia and the level of anticardiolipin antibodies (aCL) and/or the existence of lupus anticoagulant (LA) ware studied in 146 patients with systemic lupus erythematosus (SLE). These patients were divided into six groups: A, those LA positive with a high level of aCL (>10 U/ml) (10 ca...

journal_title：American journal of hematology

authors： Nojima J,Suehisa E,Kuratsune H,Machii T,Toku M,Tada H,Yamaguti K,Koike T,Kanakura Y,Kitani T,Amino N

更新日期：1998-05-01 00:00:00

abstract：:This case report describes a patient with hepatitis C virus infection responding to pegylated INF/ribaviron therapy, who developed immune thrombocytopenia. The severe thrombocytopenia failed to resolve with cessation of the peg-IFN/ribaviron. Because of rising hepatitis C virus RNA levels and evidence of rising serum ...

journal_title：American journal of hematology

authors： Weitz IC

更新日期：2005-02-01 00:00:00

abstract：:Sickle cell disease is a single amino acid molecular disorder of hemoglobin leading to its pathological polymerization, red cell rigidity that causes poor microvascular blood flow, with consequent tissue ischemia and infarction. The manifestations of this disease are protean.Among them, leg ulcers represent a particul...

journal_title：American journal of hematology

authors： Minniti CP,Eckman J,Sebastiani P,Steinberg MH,Ballas SK

更新日期：2010-10-01 00:00:00

abstract：:An adolescent male patient developed functional asplenia in the course of graft versus host disease (GVHD) after successful allogeneic bone marrow transplantation (BMT) for aplastic anemia. Coincident with the onset of the asplenia, amelioration of the ongoing GVHD was observed. Unexpectedly, after 34 months of functi...

journal_title：American journal of hematology

authors： Demetrakopoulos GE,Tsokos GC,Levine AS

更新日期：1982-02-01 00:00:00

abstract：:A discordance between p53 protein overexpression and the presence of mutations in the gene has been observed in many types of tumors, including human lymphomas. To probe this finding, we have studied a large series of 94 lymphomas of different pathologic types and histologic differentiation. Analyzing exons 5-9, we ha...

journal_title：American journal of hematology

authors： Martinez-Delgado B,Robledo M,Arranz E,Infantes F,Echezarreta G,Marcos B,Sanz C,Rivas C,Benitez J

更新日期：1997-05-01 00:00:00

abstract：:Pentosan polysulfate is a low-molecular-weight sulfated polysaccharide used as an antithrombotic drug. We present two patients who developed thrombocytopenia and venous thrombosis during treatment with pentosan polysulfate. The relationship between pentosan polysulfate and thrombocytopenia is supported by platelet agg...

journal_title：American journal of hematology

authors： Tardy-Poncet B,Tardy B,Grelac F,Reynaud J,Mismetti P,Bertrand JC,Guyotat D

更新日期：1994-03-01 00:00:00

abstract：:Recombinant DNA technology and protein engineering are creating hope that we can address ongoing challenges in hemophilia care such as reducing the costs of therapy, increasing the availability to the developing world, and improving the functional properties of these proteins. Technological advances to improve the hal...

journal_title：American journal of hematology

authors： Pipe SW

更新日期：2012-05-01 00:00:00

abstract：:Vascular complications are the main cause of morbidity in diabetes mellitus. To evaluate lipoprotein and hemostatic parameters and their relationship with clinically detectable microangiopathy, we studied 58 insulin-dependent diabetes mellitus patients and 60 controls matched for age, sex, and body mass index. Thirtee...

journal_title：American journal of hematology

authors： Reverter JL,Reverter JC,Tàssies D,Rius F,Monteagudo J,Rubiés-Prat J,Escolar G,Ordinas A,Sanmarti A

更新日期：1997-10-01 00:00:00

abstract：:The recent progress in sensitive KIT D816V mutation analysis suggests that mutation analysis of peripheral blood (PB) represents a promising diagnostic test in mastocytosis. However, there is a need for systematic assessment of the analytical sensitivity and specificity of the approach in order to establish its value ...

journal_title：American journal of hematology

authors： Kristensen T,Vestergaard H,Bindslev-Jensen C,Møller MB,Broesby-Olsen S,Mastocytosis Centre, Odense University Hospital (MastOUH).

更新日期：2014-05-01 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：:Reduced ferrochelatase activity in erythropoietic protoporphyria (EPP) causes the accumulation of protoporphyrin IX (PPIX) leading to acute cutaneous photosensitivity and liver injury. Many EPP patients also have a mild hypochromic, microcytic anemia and iron deficiency. Iron deficiency can lead to decreased PPIX accu...

journal_title：American journal of hematology

authors： Schmidt PJ,Hollowell ML,Fitzgerald K,Butler JS,Fleming MD

更新日期：2020-05-01 00:00:00

abstract：:Patients with MM are at an increased risk for life-threatening bacterial infections, primarily by organisms that require opsonization for interaction with granulocytes. In the present study we used a neutrophil chemiluminescence (CL) assay of opsonization to explore the opsonic activity of MM-Ig for zymosan particles....

journal_title：American journal of hematology

authors： Cheson BD

更新日期：1981-12-01 00:00:00

abstract：:A single point mutation of the factor V (FV) gene, leading to the substitution Arg506Gln in the FV molecule (FV-Leiden) and hence resistance to its breakdown by activated protein C (APC), is the most prevalent risk factor for venous thrombosis in the Caucasians. A ratio determined by activated partial thromboplastin t...

journal_title：American journal of hematology

authors： Ts'ao C,Neofotistos D,Oropeza M,Vahabinejad S,Finn WG

更新日期：1997-03-01 00:00:00

abstract：:A patient with Philadelphia chromosome (Ph) chronic myelogenous leukemia (CML), in chronic phase, was treated with recombinant gamma-interferon (r gamma-IFN) in a phase I clinical trial. Prior to treatment, analysis of in vitro agar culture parameters indicated hyporesponsiveness of granulocyte-macrophage colony-formi...

journal_title：American journal of hematology

authors： Pelus LM,Vadhan-Raj S

更新日期：1988-05-01 00:00:00