Protein C and protein S levels in two patients with acquired purpura fulminans.

Abstract:

:Purpura fulminans (PF) is a cutaneous manifestation of a dramatic and deadly syndrome of systemic disseminated intravascular coagulation (DIC). It is characterized by microvascular thrombosis in the dermis followed by perivascular haemorrhage. Since two other related syndromes involve the protein C (PC) system, we undertook a serial study to investigate the levels of PC and protein S (PS) in two patients with acquired PF. Laboratory findings were consistent with DIC, and both patients were treated with blood replacement and heparin therapy. The levels of PC activity were very low during the initial 24-36 h after onset and gradually increased until returning to normal levels. The total and 'free' PS were also abnormal during the initial onset of PF. The total and free PS increased to normal after 4-6 d. Although the pathogenesis is not fully understood, the infection and sepsis appears to consume PC and PS selectively during the PF and DIC phase. Acquired PF appears to selectively involve the PC system in a similar fashion to two other syndromes of PF-like lesions.

journal_name

Br J Haematol

authors

Madden RM,Gill JC,Marlar RA

doi

10.1111/j.1365-2141.1990.tb02625.x

subject

Has Abstract

pub_date

1990-05-01 00:00:00

pages

112-7

issue

1

eissn

0007-1048

issn

1365-2141

journal_volume

75

pub_type

杂志文章
  • Haploidentical donor T cells fail to facilitate engraftment but lessen the immune response of host T cells in murine fetal transplantation.

    abstract::The effects of donor T cells, or their CD8+ subset, on engraftment and tolerance induction in fetal transplantation were evaluated using an F1-into-parent mouse-model that does not permit a graft-versus-host effect. Gestational day 13 C57BL/6 (H-2Kb) fetuses were transplanted with B6D2F1 (H-2Kb/d) light density bone m...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2004.05040.x

    authors: Chen JC,Chang ML,Lee H,Muench MO

    更新日期:2004-08-01 00:00:00

  • Fibrinogen Chapel Hill II: defective in reactions with thrombin, factor XIIIa and plasmin.

    abstract::Fibrinogen Chapel Hill II is a hereditary, abnormal fibrinogen which is characterized by poor substrate reactivity toward thrombin, factor XIIIa and plasmin. The patient has a low plasma level of clottable protein with normal antigen concentration, high amounts of fibrinogen related material in serum, and prolonged th...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1982.tb03859.x

    authors: Carrell N,McDonagh J

    更新日期:1982-09-01 00:00:00

  • A UGT2B17-positive donor is a risk factor for higher transplant-related mortality and lower survival after bone marrow transplantation.

    abstract::We recently identified a human minor histocompatibility (H) antigen, encoded by UDP glycosyltransferase 2 family, polypeptide B17 (UGT2B17), whose immunogenicity results from differential expression in donor and recipient cells as a consequence of a homozygous deletion of the UGT2B17 gene. UGT2B17 is highly expressed ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2005.05427.x

    authors: Terakura S,Murata M,Nishida T,Emi N,Akatsuka Y,Riddell SR,Morishima Y,Kodera Y,Naoe T

    更新日期:2005-04-01 00:00:00

  • A new preconditioning regimen with melphalan, busulphan and total body irradiation followed by low-dose immunosuppressant in allogeneic haemopoietic stem cell transplantation.

    abstract::Twenty adult patients with high-risk leukaemia underwent allogeneic haemopoietic stem cell transplantation after melphalan, busulphan and total body irradiation followed by short-term methotrexate and low-dose cyclosporine or tacrolimus. Three patients developed veno-occlusive disease and no patient developed renal dy...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1999.01424.x

    authors: Murata M,Nishida T,Haneda M,Kanie T,Taji H,Iida H,Suzuki R,Hamaguchi M,Minami S,Kodera Y

    更新日期:1999-06-01 00:00:00

  • Advances in individual prediction of methotrexate toxicity: a review.

    abstract::As the cure rates for haematological malignancies have improved, the exploration of the balance between efficacy and side effects has become a major research target. The antifolate methotrexate is widely used in the treatment of acute lymphoblastic leukaemia, non-Hodgkin lymphoma, and osteosarcoma. Even when given ide...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2009.07765.x

    authors: Schmiegelow K

    更新日期:2009-09-01 00:00:00

  • Long-term overall- and progression-free survival after pentostatin, cyclophosphamide and rituximab therapy for indolent non-Hodgkin lymphoma.

    abstract::In a prospective phase II trial, pentostatin combined with cyclophosphamide and rituximab (PCR) induced strong responses and was well-tolerated in previously untreated patients with advanced-stage, indolent non-Hodgkin lymphoma (iNHL). After a median patient follow-up of more than 108 months, we performed an intent-to...

    journal_title:British journal of haematology

    pub_type: 杂志文章,随机对照试验

    doi:10.1111/bjh.15814

    authors: Khashab T,Hagemeister F,Romaguera JE,Fanale MA,Pro B,McLaughlin P,Rodriguez MA,Neelapu SS,Fayad L,Younes A,Feng L,Vega F,Kwak LW,Samaniego F

    更新日期:2019-05-01 00:00:00

  • Avascular necrosis of bone following intensified steroid therapy for acute lymphoblastic leukaemia and high-grade malignant lymphoma.

    abstract::Five out of nine adults (55%) with lymphoblastic disease developed severe avascular necrosis of bone (AVN) when treated with a Berlin-Frankfurt-Munster (BFM) ALL protocol similar to the current joint MRC-ECOG ALL trial (UKALL XII). The principal purpose of these intensified regimens is to improve long-term disease-fre...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1994.tb03287.x

    authors: Chan-Lam D,Prentice AG,Copplestone JA,Weston M,Williams M,Hutton CW

    更新日期:1994-01-01 00:00:00

  • F cell numbers are associated with an X-linked genetic polymorphism and correlate with haematological parameters in patients with sickle cell disease.

    abstract::Patients with sickle cell disease (SCD) with high fetal haemoglobin (HbF) tend to have a lower incidence of complications and longer survival due to inhibition of deoxyhaemoglobin S (HbS) polymerisation by HbF. HbF-containing cells, namely F cells, are strongly influenced by genetic factors. We measured the percentage...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.17102

    authors: Urio F,Nkya S,Rooks H,Mgaya JA,Masamu U,Zozimus Sangeda R,Mmbando BP,Brumat M,Mselle T,Menzel S,Luzzatto L,Makani J

    更新日期:2020-10-19 00:00:00

  • Neutropenia and anaemia associated with T-cell large granular lymphocyte leukaemia responds to fludarabine with minimal toxicity.

    abstract::T-cell large granular lymphocyte leukaemia (T-LGL) is a clonal disorder of T cells associated with neutropenia and anaemia. The clinical consequences are recurrent infections and transfusion dependence. The optimum treatment for severely affected patients remains to be defined. Current therapies require long-term admi...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2003.04148.x

    authors: Sternberg A,Eagleton H,Pillai N,Leyden K,Turner S,Pearson D,Littlewood T,Hatton C

    更新日期:2003-02-01 00:00:00

  • Critical study of prognostic factors in childhood acute lymphoblastic leukaemia: differences in outcome are poorly explained by the most significant prognostic variables. Fralle group. French Acute Lymphoblastic Leukaemia study group.

    abstract::We determined the proportion of survival variability explained by the usual prognostic factors in childhood acute lymphoblastic leukaemia (ALL) during a prognostic study of 1552 patients enrolled in three consecutive Fralle group protocols (Fralle 83, Fralle 87 and Fralle 89). The event-free survival rates at 5 years ...

    journal_title:British journal of haematology

    pub_type: 杂志文章,多中心研究

    doi:10.1046/j.1365-2141.1998.00818.x

    authors: Donadieu J,Auclerc MF,Baruchel A,Leblanc T,Landman-Parker J,Perel Y,Michel G,Cornu G,Bordigoni P,Sommelet D,Leverger G,Hill C,Schaison G

    更新日期:1998-08-01 00:00:00

  • The management and outcome of chronic graft-versus-host disease.

    abstract::Chronic graft-versus-host disease (cGVHD) is a common complication following allogeneic haematopoietic cell transplantation (HCT). It is the leading cause of non-relapse mortality in transplant survivors and has a significant impact upon their functional status and quality of life. Despite significant advances being m...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2007.06652.x

    authors: Fraser CJ,Scott Baker K

    更新日期:2007-07-01 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

  • 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

  • Moderate endurance exercise in patients with sickle cell anaemia: effects on oxidative stress and endothelial activation.

    abstract::Very few studies have investigated the effects of exercise on the biological parameters involved in vaso-occlusive events in sickle cell anaemia (SCA). The aim of this study was to test how a mild-moderate endurance exercise modulates oxidative stress, nitric oxide bioavailability and endothelial activation in SCA pat...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.12594

    authors: Faes C,Balayssac-Siransy E,Connes P,Hivert L,Danho C,Bogui P,Martin C,Pialoux V

    更新日期:2014-01-01 00:00:00

  • In vivo folic acid supplementation partially corrects in vitro methotrexate toxicity in patients with Down syndrome.

    abstract::Patients with Down syndrome have been found to have characteristic in vivo and in vitro methotrexate toxicity. The in vitro methotrexate toxicity characteristic of Down syndrome can be diminished by the in vivo administration of supplemental high doses of folic acid. A possible explanation for the increased sensitivit...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1995.tb08390.x

    authors: Peeters MA,Rethore MO,Lejeune J

    更新日期:1995-03-01 00:00:00

  • Arsenic trioxide (AT) is a novel human neutrophil pro-apoptotic agent: effects of catalase on AT-induced apoptosis, degradation of cytoskeletal proteins and de novo protein synthesis.

    abstract::The anti-cancer drug arsenic trioxide (AT) induces apoptosis in a variety of transformed or proliferating cells. However, little is known regarding its ability to induce apoptosis in terminally differentiated cells, such as neutrophils. Because neutropenia has been reported in some cancer patients after AT treatment, ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2005.05866.x

    authors: Binet F,Cavalli H,Moisan E,Girard D

    更新日期:2006-02-01 00:00:00

  • Immune neutropenia associated with anti-human neutrophil antigen-2a (NB1) antibodies following unrelated donor stem cell transplantation for chronic myeloid leukaemia: perpetuation by granulocyte colony-stimulating factor.

    abstract::A case of immune neutropenia following unrelated stem cell transplantation for chronic myeloid leukaemia is described. The neutropenia developed following herpes zoster viral infection and was associated with antibodies to the human neutrophil antigen (HNA)-2a (formerly known as NB1). The neutropenia was prolonged, pr...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2001.02779.x

    authors: Pocock CF,Lucas GF,Giles C,Vassiliou G,Cwynarski K,Rezvani K,Apperley JF,Goldman JM

    更新日期:2001-05-01 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

  • Large granular lymphocytic leukaemia after solid organ and haematopoietic stem cell transplantation.

    abstract::T-cell large granular lymphocytic leukaemia (T-LGLL) is a chronic clonal lymphoproliferative disorder of cytotoxic T lymphocytes which commonly occurs in older patients and is often associated with autoimmune diseases. Among 246 patients with T-LGLL seen at our institution over the last 10 years, we encountered 15 cas...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.16336

    authors: Awada H,Mahfouz RZ,Durrani J,Kishtagari A,Jagadeesh D,Lichtin AE,Hill BT,Hamilton BK,Carraway HE,Nazha A,Majhail NS,Sobecks R,Visconte V,Kalaycio M,Sekeres MA,Maciejewski JP

    更新日期:2020-04-01 00:00:00

  • Studies on immunological assay of vitamin K dependent factors. II. Comparison of four immunoassay methods with functional activity of protein C in human plasma.

    abstract::We describe and compare five assay systems for Protein C (PC) in human plasma; a functional assay for PC activity, Laurell electroimmunoassay with EDTA or calcium (EDTA-Laurell or Ca-Laurell), radioimmunoassay (RIA) and immunoradiometric assay (IRMA). The lower limit of sensitivity of PC in normal reference plasma was...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1986.tb02915.x

    authors: Mikami S,Tuddenham EG

    更新日期:1986-01-01 00:00:00

  • Fas/APO-1 (CD95)-mediated cytotoxicity is responsible for the apoptotic cell death of leukaemic cells induced by interleukin-2-activated T cells.

    abstract::Apoptotic cell death is induced by the cross-linking of Fas/APO-1 receptor (CD95) in acute myelogenous leukaemia (AML) cells. Since CD95 ligand (CD95L) is expressed on interleukin-2 (IL-2)-activated T cells, we investigated the involvement of CD95-CD95L pathway in T cell-mediated cytotoxicity against AML cells. Activa...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1997.8742505.x

    authors: Komada Y,Zhou YW,Zhang XL,Chen TX,Tanaka S,Azuma E,Sakurai M

    更新日期:1997-01-01 00:00:00

  • Characterization of a variant prothrombin in a patient congenitally deficient in factors II, VII, IX and X.

    abstract::An 18-month-old child, who had no evidence of liver disease, malabsorption, or chronic ingestion of coumarin compounds, was found to have plasma deficiencies of factors II, VII, IX and X. Assays for factor II and X by immunological techniques (antibody neutralization and immunoelectrophoresis) revealed normal or eleva...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1980.tb05916.x

    authors: Johnson CA,Chung KS,McGrath KM,Bean PE,Roberts HR

    更新日期:1980-03-01 00:00:00

  • Origin of human bone marrow fibroblasts.

    abstract::We investigated the origin of bone marrow fibroblasts in three bone marrow transplant recipients with aplastic anaemia and leukaemia who received grafts from HLA-identical siblings of opposite sex. The patients were conditioned for transplantation with high doses of cytotoxic drugs and 300--1000 rads total body irradi...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1980.tb01200.x

    authors: Golde DW,Hocking WG,Quan SG,Sparkes RS,Gale RP

    更新日期:1980-02-01 00:00:00

  • Lenalidomide plus rituximab (R2 ) in previously untreated marginal zone lymphoma: subgroup analysis and long-term follow-up of an open-label phase 2 trial.

    abstract::Lack of consensus for first-line marginal zone lymphoma (MZL) treatment and toxicities associated with currently available systemic therapies have inspired evaluation of immunotherapeutic agents yielding robust outcomes with improved tolerability. We previously reported durable efficacy with first-line lenalidomide an...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.15843

    authors: Becnel MR,Nastoupil LJ,Samaniego F,Davis RE,You MJ,Green M,Hagemeister FB,Fanale MA,Fayad LE,Westin JR,Wang M,Oki Y,Forbes SG,Feng L,Neelapu SS,Fowler NH

    更新日期:2019-06-01 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

  • Simultaneous SIL-TAL1 RT-PCR detection of all tal(d) deletions and identification of novel tal(d) variants.

    abstract::Site-specific deletions of the 5' part of the TAL1 gene (tal(d)) are among the most frequent non-random genetic abnormalities in T-cell acute lymphoblastic leukaemia (T-ALL). They are usually detected by PCR from DNA with several primer pairs or by Southern blot analysis. Since tal(d) lead to expression of a SIL-TAL1 ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1997.4833286.x

    authors: Delabesse E,Bernard M,Landman-Parker J,Davi F,Leboeuf D,Varet B,Valensi F,Macintyre EA

    更新日期:1997-12-01 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

  • Marizomib for central nervous system-multiple myeloma.

    abstract::Marizomib, a natural marine product, is an irreversible proteasome inhibitor currently under investigation in relapsed-refractory multiple myeloma (RRMM) and malignant glioma. Central nervous system-multiple myeloma (CNS-MM) is a rare manifestation of extra-medullary disease with few therapeutic options, highlighting ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.14498

    authors: Badros A,Singh Z,Dhakal B,Kwok Y,MacLaren A,Richardson P,Trikha M,Hari P

    更新日期:2017-04-01 00:00:00

  • Mutations of the p53 tumour suppressor gene in haematologic neoplasms.

    abstract::Mutations of the p53 tumour suppressor gene have frequently been observed in several types of solid tumours and are believed to be implicated in the development of these tumours. To determine the relevance of p53 mutations in haematologic neoplasms, we performed polymerase chain reaction-single strand conformation pol...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1992.tb08213.x

    authors: Mori N,Wada M,Yokota J,Terada M,Okada M,Teramura M,Masuda M,Hoshino S,Motoji T,Oshimi K

    更新日期:1992-06-01 00:00:00

  • Prospective clinical evaluation of lower cut-offs for galactomannan detection in adult neutropenic cancer patients and haematological stem cell transplant recipients.

    abstract::The recent advent of an improved commercial serum enzyme-linked immunosorbent assay (ELISA) for the detection of circulating galactomannan (GM), a major constituent of Aspergillus cell walls, has contributed to the diagnosis of invasive aspergillosis (IA) in many haematology and transplant centres. However, the optima...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2004.05140.x

    authors: Maertens J,Theunissen K,Verbeken E,Lagrou K,Verhaegen J,Boogaerts M,Eldere JV

    更新日期:2004-09-01 00:00:00