Prevalence of abnormal iron studies in heterozygotes for hereditary hemochromatosis: an analysis of 255 heterozygotes.

Abstract:

:Iron studies were compared in 434 patients from 80 hemochromatosis families classified as putative homozygotes, heterozygotes, and normal by HLA typing. There were 28 of 255 (11%) heterozygotes with an elevated serum ferritin and 22 of 255 (8.6%) with an elevated transferrin saturation. Serum ferritin (140 +/- 10.2 micrograms/liter; mean +/- standard error) was greater in heterozygotes than in normal subjects (87 +/- 8.5 micrograms/liter; P < .05, Mann Whitney test). Transferrin saturation was greater in heterozygotes (38% +/- 0.88%) than in normal patients (29% +/- 1.1%; P < .0001). Mean hepatic iron concentration was 54 +/- 6 mumol/g (n = 17), and the hepatic iron index was < 2 in these patients. Most heterozygotes for hemochromatosis have a normal serum ferritin and transferrin saturation. Heterozygotes with minor elevations in serum ferritin or transferrin saturation do not have significant iron overload as assessed by hepatic iron concentration.

journal_name

Am J Hematol

authors

Adams PC

doi

10.1002/ajh.2830450210

subject

Has Abstract

pub_date

1994-02-01 00:00:00

pages

146-9

issue

2

eissn

0361-8609

issn

1096-8652

journal_volume

45

pub_type

杂志文章
  • Biochemical markers of bone turnover in diagnosis of myeloma bone disease.

    abstract::This study was designed to explore the value of markers of bone turnover, macrophage inflammatory protein-1alpha (MIP-1alpha), and osteopontin (OPN) in the diagnosis of myeloma bone disease. Twenty-five patients with newly diagnosed and untreated multiple myeloma (MM), and 22 age-, sex-, and bone mineral density-match...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20794

    authors: Dizdar O,Barista I,Kalyoncu U,Karadag O,Hascelik G,Cila A,Pinar A,Celik I,Kars A,Tekuzman G

    更新日期:2007-03-01 00:00:00

  • Progressive refractory light chain amyloidosis and multiple myeloma patients are responsive to the addition of clarithromycin to IMiD based therapy.

    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

    pub_type: 杂志文章

    doi:10.1002/ajh.24596

    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

  • Superficial venous thrombosis associated with congenital absence of the inferior vena cava and previous episode of deep venous thrombosis.

    abstract::Congenital malformations of the inferior vena cava (IVC) are uncommon and may be associated with an increased risk of venous thrombosis. We report the case of a man with congenital absence of the IVC and remote history of deep venous thrombosis who now presents with severe abdominal wall superficial thrombophlebitis. ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.21089

    authors: Evanchuk DM,Von Gehr A,Zehnder JL

    更新日期:2008-03-01 00:00:00

  • Hodgkin disease in a patient with common variable immunodeficiency.

    abstract::Extensive extralymphatic Hodgkin disease developed in a young man with common variable immunodeficiency manifested by hypogammaglobulinemia, recurrent sinopulmonary infections, and multiple autoimmune phenomena. Both humoral and cell-mediated immune dysfunction were present prior to treatment. After two cycles of chem...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830320212

    authors: Fesus SM,Hagemeister FB,Manning J

    更新日期:1989-10-01 00:00:00

  • Anti-lymphoma effect of naproxen and indomethacin in a patient with relapsed diffuse large B-cell lymphoma.

    abstract::A 77-year-old man with relapsed non-Hodgkin's lymphoma, diffuse large B-cell type, was treated with naproxen, a nonsteroidal anti-inflammatory drug (NSAID), for paraneoplastic fever. A dramatic disappearance of not only the fever but also generalized lymphadenopathy was observed. Naproxen was continued, and he maintai...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/1096-8652(200103)66:3<220::aid-ajh1048>3.0

    authors: Yoshinaga K,Teramura M,Iwabe K,Kobayashi S,Masuda M,Motoji T,Mizoguchi H

    更新日期:2001-03-01 00:00:00

  • In vitro generated Rh(null) red cells recapitulate the in vivo deficiency: a model for rare blood group phenotypes and erythroid membrane disorders.

    abstract::Lentiviral modification combined with ex vivo erythroid differentiation was used to stably inhibit RhAG expression, a critical component of the Rh(rhesus) membrane complex defective in the Rh(null) syndrome. The cultured red cells generated recapitulate the major alterations of native Rh(null) cells regarding antigen ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.23414

    authors: Cambot M,Mazurier C,Canoui-Poitrine F,Hebert N,Picot J,Clay D,Picard V,Ripoche P,Douay L,Dubart-Kupperschmitt A,Cartron JP

    更新日期:2013-05-01 00:00:00

  • Clinical presentation of severe anemia in pediatric patients with sickle cell anemia seen in Enugu, Nigeria.

    abstract::Anemia is a major cause of morbidity and mortality among patients with sickle cell anemia. In this study, 108 episodes of severe anemia were prospectively evaluated in 108 patients with hemoglobin SS disease attending the pediatric sickle cell clinic of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Youn...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.10285

    authors: Juwah AI,Nlemadim A,Kaine W

    更新日期:2003-03-01 00:00:00

  • Biologic and clinical significance of CD7 expression in acute myeloid leukemia.

    abstract::CD7 antigen, a T-cell lineage associated antigen, is expressed in a minority of patients with acute myeloid leukemia (AML). The biologic and clinical significance of this finding is not clearly established. In this retrospective study of patients with de novo acute myeloid leukemia, we have identified CD7 expression a...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199808)58:4<278::aid-ajh5>

    authors: Saxena A,Sheridan DP,Card RT,McPeek AM,Mewdell CC,Skinnider LF

    更新日期:1998-08-01 00:00:00

  • T-cell non-Hodgkin lymphoma in human immunodeficiency virus-1-infected individuals.

    abstract::We present two patients with human immunodeficiency virus-1 (HIV-1) infection in whom T-cell non-Hodgkin lymphoma developed, based on pathologic diagnosis, immunophenotyping, and T-cell receptor gene rearrangement. Both cases were positive for human immunodeficiency virus-1 by enzyme-linked immunosorbent assay and imm...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830310307

    authors: Lust JA,Banks PM,Hooper WC,Paya CV,Kueck BD,Hanson GA,Ritch PS,Woloschak GE

    更新日期:1989-07-01 00:00:00

  • Continuous flow method for determination of erythrocyte osmotic fragility.

    abstract::A simple and accurate micromethod for the determination of erythrocyte osmotic fragility is introduced. The method uses a laminar parabolic flow pattern, together with gravity, to retain cells in a long, small-diameter tube while a solution with decreasing osmolarity is passed through the tube. As the cells hemolyze, ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830020411

    authors: Ito Y,Carmeci P,Steele R

    更新日期:1977-01-01 00:00:00

  • Enhancement of hemoglobin and F-cell production by targeting growth inhibition and differentiation of K562 cells with ribonucleotide reductase inhibitors (didox and trimidox) in combination with streptozotocin.

    abstract::Upon appropriate drug treatment, the human erythroleukemic K562 cells have been shown to produce hemoglobin and F-cells. Fetal hemoglobin (Hb F) inhibits the polymerization events of sickle hemoglobin (Hb S), thereby ameliorating the clinical symptoms of sickle cell disease. Ribonucleotide reductase inhibitors (RRIs) ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(200004)63:4<176::aid-ajh3>

    authors: Iyamu WE,Adunyah SE,Fasold H,Horiuchi K,Elford HL,Asakura T,Turner EA

    更新日期:2000-04-01 00:00:00

  • MALT lymphoma and Kaposi sarcoma in an HIV-negative patient.

    abstract::A 77-year-old caucasian man presented on March 2005 with important epigastric pain without any other significant history of gastritis. Patient refers a history of cutaneous Kaposi's sarcoma (KS) treated since 1974 with surgical excision or oncovorin topical injection. He underwent endoscopic evaluation showing a 1.5-c...

    journal_title:American journal of hematology

    pub_type: 信件

    doi:10.1002/ajh.21802

    authors: Mirabile A,Devizzi L,Gianni AM,Cabras A,Carbone A

    更新日期:2010-10-01 00:00:00

  • Comparative outcomes of myeloablative and reduced-intensity conditioning allogeneic hematopoietic cell transplantation for therapy-related acute myeloid leukemia with prior solid tumor: A report from the acute leukemia working party of the European societ

    abstract::Therapy-related acute myeloid leukemia (t-AML) arises as a late complication following antecedent solid tumors or hematologic diseases and their associated treatments. There are limited data regarding risk factors and outcomes following allogeneic hematopoietic cell transplantation (HCT) for t-AML following a prior so...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1002/ajh.25395

    authors: Lee CJ,Labopin M,Beelen D,Finke J,Blaise D,Ganser A,Itälä-Remes M,Chevallier P,Labussière-Wallet H,Maertens J,Yakoub-Agha I,Bourhis JH,Mailhol A,Mohty M,Savani BN,Nagler A

    更新日期:2019-04-01 00:00:00

  • von Willebrand's disease: use of collagen binding assay provides potential improvement to laboratory monitoring of desmopressin (DDAVP) therapy.

    abstract::This report describes studies investigating the use of a collagen binding assay to improve the laboratory monitoring of desmopressin (DDAVP) therapy in patients with von Willebrand's disease (vWD). We evaluated the response of seven patients with vWD (four type I, three type IIA) to DDAVP, administered using a standar...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830450303

    authors: Favaloro EJ,Dean M,Grispo L,Exner T,Koutts J

    更新日期:1994-03-01 00:00:00

  • Visceral leishmaniasis masquerading as myelodysplasia.

    abstract::We report a case of a 61-year-old man with head and neck cancer who presented with pancytopenia two months after the completion of his chemotherapy and was diagnosed with myelodysplasia on the basis of two bone marrow examinations, before the correct diagnosis of visceral leishmaniasis was established with splenectomy...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.10408

    authors: Kopterides P,Halikias S,Tsavaris N

    更新日期:2003-11-01 00:00:00

  • IDH mutations are closely associated with mutations of DNMT3A, ASXL1 and SRSF2 in patients with myelodysplastic syndromes and are stable during disease evolution.

    abstract::Current information about clinical significance of IDH mutations in myelodysplastic syndromes (MDS), their association with other genetic alterations and the stability during disease progression is limited. In this study, IDH mutations were identified in 4.6% of 477 patients with MDS based on the FAB classification an...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.23596

    authors: Lin CC,Hou HA,Chou WC,Kuo YY,Liu CY,Chen CY,Lai YJ,Tseng MH,Huang CF,Chiang YC,Lee FY,Liu MC,Liu CW,Tang JL,Yao M,Huang SY,Ko BS,Wu SJ,Tsay W,Chen YC,Tien HF

    更新日期:2014-02-01 00:00:00

  • Hepatosplenic gammadelta T-cell lymphoma in a 10-year-old boy successfully treated with hematopoietic stem cell transplantation.

    abstract::The authors report a 10-year-old boy with hepatosplenic gammadelta T-cell lymphoma, a rare form of lymphoma that is highly aggressive, exceedingly rare in children, and primarily seen in young men. Conventional multi-agent chemotherapy appears to be inadequate for cure. This is the first report with this type of lymph...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.10466

    authors: Gassas A,Kirby M,Weitzman S,Ngan B,Abla O,Doyle JJ

    更新日期:2004-02-01 00:00:00

  • The first two Japanese cases of severe type I congenital plasminogen deficiency with ligneous conjunctivitis: successful treatment with direct thrombin inhibitor and fresh plasma.

    abstract::A 71-year-old woman and her elder sister developed ligneous conjunctivitis after ocular surgery. Laboratory tests demonstrated that the proband and her sister had 6.6% and 8.1% of plasminogen activity, and 1.2 and 1.4 mg/dl of antigen, respectively. Thus, they were diagnosed as having severe type I plasminogen deficie...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.21402

    authors: Suzuki T,Ikewaki J,Iwata H,Ohashi Y,Ichinose A

    更新日期:2009-06-01 00:00:00

  • Venous thromboembolism prophylaxis in medically ill patients and the development of strategies to improve prophylaxis rates.

    abstract::Venous thromboembolism (VTE) is common but often unrecognized in medically ill patients. Over the past 5 years, three large-scale placebo-controlled trials enrolling a total of 5500 medically ill patients have highlighted the risk of VTE in this group. These trials have helped to define a specific at-risk patient prof...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20281

    authors: Stinnett JM,Pendleton R,Skordos L,Wheeler M,Rodgers GM

    更新日期:2005-03-01 00:00:00

  • Retraction: 'Number needed to treat with 4-factor prothrombin complex concentrate for urgent warfarin reversal' by Andrew Chua, Vishal Patel, Allison Perrin, Lee Stern, Jenifer Ehreth, Laurel Omert, Christopher Hood, Julie Farley, Michael McGlynn and Lipi

    abstract::The above abstract from the THSNA 2016 Summit Abstract Proceedings, first published online in the American Journal of Hematology on 20 July 2016 in Wiley OnlineLibrary (www.onlinelibrary.wiley.com), and in Volume 91, Issue 9, p. E427, has been retracted by agreement between the authors, the journal Editor-in-Chief, Ca...

    journal_title:American journal of hematology

    pub_type: 杂志文章,撤回出版物

    doi:10.1002/ajh.24678

    authors:

    更新日期:2017-04-01 00:00:00

  • Second-line treatments in children with immune thrombocytopenia: Effect on platelet count and patient-centered outcomes.

    abstract::Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder with isolated thrombocytopenia and hemorrhagic risk. While many children with ITP can be safely observed, treatments are often needed for various reasons, including to decrease bleeding, or to improve health related quality of life (HRQoL). There are a n...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1002/ajh.25479

    authors: Grace RF,Shimano KA,Bhat R,Neunert C,Bussel JB,Klaassen RJ,Lambert MP,Rothman JA,Breakey VR,Hege K,Bennett CM,Rose MJ,Haley KM,Buchanan GR,Geddis A,Lorenzana A,Jeng M,Pastore YD,Crary SE,Neier M,Neufeld EJ,Neu N

    更新日期:2019-07-01 00:00:00

  • Fetal hemoglobin expression in compound heterozygotes for -117 (G-->A)A gamma HPFH and beta zero 39 nonsense thalassemia.

    abstract::The -117(G-->A)A gamma hereditary persistence of fetal hemoglobin (Greek HPFH) and beta zero 39-thal mutations are rather frequent in Sardinia so that their interaction is to be expected. Characterization of eight compound heterozygotes for these defects indicated that HPFH was linked to haplotype VII and beta zero 39...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830490402

    authors: Pistidda P,Frogheri L,Oggiano L,Guiso L,Manca L,Dore F,Masala B,Gilman JG,Longinotti M

    更新日期:1995-08-01 00:00:00

  • The prognostic value of monosomal karyotype (MK) in higher-risk patients with myelodysplastic syndromes treated with 5-Azacitidine: A retrospective analysis of the Hellenic (Greek) Myelodysplastic syndromes Study Group.

    abstract::In this study, we investigated the incidence and prognostic impact of monosomal karyotype (MK) in 405 higher-risk Myelodysplastic Syndromes (MDS) patients treated with 5-AZA. The MK was present in 66 out of 405 (16.3%) patients, most of whom had complex karyotype (CK). MK was strongly associated with CK and the cytoge...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.25111

    authors: Papageorgiou SG,Vasilatou D,Kontos CK,Kotsianidis I,Symeonidis A,Galanopoulos AG,Hatzimichael E,Megalakaki A,Poulakidas E,Diamantopoulos P,Vassilakopoulos TP,Zikos P,Papadaki H,Mparmparousi D,Bouronikou E,Panayiotidis P,V

    更新日期:2018-07-01 00:00:00

  • Drug-associated agranulocytosis: 20 years of reporting in The Netherlands (1974-1994).

    abstract::In this descriptive study, all 425 reports were included concerning drug-associated agranulocytosis as registered between 1974 and 1994 in the files of the Drug Safety Unit of the Dutch Inspectorate for Health Care. All reports were analysed as to the probability of agranulocytosis or neutropenia according to previous...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199803)57:3<206::aid-ajh4>

    authors: van der Klauw MM,Wilson JH,Stricker BH

    更新日期:1998-03-01 00:00:00

  • Thalidomide in POEMS syndrome: case report.

    abstract::We report a patient with incapacitating POEMS syndrome characterized by serum monoclonal protein, polyneuropathy, organomegaly, endocrinopathy, mesangiocapillary glomerulonephritis, massive ascites formation, and pulmonary hypertension. A dramatic improvement in the clinical condition occurred after administration of ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20051

    authors: Sinisalo M,Hietaharju A,Sauranen J,Wirta O

    更新日期:2004-05-01 00:00:00

  • Immunologic thrombocytopenic purpura as presenting symptom of hepatitis C infection.

    abstract::We report on 3 female patients with immunologic thrombocytopenic purpura (ITP) for whom diagnostic procedures evidenced a chronic Hepatitis C virus (HCV) infection. In 2 cases, a transfusion performed more than 10 years ago represented the probable way of contamination. One patient received a course of steroids, which...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199804)57:4<338::aid-ajh12

    authors: Bauduer F,Marty F,Larrouy M,Ducout L

    更新日期:1998-04-01 00:00:00

  • Transient ischemic attack in a patient with congenital protein-C deficiency during treatment with stanozolol.

    abstract::A patient with congenital protein-C deficiency was treated with stanozolol for 8 weeks to increase circulating levels of protein C. A rise in protein C was achieved, accompanied by an increase in factor II, factor X, antithrombin III, and protein S; but at the 8th week the patient suffered a transient ischemia attack....

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830290211

    authors: De Stefano V,Leone G,Teofili L,Ferrelli R,Pollari G,Antonini V,Bizzi B

    更新日期:1988-10-01 00:00:00

  • Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment.

    abstract:DISEASE OVERVIEW:Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, h...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.25819

    authors: Gertz MA

    更新日期:2020-07-01 00:00:00

  • Systemic amyloidosis associated with chronic lymphocytic leukemia/small lymphocytic lymphoma.

    abstract::To clarify the presentation and course of patients with chronic lymphocytic leukemia (CLL) and amyloidosis. Mayo databases were interrogated for patients who carried a diagnosis of amyloidosis and CLL evaluated at Mayo Clinic, Rochester from January 1974 to October 2012. Charts were abstracted and data analyzed. Of th...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.23413

    authors: Kourelis TV,Gertz M,Zent C,Lacy M,Kyle R,Kapoor P,Zeldenrust S,Buadi F,Witzig T,Hayman S,Lust J,Russell S,Lin Y,Rajkumar VS,Kumar S,Leung N,Dingli D,Dispenzieri A

    更新日期:2013-05-01 00:00:00

  • Novel somatic mutations of the VHL gene in an erythropoietin-producing renal carcinoma associated with secondary polycythemia and elevated circulating endothelial progenitor cells.

    abstract::Mutation of the VHL tumor suppressor gene is a frequent genetic event in the carcinogenesis of renal-cell carcinoma (RCC). Circulating endothelial progenitor cells (EPCs) have important role in neoangiogenesis, and mobilization of these cells is induced by various growth factors including erythropoietin (EPO). With th...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.21019

    authors: Rad FH,Ulusakarya A,Gad S,Sibony M,Juin F,Richard S,Machover D,Uzan G

    更新日期:2008-02-01 00:00:00