Hemosiderosis with diabetes mellitus in untransfused Hemoglobin H disease.

Abstract:

:A 37-year-old untransfused, non-drinking man with Hemoglobin H-CS disease presented with insulin-dependent diabetes mellitus, markedly elevated serum ferritin level, and marked iron deposition in hepatocytes. He did not carry either of the two common mutations of the HLA-H gene for hereditary hemochromatosis, namely, Cys282Tyr and His68Asp, nor did he have the associated HLA marker (HLA-A3, B7 nor B-14) for the disease. Patient with HbH disease should be monitored for iron overload.

journal_name

Am J Hematol

authors

Chim CS,Chan V,Todd D

doi

10.1002/(sici)1096-8652(199802)57:2<160::aid-ajh12

subject

Has Abstract

pub_date

1998-02-01 00:00:00

pages

160-3

issue

2

eissn

0361-8609

issn

1096-8652

pii

10.1002/(SICI)1096-8652(199802)57:2<160::AID-AJH12

journal_volume

57

pub_type

杂志文章
  • NK-cell repertoire is feasible for diagnosing Epstein-Barr virus-infected NK-cell lymphoproliferative disease and evaluating the treatment effect.

    abstract::Epstein-Barr virus (EBV) occasionally infects T and NK cells and causes EBV-infected T/NK-cell lymphoproliferative disease (LPD), which comprises chronic active EBV infection, EBV-associated hemophagocytic syndrome, mosquito allergy, hydroa vacciniforme, aggressive NK-cell leukemia, and NK/T-cell lymphoma. The diagnos...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20659

    authors: Sawada A,Sato E,Koyama M,Higuchi B,Kusuki S,Kim JY,Takeshita Y,Sakata A,Sakata N,Okamura T,Yasui M,Inoue M,Kawa K

    更新日期:2006-08-01 00:00:00

  • Laparoscopic splenectomies for idiopathic thrombocytopenic purpura: experience of sixty cases.

    abstract::We performed a laparoscopic splenectomy (LS) in 60 patients (age 9-83, 45 females) with idiopathic thrombocytopenic purpura (ITP) who did not achieve sustained remission on steroid therapy. Using a modified procedure, the mean duration of LS was 78 min (range 25-240 min) and surgery was associated with only 5% major a...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(200001)63:1<7::aid-ajh2>3.

    authors: Szold A,Schwartz J,Abu-Abeid S,Bulvik S,Eldor A

    更新日期:2000-01-01 00:00:00

  • Evaluation of factor VIII pharmacokinetics in hemophilia-A subjects undergoing surgery and description of a nomogram for dosing calculations.

    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

    pub_type: 杂志文章

    doi:10.1002/ajh.2830300305

    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

  • Addition of cladribine to the standard induction treatment improves outcomes in a subset of elderly acute myeloid leukemia patients. Results of a randomized Polish Adult Leukemia Group (PALG) phase II trial.

    abstract::Intensive induction chemotherapy using anthracycline and cytarabine backbone is considered the most effective upfront therapy in physically fit older patients with acute myeloid leukemia (AML). However, outcomes of the standard induction in elderly AML are inferior to those observed in younger patients, and they are s...

    journal_title:American journal of hematology

    pub_type: 杂志文章,随机对照试验

    doi:10.1002/ajh.24654

    authors: Pluta A,Robak T,Wrzesien-Kus A,Katarzyna Budziszewska B,Sulek K,Wawrzyniak E,Czemerska M,Zwolinska M,Golos A,Holowiecka-Goral A,Kyrcz-Krzemien S,Piszcz J,Kloczko J,Mordak-Domagala M,Lange A,Razny M,Madry K,Wiktor-Jedrze

    更新日期:2017-04-01 00:00:00

  • Autoimmune diseases and myelodysplastic syndromes.

    abstract::Immune dysregulation and altered T-cell hemostasis play important roles in the pathogenesis of myelodysplastic syndromes (MDS). Recent studies suggest an increased risk of MDS among patients with autoimmune diseases. Here, we investigated the prevalence of autoimmune diseases among MDS patients, comparing characterist...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.24333

    authors: Komrokji RS,Kulasekararaj A,Al Ali NH,Kordasti S,Bart-Smith E,Craig BM,Padron E,Zhang L,Lancet JE,Pinilla-Ibarz J,List AF,Mufti GJ,Epling-Burnette PK

    更新日期:2016-05-01 00:00:00

  • Hypercalcemia in idiopathic myelofibrosis: modulation of calcium and collagen homeostasis by 1,25-dihydroxyvitamin D3.

    abstract::A case of idiopathic myelofibrosis (IMF) presenting with hypercalcemia and hypercalcitriolemia is reported. It is proposed that ectopic production of the active vitamin D metabolite related to ongoing clonal expansion in the bone marrow accounts for the hypercalcemic state. Consistently low levels of circulating type ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830390316

    authors: Voss A,Schmidt K,Hasselbalch H,Junker P

    更新日期:1992-03-01 00:00:00

  • Autologous bone marrow transplantation versus chemotherapy in relapsed/refractory non-Hodgkin's lymphoma: estimates of long-term survival from the recent literature.

    abstract::Long-term survival following chemotherapy or autologous bone marrow transplantation in adults with relapsed/refractory non-Hodgkin's lymphoma was evaluated. English language articles published from January 1, 1988 to September 1, 1993 were obtained from a broad-based MEDLINE search retrieving 3,854 citations regarding...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.2830500208

    authors: Meehan KR,Pritchard RS,Leichter JW,Littenberg B,Welch HG

    更新日期:1995-10-01 00:00:00

  • Chronic lymphocytic leukemia: correlation of clinical course and therapeutic response with in vitro testing and morphology of lymphocytes.

    abstract::Forty-two patients with chronic lymphocytic leukemia (CLL) were studied for morphology of lymphocytes by light and electron microscopy (EM), in vitro responses of lymphocytes to a battery of physical and chemical agents, overall clinical status, immunologic status, course, and response to therapy. CLL lymphocytes coul...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830020110

    authors: Knospe WH,Gregory SA,Trobaugh FE Jr,Stedronsky JA,Schrek R

    更新日期:1977-01-01 00:00:00

  • The choice between splenectomy and medical treatment in patients with advanced agnogenic myeloid metaplasia.

    abstract::The objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myeloid metaplasia (AMM). We searched the literature (Medline, 1970-1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or trans...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.2830330210

    authors: Benbassat J,Gilon D,Penchas S

    更新日期:1990-02-01 00:00:00

  • Patterns of survival in lymphoplasmacytic lymphoma/Waldenström macroglobulinemia: a population-based study of 1,555 patients diagnosed in Sweden from 1980 to 2005.

    abstract::Clinical management of lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM) has changed considerably over recent years, reflected in the use of new therapeutic agents (purine analogs, monoclonal antibodies, thalidomide- and bortezomib-based therapies). No population-based studies and few randomized tria...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.23351

    authors: Kristinsson SY,Eloranta S,Dickman PW,Andersson TM,Turesson I,Landgren O,Björkholm M

    更新日期:2013-01-01 00:00:00

  • Coincidental polycythemia vera and multiple myeloma: case report and review.

    abstract::Polycythemia vera (PCV) and multiple myeloma are both clonal disorders of hematopoietic stem cells. The simultaneous occurrence of these diseases in an individual patient is rare. A case of synchronous PCV and smoldering myeloma is presented and the literature is reviewed. The issues of clinical importance in this unu...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.2830440311

    authors: Fink L,Bauer F,Perry JJ

    更新日期:1993-11-01 00:00:00

  • Enhancement of sickle erythrocyte adherence to endothelium by autologous platelets.

    abstract::The increased adhesiveness of sickle erythrocytes (SS RBC) to endothelial cells has been confirmed in a static system utilizing fresh umbilical vein endothelium. Adherence of SS RBC to the endothelium was as great in the presence of calcium-containing buffer as when incubated in plasma. SS RBC suspended in autologous ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830340110

    authors: Antonucci R,Walker R,Herion J,Orringer E

    更新日期:1990-05-01 00:00:00

  • Deficient proliferation of myeloid, erythroid, and multipotent progenitor cells in long-term marrow cultures from patients with aplastic anemia treated with immunosuppressive therapy.

    abstract::By using Dexter-type long-term marrow cultures (D-LTMC), it has been shown previously that hematopoietic progenitor cells (HPC) from patients with aplastic anemia (AA) have a deficient proliferation in vitro. The studies reported to date, however, have focused exclusively on granulomonocytic progenitors and no informa...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199810)59:2<149::aid-ajh8>

    authors: Gómez-Morales E,Martinez-Jaramillo G,Sánchez-Valle E,Valencia-Plata I,Arana-Trejo RM,Castro MA,Pizzuto-Chávez J,Mayani H

    更新日期:1998-10-01 00:00:00

  • Occult hemorrhage in children with severe ITP.

    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

    pub_type: 杂志文章

    doi:10.1002/ajh.24264

    authors: Flores A,Buchanan GR

    更新日期:2016-03-01 00:00:00

  • Phase II trial of high-dose dexamethasone for previously treated immunoglobulin light-chain amyloidosis.

    abstract::Immunoglobulin light-chain amyloidosis (AL) is a rare disorder characterized by production of a monoclonal light chain. This insoluble light chain, or a fragment thereof, deposits in tissues as amyloid and results in disruption of organ function and, ultimately, in death. Although melphalan and prednisone are benefici...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1002/(sici)1096-8652(199906)61:2<115::aid-ajh7>

    authors: Gertz MA,Lacy MQ,Lust JA,Greipp PR,Witzig TE,Kyle RA

    更新日期:1999-06-01 00:00:00

  • New alpha 2 globin chain variant with low oxygen affinity affecting the N-terminal residue and leading to N-acetylation [Hb Lyon-Bron alpha 1(NA1)Val --> Ac-Ala].

    abstract::Hemoglobin Lyon-Bron was found in two members of a family of German ascent presenting with a moderate normocytic anemia. In this alpha 2 globin variant, the N-terminal valine of the chain was replaced by an alanine. Electrospray mass spectrometry of the alpha chain showed that, as normally, the initiator methionine wa...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.10051

    authors: Lacan P,Souillet G,Aubry M,Promé D,Richelme-David S,Kister J,Wajcman H,Francina A

    更新日期:2002-03-01 00:00:00

  • Epidemiology, clinical picture and long-term outcomes of FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia: Data from 151 patients.

    abstract::FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been i...

    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.25945

    authors: Rohmer J,Couteau-Chardon A,Trichereau J,Panel K,Gesquiere C,Ben Abdelali R,Bidet A,Bladé JS,Cayuela JM,Cony-Makhoul P,Cottin V,Delabesse E,Ebbo M,Fain O,Flandrin P,Galicier L,Godon C,Grardel N,Guffroy A,Hamidou M,

    更新日期:2020-11-01 00:00:00

  • The hope and reality of long-acting hemophilia products.

    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

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.23146

    authors: Pipe SW

    更新日期:2012-05-01 00:00:00

  • Cardioprotective effect of metoprolol and enalapril in doxorubicin-treated lymphoma patients: a prospective, parallel-group, randomized, controlled study with 36-month follow-up.

    abstract::Anthracyclines have contributed to a marked increase in survival in different types of cancer [1,2]. Unfortunately, they are associated with dose-dependent cardiotoxicity and heart failure (HF) [3–8]. Change to a weekly dosage schedule with slow infusions has been tested, a strategy that requires more frequent hospita...

    journal_title:American journal of hematology

    pub_type: 杂志文章,随机对照试验

    doi:10.1002/ajh.21840

    authors: Georgakopoulos P,Roussou P,Matsakas E,Karavidas A,Anagnostopoulos N,Marinakis T,Galanopoulos A,Georgiakodis F,Zimeras S,Kyriakidis M,Ahimastos A

    更新日期:2010-11-01 00:00:00

  • High-density lipoprotein fails to inhibit serotonin-induced activation of blood platelets.

    abstract::High-density lipoprotein (HDL) of 100-400 micrograms/ml did not prevent morphological alterations of human blood platelets treated with serotonin (1-5 microM). Highly concentrated HDL (1,200 micrograms/ml) appeared to activate platelets in vitro. These findings indicate that whole HDL may not inhibit agonist-induced p...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830500417

    authors: Pfennig O,Zhao B,Frye S,Dierichs R

    更新日期:1995-12-01 00:00:00

  • A single-center experience in 20 patients with infantile malignant osteopetrosis.

    abstract::Infantile malignant osteopetrosis (IMO) includes various genetic disorders that affect osteoclast development and/or function. Genotype-phenotype correlation studies in IMO have been hampered by the rarity and heterogeneity of the disease and by the severity of the clinical course, which often leads to death early in ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.21447

    authors: Mazzolari E,Forino C,Razza A,Porta F,Villa A,Notarangelo LD

    更新日期:2009-08-01 00:00:00

  • Exclusion of the stomatin, alpha-adducin and beta-adducin loci in a large kindred with dehydrated hereditary stomatocytosis.

    abstract::Defects in stomatin, alpha-adducin, and beta-adducin have been implicated in erythrocyte disorders of cation permeability. We performed linkage analysis of the genetic loci for these proteins in a large kindred with xerocytosis (dehydrated hereditary stomatocytosis). Using polymerase chain reaction-based genotyping te...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199901)60:1<72::aid-ajh13>

    authors: Innes DS,Sinard JH,Gilligan DM,Snyder LM,Gallagher PG,Morrow JS

    更新日期:1999-01-01 00:00:00

  • Familial association of autoimmune thrombocytopenia and hyperthyroidism.

    abstract::An association between thrombocytopenia and thyrotoxicosis in a single individual is well documented, and the theories for this event include a common immunologic cause or a thyrotoxic-induced decrease in platelet survival. We report the first description of the coexistence of autoimmune thrombocytopenic purpura (AITP...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830390411

    authors: Bizzaro N

    更新日期:1992-04-01 00:00:00

  • C/EBPA gene mutation and C/EBPA promoter hypermethylation in acute myeloid leukemia with normal cytogenetics.

    abstract::In the current study, we investigated C/EBPA gene mutations and promoter hypermethylation in a series of 53 patients with CN-AML. In addition, we also analyzed two other frequent mutations (FLT3/ITD and NPM1) in these patients and correlated them with C/EBPA gene alterations. 13/53 patients were FLT3/ITD+/NPM1-, 11/53...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.21706

    authors: Lu Y,Chen W,Chen W,Stein A,Weiss LM,Huang Q

    更新日期:2010-06-01 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

  • Association of single nucleotide polymorphisms in the thrombopoietin-receptor gene, but not the thrombopoietin gene, with differences in platelet count.

    abstract::Little is known about the mechanisms explaining the wide variation in platelet counts (PLT) and other hematologic parameters in humans. We previously showed that the sex-based difference in hematocrit was associated with nucleotide variation in the erythropoietin receptor gene (EPOR). We sought to identify new polymor...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20095

    authors: Zeng SM,Murray JC,Widness JA,Strauss RG,Yankowitz J

    更新日期:2004-09-01 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

  • Evidence that circulating immune complexes remove transfused platelets from the circulation.

    abstract::Fifteen recently diagnosed patients with acute leukemias admitted for induction chemotherapy were selected for study. When thrombocytopenic (venous platelet count less than 20 X 10(9)/l) these patients received prophylactic platelet transfusions. A total of 67 platelet transfusion therapies were administered and evalu...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830110305

    authors: Kutti J,Zaroulis CG,Safai-Kutti S,Dinsmore RE,Day NK,Good RA

    更新日期:1981-11-01 00:00:00

  • Clinical and molecular predictors of disease severity and survival in chronic lymphocytic leukemia.

    abstract::Several parameters may predict disease severity and overall survival in chronic lymphocytic leukemia (CLL). The purpose of our study of 190 CLL patients was to compare immunoglobulin heavy chain variable region (IgV(H)) mutation status, cytogenetic abnormalities, and leukemia cell CD38 and Zap-70 to older, traditional...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20987

    authors: Weinberg JB,Volkheimer AD,Chen Y,Beasley BE,Jiang N,Lanasa MC,Friedman D,Vaccaro G,Rehder CW,Decastro CM,Rizzieri DA,Diehl LF,Gockerman JP,Moore JO,Goodman BK,Levesque MC

    更新日期:2007-12-01 00:00:00

  • Quality of life and use of red cell transfusion in patients with myelodysplastic syndromes. A systematic review.

    abstract::The main treatment for many patients with Myelodysplastic Syndromes (MDS) remains red cell transfusion to attenuate the symptoms of chronic anemia. Fatigue can reduce a patient's health related quality of life (HRQoL), but there is little understanding of the optimal use of transfusions to improve this. A systematic r...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.21503

    authors: Pinchon DJ,Stanworth SJ,Dorée C,Brunskill S,Norfolk DR

    更新日期:2009-10-01 00:00:00