Association between hyperflexibility of the thumb and an unexplained bleeding tendency: is it a rule of thumb?

Abstract:

:A bleeding tendency manifested by petechiae and ecchymoses is one of the most common causes for referral of patients to haematology clinics. Vessel wall pathology is not usually considered to be a cause for deranged haemostasis, although coexistence of increased capillary fragility and joint hypermobility have been reported. We determined the frequency of thumb hyperextensibility and scored the findings in a series of 44 patients referred because of ecchymoses and petechiae, as well as 261 control children and their mothers. All 44 patients had normal coagulation studies. Thumb flexibility score was +4 in 30 patients, +3 in eight patients, +2 in five patients and +1 in one of the index patients. In the control group, only one of 261 had a +4, and three had a +3 score, and two of 260 mothers had a +4 score. Ecchymoses were not observed in any of these subjects, nor in the +1 patients. Based on clinical presentation and normal coagulation studies, we suggest that our patients had an underlying subtype of Ehlers-Danlos syndrome. In view of the dramatically high occurrence of thumb hyperextensibility in patients with unexplained mild bleeding tendency, costly haemostatic and coagulation studies on such patients may not be necessary.

journal_name

Br J Haematol

authors

Kaplinsky C,Kenet G,Seligsohn U,Rechavi G

doi

10.1046/j.1365-2141.1998.00697.x

subject

Has Abstract

pub_date

1998-05-01 00:00:00

pages

260-3

issue

2

eissn

0007-1048

issn

1365-2141

journal_volume

101

pub_type

杂志文章
  • Autologous bone marrow transplantation with 4-hydroperoxycyclophosphamide purging for acute myeloid leukaemia beyond first remission: a 10-year experience.

    abstract::Between January 1987 and January 1997, 69 eligible patients with acute myeloid leukaemia (AML) in either second (CR2) or third (CR3) complete remission (CR2 = 60, CR3 = 9) underwent 4-hydroperoxycyclophosphamide-purged autologous bone marrow transplantation (BMT) at the Johns Hopkins Oncology Center. The patients' med...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03530.x

    authors: Smith BD,Jones RJ,Lee SM,Piantadosi S,Vala MS,Fuller D,Gore SD,Noga SJ,O'Donnell PV,Braine H,Vogelsang GB,Fuchs EJ,Flinn IW,Brodsky RA,Ambinder RF,Miller CB

    更新日期:2002-06-01 00:00:00

  • Dendritic cells in acute promyelocytic leukaemia.

    abstract::Dendritic cell (DC) differentiation was investigated in samples from two acute promyelocytic leukaemia (APL) patients with classic translocation t(15;17)(q22;q21). After 18 d of culture in the presence of granulocyte-macrophage colony-stimulating factor, interleukin 4 and tumour necrosis factor alpha, 10-15% of pathol...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2001.03023.x

    authors: Rigolin GM,Della Porta M,Bigoni R,Tieghi A,Cuneo A,Castoldi G

    更新日期:2001-09-01 00:00:00

  • Absence of mutations in the HoxA10, HoxA11 and HoxD11 nucleotide coding sequences in thrombocytopenia with absent radius syndrome.

    abstract::Recent studies have suggested the HoxA10, HoxA11 and HoxD11 homeobox genes as candidate loci for the thrombocytopenia with absent radius (TAR) syndrome. For example, targeted disruptions of these Hox genes result in abnormal development of the mouse radius, while overexpression of HoxA10 stimulates mouse megakaryocyte...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03263.x

    authors: Fleischman RA,Letestu R,Mi X,Stevens D,Winters J,Debili N,Vainchenker W

    更新日期:2002-02-01 00:00:00

  • Combination of cytokines: current status and future prospects.

    abstract::Clinical trials with individual cytokines and extensive in vitro studies have provided the basis for the in vivo use of these molecules in combination. Animal models, with haemopoietic growth factors as well as preliminary studies in humans--as shown by our studies with the sequential use of IL-3 and GM-CSF in patient...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.1991.tb08131.x

    authors: Kanz L,Brugger W,Bross K,Mertelsmann R

    更新日期:1991-10-01 00:00:00

  • Effect of myeloablative bone marrow transplantation on growth in children with sickle cell anaemia: results of the multicenter study of haematopoietic cell transplantation for sickle cell anaemia.

    abstract::Although haematopoietic cell transplantation (HCT) is curative for sickle cell anaemia (SCA), concerns about its short- and long-term toxicities limit its application. A potential toxicity is an adverse effect on growth. To identify an HCT growth effect, serial height and weight measurements from 53 children and adole...

    journal_title:British journal of haematology

    pub_type: 杂志文章,多中心研究

    doi:10.1111/j.1365-2141.2006.06486.x

    authors: Eggleston B,Patience M,Edwards S,Adamkiewicz T,Buchanan GR,Davies SC,Dickerhoff R,Donfield S,Feig SA,Giller RH,Haight A,Horan J,Hsu LL,Kamani N,Lane P,Levine JE,Margolis D,Moore TB,Ohene-Frempong K,Redding-Lallinger

    更新日期:2007-02-01 00:00:00

  • Production of colony stimulating activity in mixed mononuclear cell culture.

    abstract::Culture medium was harvested after co-incubation of mononuclear cells collected and pooled from the peripheral blood of two different normal donors and was tested for colony-stimulating activity (CSA) in agar culture. With bone marrow from normal donors or peripheral blood from patients with chronic granulocytic leuka...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1980.tb07144.x

    authors: Hellmann A,Th'ng KH,Goldman JM

    更新日期:1980-06-01 00:00:00

  • Review of the relevance of aberrant antigen expression by flow cytometry in myeloid neoplasms.

    abstract::This article reviews the use of aberrant antigen expression detected by flow cytometry in the diagnosis and clinical handling of acute myeloid leukaemia (AML) and the myelodysplastic syndromes (MDS). Such aberrancies offer a valuable tool for the proper classification of these myeloid malignancies according the World ...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2011.08595.x

    authors: Ossenkoppele GJ,van de Loosdrecht AA,Schuurhuis GJ

    更新日期:2011-05-01 00:00:00

  • Glyburide-induced acute haemolysis in a G6PD-deficient patient with NIDDM.

    abstract::In a 61-year-old man with glucose-6-phosphate dehydrogenase (G6PD) deficiency and poorly controlled non-insulin-dependent diabetes mellitus, an episode of acute haemolysis occurred after the administration of glyburide (glibenclamide). Erythrocyte fragmentation, with haemoglobin condensation zones next to clear zones,...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1996.275810.x

    authors: Meloni G,Meloni T

    更新日期:1996-01-01 00:00:00

  • The role of apoptosis in megakaryocytes and platelets.

    abstract::The role of apoptotic pathways in the development and function of the megakaryocyte lineage has generated renewed interest in recent years. This has been driven by the advent of BH3 mimetic drugs that target BCL2 family proteins to induce apoptosis in tumour cells: agents such as ABT-263 (navitoclax, which targets BCL...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/bjh.12757

    authors: Kile BT

    更新日期:2014-04-01 00:00:00

  • Minimal residual disease after allogeneic bone marrow transplantation for chronic myeloid leukaemia in first chronic phase: correlations with acute graft-versus-host disease and relapse.

    abstract::We have studied 61 patients who underwent allogeneic bone marrow transplantation (BMT) for chronic myeloid leukaemia (CML) in first chronic phase. Minimal residual disease was detected by the amplification of the leukaemia-specific BCR-ABL fusion mRNA with the polymerase chain reaction (PCR) using a highly sensitive n...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1993.tb03026.x

    authors: Cross NC,Hughes TP,Feng L,O'Shea P,Bungey J,Marks DI,Ferrant A,Martiat P,Goldman JM

    更新日期:1993-05-01 00:00:00

  • Bone marrow biopsy in myelodysplastic syndromes: morphological characteristics and contribution to the study of prognostic factors.

    abstract::Ten characteristics of bone marrow (BM) biopsies in paraffin sections, obtained at diagnosis from patients with myelodysplastic syndromes (MDS) classified according to the FAB criteria, were analysed to identify both the most relevant morphologic data and any possible influence on survival. Agreement between two obser...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1990.tb02612.x

    authors: Ríos A,Cañizo MC,Sanz MA,Vallespí T,Sanz G,Torrabadella M,Gomis F,Ruiz C,San Miguel JF

    更新日期:1990-05-01 00:00:00

  • Proposals for the classification of the acute leukaemias. French-American-British (FAB) co-operative group.

    abstract::A uniform system of classification and nomenclature of the acute leukaemias, at present lacking, should permit more accurate recording of the distribution of cases entered into clinical trials, and could provide a reference standard when newly developed cell-surface markers believed to characterize specific cell types...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1976.tb03563.x

    authors: Bennett JM,Catovsky D,Daniel MT,Flandrin G,Galton DA,Gralnick HR,Sultan C

    更新日期:1976-08-01 00:00:00

  • How we prevent and manage infection in sickle cell disease.

    abstract::Sickle cell disease (SCD) affects approximately 100,000 people in the US, 12,500 in the UK, and millions worldwide. SCD is typified by painful vaso-occlusive episodes, haemolytic anaemia and organ damage. A secondary complication is infection, which can be bacterial, fungal or viral. Universal newborn screening, routi...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/bjh.13526

    authors: Sobota A,Sabharwal V,Fonebi G,Steinberg M

    更新日期:2015-09-01 00:00:00

  • Myelofibrosis biology and contemporary management.

    abstract::Myelofibrosis is an enigmatic myeloproliferative neoplasm, despite noteworthy strides in understanding its genetic underpinnings. Driver mutations involving JAK2, CALR or MPL in 90% of patients mediate constitutive JAK-STAT signaling which, in concert with epigenetic alterations (ASXL1, DNMT3A, SRSF2, EZH2, IDH1/2 mut...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/bjh.16576

    authors: Gangat N,Tefferi A

    更新日期:2020-10-01 00:00:00

  • VLA-4 blockade by natalizumab inhibits sickle reticulocyte and leucocyte adhesion during simulated blood flow.

    abstract::Very Late Antigen-4 (VLA-4, α4β1-integrin, ITGA4) orchestrates cell-cell and cell-endothelium adhesion. Given the proposed role of VLA-4 in sickle cell disease (SCD) pathophysiology, we evaluated the ability of the VLA-4 blocking antibody natalizumab to inhibit SCD blood cell adhesion. Natalizumab recognized surface V...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.14158

    authors: White J,Krishnamoorthy S,Gupta D,Lancelot M,Moore N,Sarnaik S,Hobbs WE 2nd,Light DR,Hines P

    更新日期:2016-09-01 00:00:00

  • Splenic irradiation before bone marrow transplantation for chronic myeloid leukaemia. Chronic Leukaemia Working Party of the European Group for Blood and Marrow Transplantation (EBMT)

    abstract::A total of 229 patients with chronic myeloid leukaemia (CML) in chronic phase were randomized between 1986 and 1990 to receive or not receive additional splenic irradiation as part of their conditioning prior to bone marrow transplantation (BMT). Both groups, 115 patients with and 114 patients without splenic irradiat...

    journal_title:British journal of haematology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1046/j.1365-2141.1996.d01-1929.x

    authors: Gratwohl A,Hermans J,van Biezen A,Arcese W,Debusscher L,Ernst P,Ferrant A,Frassoni F,Gahrton G,Iriondo A,Kolb HJ,Link H,Niederwieser D,Ruutu T,Siegert W,Zwaan FE

    更新日期:1996-12-01 00:00:00

  • Bone involvement in sickle cell disease.

    abstract::Bone involvement is the commonest clinical manifestation of sickle cell disease both in the acute setting such as painful vaso-occlusive crises, and as a source of chronic, progressive disability such as avascular necrosis. Management of these problems is often difficult because of the diagnostic imprecision of most l...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2005.05476.x

    authors: Almeida A,Roberts I

    更新日期:2005-05-01 00:00:00

  • Genomic organisation and regulation of murine alpha haemoglobin stabilising protein by erythroid Kruppel-like factor.

    abstract::Alpha haemoglobin stabilising protein (AHSP) binds free alpha-globin chains and plays an important role in the protection of red cells, particularly during beta-thalassaemia. Murine ASHP was discovered as a GATA-1 target gene and human AHSP is directly regulated by GATA-1. More recently, AHSP was rediscovered as a hig...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2006.06381.x

    authors: Keys JR,Tallack MR,Hodge DJ,Cridland SO,David R,Perkins AC

    更新日期:2007-01-01 00:00:00

  • Mobilization of Ph chromosome-negative peripheral blood stem cells in chronic myeloid leukaemia patients with imatinib mesylate-induced complete cytogenetic remission.

    abstract::Imatinib mesylate (IM, STI 571, Glivec) can induce a high rate of complete cytogenetic response (CCR) in chronic myeloid leukaemia (CML) patients, although to date the majority of patients continue to have detectable disease by sensitive reverse transcription polymerase chain reaction (RT-PCR). It is therefore possibl...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2003.04599.x

    authors: Drummond MW,Marin D,Clark RE,Byrne JL,Holyoake TL,Lennard A,United Kingdom Chronic Myeloid Leukaemia (UK CML) Working Party.

    更新日期:2003-11-01 00:00:00

  • Enhancement of human platelet activation by the combination of low concentrations of collagen and rabbit anticardiolipin antibodies.

    abstract::Low concentrations of collagen and anticardiolipin antibodies (ACLA), which were raised in rabbits by immunization with cardiolipin (CL), co-operatively activated human gel-filtrated platelets (GFP). GFP activated by adding ACLA 5 min prior to collagen (ACLA + Col) showed strong responses in cytosolic Ca2+ mobilizatio...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03739.x

    authors: Wang L,Su CY,Chou KY,Wang CT

    更新日期:2002-09-01 00:00:00

  • Lymphocytes from multi-transfused patients exhibit cytotoxicity against autologous cells.

    abstract::We previously demonstrated that multitransfused patients with severe aplastic anaemia (SAA) exhibit high numbers of alloreactive cytotoxic T lymphocyte precursors directed against their HLA identical siblings. In this study a group of patients who had received multiple blood transfusions for SAA, other haematological ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1992.tb08165.x

    authors: Kaminski ER,Hows JM,Goldman JM,Batchelor JR

    更新日期:1992-05-01 00:00:00

  • Clinical and haematological consequences of recurrent G6PD mutations and a single new mutation causing chronic nonspherocytic haemolytic anaemia.

    abstract::We have determined the causative mutation in 12 cases of glucose-6-phosphate dehydrogenase deficiency associated with chronic non-spherocytic haemolytic anaemia. In 11 of them the mutation we found had been previously reported in unrelated individuals. These mutations comprise seven different missense mutations and a ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1998.00763.x

    authors: Vulliamy TJ,Kaeda JS,Ait-Chafa D,Mangerini R,Roper D,Barbot J,Mehta AB,Athanassiou-Metaxa M,Luzzatto L,Mason PJ

    更新日期:1998-06-01 00:00:00

  • Elevation of serum lactate dehydrogenase in AL amyloidosis reflects tissue damage and is an adverse prognostic marker in patients not eligible for stem cell transplantation.

    abstract::The significance of serum lactate dehydrogenase (LDH) in light chain (AL) amyloidosis has not been previously explored. We studied 1019 newly diagnosed patients and correlated the elevation of LDH above the upper limit of normal (ULN) with disease characteristics and outcome. Four hundred and nine patients had an LDH ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.14830

    authors: Muchtar E,Dispenzieri A,Lacy MQ,Buadi FK,Kapoor P,Hayman SR,Gonsalves W,Warsame R,Kourelis TV,Chakraborty R,Russell S,Lust JA,Lin Y,Go RS,Zeldenrust S,Dingli D,Leung N,Rajkumar SV,Kyle RA,Kumar SK,Gertz MA

    更新日期:2017-09-01 00:00:00

  • Evidence for a link between sphingolipid metabolism and expression of CD1d and MHC-class II: monocytes from Gaucher disease patients as a model.

    abstract::Gaucher disease (GD) is an autosomal recessive inherited defect of the lysosomal enzyme glucocerebrosidase (GluCerase) that leads to glucosylceramide (GluCer) accumulation. We previously demonstrated the existence of imbalances in certain lymphocyte populations in GD patients. We now show that GluCerase-deficient mono...

    journal_title:British journal of haematology

    pub_type: 临床试验,杂志文章

    doi:10.1111/j.1365-2141.2005.05503.x

    authors: Balreira A,Lacerda L,Miranda CS,Arosa FA

    更新日期:2005-06-01 00:00:00

  • Expression of bcr-abl mRNA in individual chronic myelogenous leukaemia cells as determined by in situ amplification.

    abstract::We present the results of a novel method developed for evaluation of in situ amplification, a molecular genetic method at the cellular level. Reverse transcription polymerase chain reaction (RT-PCR) was used to study bcr-abl transcript levels in individual cells from patients with chronic myelogenous leukaemia (CML). ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2001.02510.x

    authors: Pachmann K,Zhao S,Schenk T,Kantarjian H,El-Naggar AK,Siciliano MJ,Guo JQ,Arlinghaus RB,Andreeff M

    更新日期:2001-03-01 00:00:00

  • CD59-deficient blood cells and PIG-A gene abnormalities in Japanese patients with aplastic anaemia.

    abstract::Patients with aplastic anaemia (AA) frequently develop paroxysmal nocturnal haemoglobinuria (PNH) as a late complication. We investigated the frequency of the development of PNH features including a glycosyl phosphatidylinositol (GPI) anchoring defect in 73 Japanese patients with AA. A deficient expression of CD59 was...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1999.01214.x

    authors: Azenishi Y,Ueda E,Machii T,Nishimura J,Hirota T,Shibano M,Nakao S,Kinoshita T,Mizoguchi H,Kitani T

    更新日期:1999-03-01 00:00:00

  • A novel CSF-1 binding factor in a patient in complete remission following cytotoxic therapy for lymphoma.

    abstract::A novel colony stimulating factor-1 (CSF-1) binding factor present in the serum from a patient in remission from lymphoma is described. Radioimmunoassay (RIA) repeatedly failed to detect circulating levels of CSF-1 in the peripheral blood system of this patient. Molecular analysis showed a normal CSF-1 gene structure ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1995.tb08937.x

    authors: Baker AH,Cachia PG,Tennant GB,Whittaker JA,White D,Stanley ER,Burnett AK,Padua RA

    更新日期:1995-01-01 00:00:00

  • Pre- and post-transplant minimal residual disease predicts relapse occurrence in children with acute lymphoblastic leukaemia.

    abstract::Relapse remains the leading cause of treatment failure in children with acute lymphoblastic leukaemia (ALL) undergoing allogeneic haematopoietic stem cell transplantation (HSCT). We retrospectively investigated the prognostic role of minimal residual disease (MRD) before and after HSCT in 119 children transplanted in ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.15086

    authors: Lovisa F,Zecca M,Rossi B,Campeggio M,Magrin E,Giarin E,Buldini B,Songia S,Cazzaniga G,Mina T,Acquafredda G,Quarello P,Locatelli F,Fagioli F,Basso G

    更新日期:2018-03-01 00:00:00

  • CD21S antigen expression in tumour cells of diffuse large B-cell lymphomas is an independent prognostic factor indicating better overall survival.

    abstract::To evaluate the clinical significance of CD21S expression of diffuse large B-cell lymphoma (DLBCL) tumour cells, we compared their clinical features, immunophenotype, response to therapy and outcome in relation to CD21S expression. Between 1987 and 1999, frozen sections from 240 DLBCL cases were examined for CD21S exp...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2004.04900.x

    authors: Ogawa S,Yamaguchi M,Oka K,Taniguchi M,Ito M,Nishii K,Nakase K,Ohno T,Kita K,Kobayashi T,Shiku H

    更新日期:2004-04-01 00:00:00

  • Partial depletion of tissue factor pathway inhibitor during subcutaneous administration of unfractionated heparin, but not with two low molecular weight heparins.

    abstract::Tissue factor pathway inhibitor (TFPI) is released to circulating blood after intravenous (i.v.) and subcutaneous (s.c.) injections of heparins, and may thus contribute to the antithrombotic effect of heparins. We have recently shown that total TFPI activity, plasma free TFPI antigen, and heparin releasable TFPI were ...

    journal_title:British journal of haematology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1046/j.1365-2141.1999.01791.x

    authors: Bendz B,Hansen JB,Andersen TO,Ostergaard P,Sandset PM

    更新日期:1999-12-01 00:00:00