The oxygen affinity of haemoglobin Hammersmith.

Abstract:

:Oxygen dissociation studies were carried out on red cells and lysates from a patient heterozygous for Hb Hammersmith. The oxygen affinity of the cells at pH 7.1 was decreased, partly by an increased cellular concentration of 2,3-DPG and more importantly by an intrinsic low affinity of Hb Hammersmity. Haem-haem interactions were reduced and an abnormal Bohr effect (pH 7.1-7.4) was found in the cells which would bring about an additional decrease in oxygen affinity at physiological pH. Oxygen dissociation studies on the lysates showed a low oxygen affinity, a normal Bohr effect at 50% saturation, slightly decreased haem-haem interactions and a normal interaction with 2,3-DPG. Estimation of the percentage of Hb Hammersmith in the lysates of the patient's cells by selective precipitation of the abnormal beta chain with p-chloromercuribenzoate (PCMB) showed the presence of 30-33% abnormal haemoglobin. Assuming no interaction between the Hb A and the Hb Hammersmith, oxygen dissociation curves were calculated for Hb Hammersmith alone and these indicated that it has greatly decreased oxygen affinity, a normal Bohr effect, normal 2,3-DPG interaction and (somewhat) decreased haem-haem interactions.

journal_name

Br J Haematol

authors

May A,Huehns ER

doi

10.1111/j.1365-2141.1975.tb00533.x

subject

Has Abstract

pub_date

1975-06-01 00:00:00

pages

185-95

issue

2

eissn

0007-1048

issn

1365-2141

journal_volume

30

pub_type

杂志文章
  • A randomized, double-blind trial of pegfilgrastim versus filgrastim for the management of neutropenia during CHASE(R) chemotherapy for malignant lymphoma.

    abstract::Pegfilgrastim is a pegylated form of the granulocyte-colony stimulating factor, filgrastim. Herein, we report the results of a multicentre, randomized, double-blind phase III trial comparing the efficacy and safety of pegfilgrastim with filgrastim in patients with malignant lymphoma. Patients were randomized to receiv...

    journal_title:British journal of haematology

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1111/bjh.14088

    authors: Kubo K,Miyazaki Y,Murayama T,Shimazaki R,Usui N,Urabe A,Hotta T,Tamura K

    更新日期:2016-08-01 00:00:00

  • Translocation t(5;12)(q31-q33;p12-p13): a non-random translocation associated with a myeloid disorder with eosinophilia.

    abstract::A t(5;12)(q33;p13) translocation has been detected in two patients with myeloid disorder and eosinophilia. Six other patients with haematological disease with eosinophilia with similar translocation have been published previously. The existence of a new entity, a myeloproliferative disorder with eosinophilia and t(5;1...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.1994.tb05029.x

    authors: Baranger L,Szapiro N,Gardais J,Hillion J,Derre J,Francois S,Blanchet O,Boasson M,Berger R

    更新日期:1994-10-01 00:00:00

  • P-glycoprotein expression on acute myeloid leukaemia blast cells at diagnosis predicts response to chemotherapy and survival.

    abstract::P-glycoprotein (Pgp) expression, which is associated with the multi-drug resistance (MDR) phenotype, has been reported to be a useful predictor of treatment outcome in acute leukaemia. We have examined the expression of Pgp on acute myeloid leukaemia (AML) cells in 54 newly diagnosed patients, using a novel streptavid...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1994.tb08305.x

    authors: Wood P,Burgess R,MacGregor A,Yin JA

    更新日期:1994-07-01 00:00:00

  • E4BP4 expression is regulated by the t(17;19)-associated oncoprotein E2A-HLF in pro-B cells.

    abstract::The E4BP4 basic leucine zipper (bZIP) transcription factor is regulated by interleukin-3 (IL-3) in pro-B cells and has been reported to promote survival of the murine IL-3-dependent pro-B cell lines, FL5.12 and Baf-3. The E2A-HLF oncoprotein arises from a t(17;19) translocation in childhood pro-B cell acute lymphoblas...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2004.04953.x

    authors: Yeung J,O'Sullivan E,Hubank M,Brady HJ

    更新日期:2004-06-01 00:00:00

  • Analysis of the kinetics of band 3 diffusion in human erythroblasts during assembly of the erythrocyte membrane skeleton.

    abstract::During definitive erythropoiesis, erythroid precursors undergo differentiation through multiple nucleated states to an enucleated reticulocyte, which loses its residual RNA/organelles to become a mature erythrocyte. Over the course of these transformations, continuous changes in membrane proteins occur, including shif...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2010.08268.x

    authors: Kodippili GC,Spector J,Kang GE,Liu H,Wickrema A,Ritchie K,Low PS

    更新日期:2010-09-01 00:00:00

  • In vitro growth in acute myeloblastic leukaemia: relationship with other clinico-biological characteristics of the disease.

    abstract::The in vitro growth characteristics of a large series of acute myeloid leukaemia (AML) patients and their relationship with other clinical and biological disease characteristics were analysed. Patients with AML were studied, 181 with de novo AML and 45 with secondary AML (24 myelodysplastic syndrome, sAML-MDS, 21 myel...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1998.00962.x

    authors: del Cañizo MC,Brufau A,Almeida J,Galende J,García Marcos MA,Mota A,García R,Fernández Calvo J,Ramos F,Fisac P,Orfao A,San Miguel JF

    更新日期:1998-10-01 00:00:00

  • Osteitis fibrosa cystica generalizata with adult T-cell leukaemia: a case report.

    abstract::We report on a 62-year-old female initially suffering from extreme pain in both her lower extremities. Plain radiographs revealed multiple osteolytic lesions. Laboratory analyses indicated high levels of serum calcium and parathyroid hormone related protein (PTHrP) and detected HTLV-1 antibody. Histological examinatio...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1999.01776.x

    authors: Yamaguchi T,Hirano T,Kumagai K,Tsurumoto T,Shindo H,Majima R,Arima N

    更新日期:1999-12-01 00:00:00

  • Acute leukaemia immunophenotyping in bone-marrow routine sections.

    abstract::Immunohistochemistry of acute leukaemias in bone-marrow paraffin sections is commonly thought to be useless because of the poor preservation of many lineage-related markers. The recent development of antibodies against fixative-resistant epitopes and of new antigen retrieval techniques, however, has expanded the possi...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:

    authors: Pileri SA,Ascani S,Milani M,Visani G,Piccioli M,Orcioni GF,Poggi S,Sabattini E,Santini D,Falini B

    更新日期:1999-05-01 00:00:00

  • Cytoskeleton organization of normal and neoplastic lymphocytes and lymphoid cell lines of T and B origin.

    abstract::An anomalous organization of the cytoskeleton has been described in lymphocytes from chronic lymphatic leukaemia and in only few cell lines. We have now studied normal and neoplastic lymphocytes and lymphoid cell lines of both T and B lineage in order to detect morphological differences in the expression of microfilam...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1988.tb04226.x

    authors: Zauli D,Gobbi M,Crespi C,Tazzari PL,Miserocchi F,Tassinari A

    更新日期:1988-04-01 00:00:00

  • Otlertuzumab (TRU-016), an anti-CD37 monospecific ADAPTIR(™) therapeutic protein, for relapsed or refractory NHL patients.

    abstract::CD37 is cell surface tetraspanin present on normal and malignant B cells. Otlertuzumab (TRU-016) is a novel humanized anti-CD37 protein therapeutic. Patients with relapsed or refractory follicular non-Hodgkin lymphoma (FL), mantle cell lymphoma (MCL), or Waldenström's macroglobulinaemia (WM) received otlertuzumab at 2...

    journal_title:British journal of haematology

    pub_type: 杂志文章,多中心研究

    doi:10.1111/bjh.13099

    authors: Pagel JM,Spurgeon SE,Byrd JC,Awan FT,Flinn IW,Lanasa MC,Eisenfeld AJ,Stromatt SC,Gopal AK

    更新日期:2015-01-01 00:00:00

  • Acute tumour lysis syndrome: a case in AL amyloidosis.

    abstract::Tumour lysis syndrome (TLS) in plasma cell dyscrasias is extremely rare. TLS has been described in eight cases of multiple myeloma undergoing high-dose therapy with autologous stem cell transplant (ASCT). Recently, clinical trials of intensive chemotherapy followed by autologous or allogeneic stem cell support has bee...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1999.01719.x

    authors: Akasheh MS,Chang CP,Vesole DH

    更新日期:1999-11-01 00:00:00

  • A point mutation (Arg271-->Cys) of a homozygote for dysfunctional prothrombin, prothrombin Obihiro, which has a region of high sequence variability.

    abstract::The molecular defect of a congenitally dysfunctional form of prothrombin, prothrombin Obihiro, was identified in a patient with a severe bleeding tendency. He showed reduced fibrinogen clotting activity, despite a normal prothrombin antigen level. Nucleotide sequencing of amplified DNA revealed a C-->T change at nucle...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1995.tb05601.x

    authors: Miyata T,Zheng YZ,Kato A,Kato H

    更新日期:1995-07-01 00:00:00

  • Variations in globin synthesis in delta-beta-thalassaemia.

    abstract::Peripheral blood globin synthesis studies were done in 11 patients with delta beta-thalassaemia trait, Hb S-delta beta-thalassaemia or delta beta/betao-thalassaemia from two black and two Caucasian families. All patients had elevated Hb F and normal or decreased Hb A2 levels and 10 had family studies confirming the di...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1978.tb07103.x

    authors: Kinney TR,Friedman S,Cifuentes E,Kim HC,Schwartz E

    更新日期:1978-01-01 00:00:00

  • Immunotoxin BL22 induces apoptosis in mantle cell lymphoma (MCL) cells dependent on Bcl-2 expression.

    abstract::Mantle cell lymphoma (MCL) is an incurable mature B cell proliferation, combining the unfavourable clinical features of aggressive and indolent lymphomas. The blastic variant of MCL has an even worse prognosis and new treatment options are clearly needed. We analysed the effects of BL22, an immunotoxin composed of the...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2009.07939.x

    authors: Bogner C,Dechow T,Ringshausen I,Wagner M,Oelsner M,Lutzny G,Licht T,Peschel C,Pastan I,Kreitman RJ,Decker T

    更新日期:2010-01-01 00:00:00

  • Is the beta thalassaemia trait of clinical importance?

    abstract::Although the beta thalassaemia trait affects millions of people worldwide, there have been no controlled studies to determine whether it is associated with any clinical disability or abnormal physical signs. To address this question, 402 individuals were studied: 217 with beta thalassaemia trait, of whom 154 were awar...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2008.07071.x

    authors: Premawardhena A,Arambepola M,Katugaha N,Weatherall DJ

    更新日期:2008-05-01 00:00:00

  • Inactivation of the retinoblastoma gene appears to be very uncommon in myelodysplastic syndromes.

    abstract::Rearrangements of the retinoblastoma (RB) gene have been reported in a few cases of myelodysplastic syndromes (MDS). In addition, low or absent expression of the RB protein is found in 20-30% of cases of acute myeloid leukaemias (AML), particularly in AML with a monocytic component (M4 or M5). We performed Southern bl...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1994.tb04871.x

    authors: Preudhomme C,Vachee A,Lepelley P,Vanrumbeke M,Zandecki M,Quesnel B,Cosson A,Fenaux P

    更新日期:1994-05-01 00:00:00

  • Continuous complete remission in adult patients with acute lymphocytic leukaemia at a median observation of 12 years after autologous bone marrow transplantation.

    abstract::We report our long-term experience with autologous bone marrow transplantation (ABMT) for 32 adult patients with acute lymphocytic leukaemia (ALL) in second or later remission (CR), or in first CR but with high-risk. Bone marrow was purged with mafosfamide (n = 25) or with immunomagnetic beads and monoclonal antibodie...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2001.02634.x

    authors: Abdallah A,Egerer G,Goldschmidt H,Wannenmacher M,Körbling M,Ho AD

    更新日期:2001-03-01 00:00:00

  • Plasma microparticles and vascular disorders.

    abstract::Microparticles are circulating, phospholipid rich, submicron particles released from the membranes of endothelial cells, platelets, leucocytes and erythrocytes. Investigation into their biological activity has revealed diverse actions in coagulation, cell signalling and cellular interactions. These actions are mediate...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2007.06514.x

    authors: Lynch SF,Ludlam CA

    更新日期:2007-04-01 00:00:00

  • A molecular study of the t(4;14) in multiple myeloma.

    abstract::The t(4;14) translocation is found in approximately 10% of myeloma patients and results in the deregulation of at least two genes, MMSET and fibroblast growth factor receptor 3 (FGFR3), with the formation of a fusion product between MMSET and the immunoglobulin heavy chain (IgH) locus and overexpression of FGFR3. We h...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03618.x

    authors: Sibley K,Fenton JA,Dring AM,Ashcroft AJ,Rawstron AC,Morgan GJ

    更新日期:2002-08-01 00:00:00

  • Podocalyxin in human haematopoietic cells.

    abstract::Podocalyxin-like protein (PCLP) is a sialomucin-type membrane protein structurally related to CD34 and endoglycan. It was first described in glomerular podocytes and endothelial cells. In mice, PCLP is present in haemangioblasts, and in both chicken and mice it is a marker of early haematopoietic stem cells and lineag...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2004.04840.x

    authors: Kerosuo L,Juvonen E,Alitalo R,Gylling M,Kerjaschki D,Miettinen A

    更新日期:2004-03-01 00:00:00

  • Locus assignment of human alpha globin mutations by selective amplification and direct sequencing.

    abstract::We describe a simple approach for molecular characterization and locus assignment of structural mutants by direct sequencing of enzymatically amplified DNA selective to alpha 1 and alpha 2 globin gene regions. Nucleotide substitution of two structural variants (Stanleyville II alpha 2(78Lys) and J Mexico alpha 2(54Glu...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1990.tb07884.x

    authors: Dodé C,Rochette J,Krishnamoorthy R

    更新日期:1990-10-01 00:00:00

  • Simultaneous inheritance of mutant isoenzymes of erythrocyte pyruvate kinase associated with chronic haemolytic anaemia.

    abstract::The heterogeneity of pyruvate kinase (PK) deficiency associated with hereditary haemolytic anaemia is emphasized by studies of a kindred harbouring two distinct mutant forms of this enzyme, both of which were kinetically defective with markedly decreased affinities for the substrate, phosphoenolypyruvate. The two isoe...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1976.tb00174.x

    authors: Pagila DE,Gray GR,Growe GH,Valentine WN

    更新日期:1976-09-01 00:00:00

  • A novel polymorphism in intron 1a of the human factor VII gene (G73A): study of a healthy Italian population and of 190 young survivors of myocardial infarction.

    abstract::We have identified a novel polymorphism located in intron 1a of the human factor VII gene, caused by the nucleotide change G to A at position + 73. In a population of 128 healthy individuals from northern Italy, the variant A73 allele had a frequency of 0.21, whereas the frequency of the previously reported 10 bp inse...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2000.01833.x

    authors: Peyvandi F,Mannucci PM,Bucciarelli P,Zeinali S,Akhavan S,Sacchi E,Merlini PA,Perry DJ

    更新日期:2000-02-01 00:00:00

  • Effect of VDR polymorphisms on growth and bone mineral density in homozygous beta thalassaemia.

    abstract::We examined the effect of vitamin D receptor (VDR) polymorphisms at exon 2 (FokI) and intron 8 (BsmI) on the stature and bone mineral density at femoral neck (FBMD) and lumbar spine (LBMD) in 108 prepubertal and pubertal homozygous beta thalassaemic patients, regularly treated. We found significantly shorter stature a...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03426.x

    authors: Ferrara M,Matarese SM,Francese M,Borrelli B,Coppola A,Coppola L,Esposito L

    更新日期:2002-05-01 00:00:00

  • Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases.

    abstract::We retrospectively analysed 71 cases of Unicentric Castleman disease, a rare, usually asymptomatic, benign lymphoproliferative disorder presenting as a unique nodal mass. Although surgery is considered as the gold standard therapy, only 38 patients (54%) underwent initial surgical resection and 95% were cured. An addi...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.15921

    authors: Boutboul D,Fadlallah J,Chawki S,Fieschi C,Malphettes M,Dossier A,Gérard L,Mordant P,Meignin V,Oksenhendler E,Galicier L

    更新日期:2019-07-01 00:00:00

  • Unbalanced X-chromosome inactivation in haemopoietic cells from normal women.

    abstract::We studied X-chromosome inactivation patterns in blood cells from normal females in three age groups: neonates (umbilical cord blood), 25-32 years old (young women group) and >75 years old (elderly women). Using PCR, the differential allele methylation status was evaluated on active and inactive X chromosomes at the h...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1998.00867.x

    authors: Tonon L,Bergamaschi G,Dellavecchia C,Rosti V,Lucotti C,Malabarba L,Novella A,Vercesi E,Frassoni F,Cazzola M

    更新日期:1998-09-01 00:00:00

  • Specific binding between human neutrophils and heparin.

    abstract::Heparin binding on polymorphonuclear leucocytes (PMNL) was characterized. Heparin binding was specific, rapid, saturable and reversible. One single class of heparin binding sites was found with a dissociation constant of 1.22 mumol/l and 7.7 x 10(6) sites per PMNL. The binding was independent of the anticoagulant acti...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1992.tb08176.x

    authors: Leculier C,Benzerara O,Couprie N,Francina A,Lasne Y,Archimbaud E,Fiere D

    更新日期:1992-05-01 00:00:00

  • The effect of urea on sickling.

    abstract::The effect of urea on the oxygen affinity of sickle cells and normal cells was studied up to a concentration of about 1.0 M. Besides the increase in oxygen affinity found in both normal and sickle cells there was a further increase found only in the sickle cells. This specific increase was caused by the direct inhibit...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1975.tb00513.x

    authors: May A,Huehns ER

    更新日期:1975-05-01 00:00:00

  • Challenges in the management of patients with systemic light chain (AL) amyloidosis during the COVID-19 pandemic.

    abstract::The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated coronavirus disease 2019 (COVID-19) is primarily manifested as a respiratory tract infection, but may affect and cause complications in multiple organ systems (cardiovascular, gastrointestinal, kidneys, haematopoietic and immune systems), whil...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/bjh.16898

    authors: Kastritis E,Wechalekar A,Schönland S,Sanchorawala V,Merlini G,Palladini G,Minnema M,Roussel M,Jaccard A,Hegenbart U,Kumar S,Cibeira MT,Blade J,Dimopoulos MA

    更新日期:2020-08-01 00:00:00

  • High prevalence of a mutation in the factor V gene within the U.K. population: relationship to activated protein C resistance and familial thrombosis.

    abstract::Recent findings have indicated the importance of factor V (FV) in causing resistance to activated protein C (APC) in a high proportion of patients with venous thrombosis. This prompted us to investigate whether resistance could be due to defective inactivation of FVa by APC. Consequently, we amplified a 3.2 kb fragmen...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1994.tb05005.x

    authors: Beauchamp NJ,Daly ME,Hampton KK,Cooper PC,Preston FE,Peake IR

    更新日期:1994-09-01 00:00:00