Determinants of haemoglobin level in sickle cell-haemoglobin C disease.

Abstract:

:The determinants of steady state haemoglobin levels in sickle cell-haemoglobin C (SC) disease were investigated by measuring routine haematological and biochemical indices, red cell survival, oxygen affinity, pitted erythrocytes, and red cell and plasma volumes in 31 adult patients (15 male; 16 female). Red cell survival was shortened in all subjects, and was positively correlated with haemoglobin level. However, many haemoglobin values were within the normal range, especially in male subjects. Palpable splenomegaly, which occured in 53% of patients, did not appear to affect haemoglobin level, red cell survival, plasma volume, or red cell volume, but was associated with lower platelet counts and decreased pitted red cells. Sex related differences were found in total haemoglobin, packed cell volume, conductivity cell volume, red cell count, and in the blood volume measurements. Red cell, plasma and total blood volumes in patients varied with weight and cube of height in manner observed in normal subjects, although red cell volumes were lower and plasma volumes were greater than in normal subjects of given height and weight. Anaemia in SC disease is related to the haemolytic rate but the major determinant appears to be an inappropriate increase in plasma volume.

journal_name

Br J Haematol

authors

Bannerman RM,Serjeant B,Seakins M,England JM,Serjeant GR

doi

10.1111/j.1365-2141.1979.tb03718.x

subject

Has Abstract

pub_date

1979-09-01 00:00:00

pages

49-56

issue

1

eissn

0007-1048

issn

1365-2141

journal_volume

43

pub_type

杂志文章
  • Role of granulocyte/neutrophil transfusions for haematology/oncology patients in the modern era.

    abstract::Infections continue to be a serious problem for severely neutropenic oncology and haematopoietic progenitor cell (HPC) transplant patients. Although it is now possible to collect much larger numbers of neutrophils (PMNs) from donors stimulated with granulocyte colony-stimulating factor + corticosteroids, the efficacy ...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2012.09190.x

    authors: Strauss RG

    更新日期:2012-08-01 00:00:00

  • Enemy of the (immunosuppressed) state: an update on the pathogenesis of Aspergillus fumigatus infection.

    abstract::Aspergillus fumigatus is an opportunistic filamentous fungus that is currently the most frequent cause of invasive fungal disease in immunosuppressed individuals. Recent advances in our understanding of the pathogenesis of invasive aspergillosis have highlighted the multifactorial nature of A. fumigatus virulence and ...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2010.08283.x

    authors: Ben-Ami R,Lewis RE,Kontoyiannis DP

    更新日期:2010-08-01 00:00:00

  • Targeted mutation screening of 292 candidate genes in 38 children with inborn haematological cytopenias efficiently identifies novel disease-causing mutations.

    abstract::Establishing a precise diagnosis is essential in inborn haematological cytopenias to enable appropriate treatment decisions and avoid secondary organ damage. However, both diversity and phenotypic overlap of distinct disease entities may make the identification of underlying genetic aetiologies by classical Sanger seq...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.15389

    authors: Kager L,Jimenez Heredia R,Hirschmugl T,Dmytrus J,Krolo A,Müller H,Bock C,Zeitlhofer P,Dworzak M,Mann G,Holter W,Haas O,Boztug K

    更新日期:2018-07-01 00:00:00

  • Incidence, risk factors and management of osteonecrosis of the jaw in patients with multiple myeloma: a single-centre experience in 303 patients.

    abstract::The incidence, characteristics and risk factors for the development of osteonecrosis of the jaw (ONJ) were evaluated among 303 myeloma patients. Only patients who received bisphosphonates developed ONJ (28/254; 11%). Zoledronic acid produced 9.5-fold greater risk for developing ONJ than pamidronate alone (P = 0.042) a...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2006.06230.x

    authors: Zervas K,Verrou E,Teleioudis Z,Vahtsevanos K,Banti A,Mihou D,Krikelis D,Terpos E

    更新日期:2006-09-01 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

  • Different membrane expression of CD11b and CD14 on blood neutrophils following in vivo administration of myeloid growth factors.

    abstract::During the administration of recombinant human granulocyte colony-stimulating factor (rhG-CSF) or granulocyte-macrophage CSF (rhGM-CSF) we studied the early and late changes of membrane antigen density on neutrophils. RhG-CSF and rhGM-CSF both caused an early transient reduction in blood neutrophilic granulocyte-conce...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1993.tb08644.x

    authors: Hansen PB,Kjaersgaard E,Johnsen HE,Gram J,Pedersen M,Nikolajsen K,Hansen NE

    更新日期:1993-09-01 00:00:00

  • Significant amount of hepatocyte growth factor detected in blood and bone marrow plasma of leukaemia patients.

    abstract::Hepatocyte growth factor (HGF) has been known as a versatile functional molecule, and as being involved in the colony formation of haemopoietic progenitor cells. Clinically, an elevated HGF level in the blood has been associated with liver diseases such as fulminant hepatic failure and acute hepatitis. We have found a...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1994.tb08330.x

    authors: Nakamura S,Gohda E,Matsuo Y,Yamamoto I,Minowada J

    更新日期:1994-07-01 00:00:00

  • Preliminary experience of allogeneic stem cell transplantation for lymphoproliferative disorders using BEAM-CAMPATH conditioning: an effective regimen with low procedure-related toxicity.

    abstract::Autologous transplantation has an established role in the treatment of lymphoproliferative disorders, but allogeneic transplantation remains controversial. In an attempt to reduce the high procedure-related mortality reported with allografting in lymphoma, we have used BEAM (BCNU, etoposide, cytarabine and melphalan),...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2000.01879.x

    authors: Cull GM,Haynes AP,Byrne JL,Carter GI,Miflin G,Rebello P,Hale G,Waldmann H,Russell NH

    更新日期:2000-03-01 00:00:00

  • Impaired immune responses to herpesviruses and microbial ligands in patients with MonoMAC.

    abstract::MonoMAC is a complex primary immunodeficiency caused by mutations in the myeloid transcription factor GATA2, characterized by multilineage cytopenia with malignant complications and severe infections, including mycobacteria and herpesviruses. We describe the clinical presentation, genetics and antiviral inflammatory r...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.15947

    authors: Mardahl M,Jørgensen SE,Schneider A,Raaschou-Jensen K,Holm M,Veirum J,Kristensen TK,Johansen IS,Christiansen M,Assing K,Mogensen TH

    更新日期:2019-08-01 00:00:00

  • A variant of the EPB3 gene of the anti-Lepore type in hereditary spherocytosis.

    abstract::The EPB3 gene encodes band 3 (anion exchanger 1) of the red cell membrane. A subset of hereditary spherocytosis (HS) is associated with EPB3 gene mutations and band 3 deficiency. We report a large Italian family in which 10 of the 27 members investigated displayed an autosomal dominant HS. SDS-PAGE revealed a reductio...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1997.1983013.x

    authors: Bianchi P,Zanella A,Alloisio N,Barosi G,Bredi E,Pelissero G,Zappa M,Vercellati C,Baronciani L,Delaunay J,Sirchia G

    更新日期:1997-08-01 00:00:00

  • Immune reconstitution after haematopoietic cell transplantation in children: immunophenotype analysis with regard to factors affecting the speed of recovery.

    abstract::Immune reconstitution was studied prospectively in 66 children who underwent 77 haematopoietic cell transplantations (HCT): 46 autologous HCTs in 39 patients and 31 allogeneic HCTs in 27 patients. We studied the dynamic analysis of immune recovery with regard to potential factors affecting its speed, including age, ty...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03560.x

    authors: Kalwak K,Gorczyńska E,Toporski J,Turkiewicz D,Slociak M,Ussowicz M,Latos-Grazyńska E,Król M,Boguslawska-Jaworska J,Chybicka A

    更新日期:2002-07-01 00:00:00

  • Is refractory anaemia with ring sideroblasts and thrombocytosis (RARS-T) a necessary or useful diagnostic category?

    abstract::Both the 2001 World Health Organisation (WHO) classification of haematopoietic neoplasms and the 2008 WHO classification revision include a distinctive diagnostic category, refractory anaemia with ring sideroblasts and thrombocytosis (RARS-T), to describe those rare patients who have both >or=15% ring sideroblasts and...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2008.07526.x

    authors: Wardrop D,Steensma DP

    更新日期:2009-03-01 00:00:00

  • Refractory anaemia with excess of blasts (RAEB): analysis of reclassification according to the WHO proposals.

    abstract::The French-American-British (FAB) classification assigns patients with myelodysplastic syndromes to the category of refractory anaemia with excess blasts (RAEB) if they have a medullary blast count of 5-20%, and/or a peripheral blast count of 2-5%. The new World Health Organization (WHO) classification subdivides RAEB...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2005.05853.x

    authors: Germing U,Strupp C,Kuendgen A,Aivado M,Giagounidis A,Hildebrandt B,Aul C,Haas R,Gattermann N

    更新日期:2006-01-01 00:00:00

  • Stimulation of human haemopoietic cells by colony stimulating factors: adherent cell dependent colony stimulating activity in human serum.

    abstract::The number of granulocyte-macrophage clones formed in agar culture of bone marrow is dependent on levels of colony stimulating activity (CSA) a proposed in vivo haemopoietic regulator. A dose-response relationship for stimulation of human haemopoietic cells by CSA is demonstrated, which could be explained by threshold...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1977.tb00626.x

    authors: Francis GE,Berney JJ,Hoffebrand AV

    更新日期:1977-04-01 00:00:00

  • Identification of patients with smouldering multiple myeloma at ultra-high risk of progression using serum parameters: the Czech Myeloma Group model.

    abstract::Smouldering multiple myeloma (SMM) presents without MM defining symptoms. We aimed to identify patients with SMM with an 80% risk of progression within 2 years using only serum parameters. In total, 527 patients with SMM were included and divided into a training group (287 patients from the Czech Myeloma Group [CMG]) ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.16572

    authors: Hájek R,Sandecka V,Špička I,Raab M,Goldschmidt H,Beck S,Minařík J,Pavlíček P,Radocha J,Heindorfer A,Jelínek T,Stejskal L,Brožová L,Ševčíková S,Straub J,Pika T,Pour L,Maisnar V,Seckinger A,Hose D

    更新日期:2020-07-01 00:00:00

  • Induction of antitumour immunity using survivin peptide-pulsed dendritic cells in a murine lymphoma model.

    abstract::Survivin is overexpressed in several types of haematological malignancies making it an attractive target for therapeutic cytotoxic T-lymphocyte responses. Here, we identify two peptide epitopes derived from the murine survivin protein and demonstrate that Balb/c mice treated with syngeneic dendritic cells pulsed with ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2003.04535.x

    authors: Siegel S,Wagner A,Schmitz N,Zeis M

    更新日期:2003-09-01 00:00:00

  • The fundamental contribution of William Bate Hardy to shape the concept of mast cell heterogeneity.

    abstract::This review article acknowledges the pioneering contribution of William Bate Hardy in shaping the concept of mast cell heterogeneity. In two outstanding papers, published in 1894 and 1895, he focussed on the 'wandering cells' (the modern leucocytes) in different mammalian species and distinguished two types of granula...

    journal_title:British journal of haematology

    pub_type: 传,历史文章,杂志文章

    doi:10.1111/j.1365-2141.2009.07938.x

    authors: Crivellato E,Ribatti D

    更新日期:2010-07-01 00:00:00

  • Post-haematopoietic cell transplantation outcomes: why ST2 became a 'golden nugget' biomarker.

    abstract::Immunotherapies have emerged as highly promising approaches to treat cancer patients. Allogeneic haematopoietic cell transplantation (HCT) is the most validated tumour immunotherapy available to date but its clinical efficacy is limited by toxicities, such as graft-versus-host disease (GVHD) and treatment resistance l...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.16497

    authors: Paczesny S

    更新日期:2020-02-10 00:00:00

  • Four new cases of stomatin-deficient hereditary stomatocytosis syndrome: association of the stomatin-deficient cryohydrocytosis variant with neurological dysfunction.

    abstract::This report concerns congenitally Na(+)-K(+) leaky red cells of the 'hereditary stomatocytosis' class. Three new isolated cases and one new pedigree are described, and one previously reported case is expanded. In all cases, Western blotting of red cell membranes revealed a deficiency in the 32 kDa membrane protein, st...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2004.04965.x

    authors: Fricke B,Jarvis HG,Reid CD,Aguilar-Martinez P,Robert A,Quittet P,Chetty M,Pizzey A,Cynober T,Lande WF,Mentzer WC,Düring M,Winter S,Delaunay J,Stewart GW

    更新日期:2004-06-01 00:00:00

  • Second transplantation with CD34+ bone marrow cells selected from a two-loci HLA-mismatched sibling for a patient with chronic myeloid leukaemia.

    abstract::A 43-year-old man with chronic myeloid leukaemia underwent a second transplant with CD34+ bone marrow cells selected from his two-loci HLA-mismatched sibling after rejection of the first graft from an HLA-matched unrelated donor. By immunomagnetic positive selection, CD34+ marrow cells at 0.95 x 10(6)/kg with 97% puri...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1996.d01-1763.x

    authors: Fujimori Y,Kanamaru A,Hashimoto N,Okamoto T,Okada M,Kawaguchi K,Mori A,Saheki K,Takatsuka H,Wada H,Takemoto Y,Kohsaki M,Imai N,Kakishita E,Nagai K

    更新日期:1996-07-01 00:00:00

  • Unrelated donor bone marrow transplantation for children with relapsed acute lymphoblastic leukaemia in second complete remission.

    abstract::Allogeneic sibling bone marrow transplantation (BMT) is the recommended treatment for relapsed childhood acute lymphoblastic leukaemia (ALL), but appropriate donors are only available in 30% of cases. Unfortunately, BMT from unrelated donors (UD) has been associated with high rates of severe graft-versus-host disease ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1996.d01-1834.x

    authors: Oakhill A,Pamphilon DH,Potter MN,Steward CG,Goodman S,Green A,Goulden P,Goulden NJ,Hale G,Waldmann H,Cornish JM

    更新日期:1996-09-01 00:00:00

  • Role of Epstein-Barr virus and soluble CD21 in persistent polyclonal B-cell lymphocytosis.

    abstract::The expression of EBV proteins and immunological properties were studied in the first stable cell line (SM) established from a patient presenting with persistent polyclonal B-cell lymphocytosis (PPBL). SM cells which represent a small population of EBV-positive atypical cells found in the peripheral blood of the patie...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1995.tb05580.x

    authors: Larcher C,Fend F,Mitterer M,Prang N,Schwarzmann F,Huemer HP

    更新日期:1995-07-01 00:00:00

  • Characterization of a new alpha zero thalassaemia defect in the South African population.

    abstract::A new alpha thalassemia defect has been detected in the South African population. Restriction mapping of the alpha globin gene cluster in affected individuals has established that the defect is associated with the removal of 22.8-23.7 kb of DNA, including the psi zeta 1, psi alpha 1, psi alpha 2, alpha 2 and alpha 1 g...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1987.tb01341.x

    authors: Vandenplas S,Higgs DR,Nicholls RD,Bester AJ,Mathew CG

    更新日期:1987-08-01 00:00:00

  • Production of immunoreactive calcitonin by myeloid leukaemia cells.

    abstract::Raised plasma levels of immunoreactive human calcitonin (i-HCT) have been found in patients with chronic granulocytic leukaemia (CGL) in chronic phase and myeloblastic transformation and in patients with acute myeloid leukaemia at presentation and in relapse. In CGL levels were significantly higher in myeloblastic tra...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1982.tb01911.x

    authors: Foa R,Oscier DG,Hillyard CJ,Incarbone E,McIntyre I,Goldman JM

    更新日期:1982-02-01 00:00:00

  • Ber-H2 (anti-CD30)-saporin immunotoxin: a new tool for the treatment of Hodgkin's disease and CD30+ lymphoma: in vitro evaluation.

    abstract::An immunotoxin containing an anti-CD30 monoclonal antibody (Ber-H2) and saporin, a ribosome-inactivating protein type 1, is described. It specifically inhibits protein synthesis by Hodgkin derived target cell lines with a very high efficiency (IC50 ranging from 5 x 10(-12) M to 5 x 10(-14) M, as saporin), while irrele...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1992.tb08208.x

    authors: Tazzari PL,Bolognesi A,de Totero D,Falini B,Lemoli RM,Soria MR,Pileri S,Gobbi M,Stein H,Flenghi L

    更新日期:1992-06-01 00:00:00

  • The anti-mitotic agents PTC-028 and PTC596 display potent activity in pre-clinical models of multiple myeloma but challenge the role of BMI-1 as an essential tumour gene.

    abstract::Future progress in the treatment of multiple myeloma (MM) requires both the characterisation of key drivers of the disease and novel, innovative approaches to tackle these vulnerabilities. The present study focussed on the pre-clinical evaluation of a novel drug class, BMI-1 modulators, in MM. We demonstrate potent ac...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.16595

    authors: Bolomsky A,Muller J,Stangelberger K,Lejeune M,Duray E,Breid H,Vrancken L,Pfeiffer C,Hübl W,Willheim M,Weetall M,Branstrom A,Zojer N,Caers J,Ludwig H

    更新日期:2020-09-01 00:00:00

  • Probable in vivo induction of differentiation by retinoic acid of promyelocytes in acute promyelocytic leukaemia.

    abstract::A 30-year-old woman was diagnosed as having an acute promyelocytic leukaemia in September 1981. Chemotherapeutic courses of daunomycin, ara-C, thioguanine and prednisolone were administered, resulting in a complete remission. A relapse occurred in January 1982, and chemotherapy did not lead to a second complete remiss...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1984.tb02910.x

    authors: Nilsson B

    更新日期:1984-07-01 00:00:00

  • Depression of the platelet count after inoculation of mice with L1210 or L5178Y cells.

    abstract::The inoculation of L1210 or L5178Y leukaemia cells decreased the platelet count of the recipient mouse before extensive infiltration of marrow was expected by either tumour line. The decrease was more pronounced after intravenous inoculation of L1210 leukaemia than after intraperitoneal inoculation. Inoculation of L51...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1977.tb00608.x

    authors: Hacker M,Roberts D,Jackson C

    更新日期:1977-03-01 00:00:00

  • Functional granulocyte/macrophage colony stimulating factor receptor is constitutively expressed on neoplastic plasma cells and mediates tumour cell longevity.

    abstract::It has been shown that granulocyte/macrophage colony stimulating factor (GM-CSF) is able to support myeloma cell propagation in cooperation with interleukin (IL)-6, the major growth factor for malignant plasma cells, although the biological mechanisms involved remain unknown. Therefore we investigated (i) the expressi...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1998.00880.x

    authors: Villunger A,Egle A,Kos M,Egle D,Tinhofer I,Henn T,Uberall F,Maly K,Greil R

    更新日期:1998-09-01 00:00:00

  • In vitro differentiation of myeloid progenitor cells in patients with eosinophilia.

    abstract::Colony-forming cells of the granulocytic/macrophage ( CFCGM ) and eosinophilic ( CFCEo ) series were grown from bone marrow and/or peripheral blood of 20 patients with persistent eosinophilia mainly due to helminthic diseases and of 17 patients without eosinophilia. The semi-solid culture technique of haemopoietic cel...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:

    authors: Kern P,Dietrich M

    更新日期:1984-05-01 00:00:00