Familial type II protein C deficiency associated with warfarin-induced skin necrosis and bilateral adrenal hemorrhage.

Abstract:

:A family is described in which venous thromboembolic disease is associated with reduced plasma protein C activity and normal levels of protein C antigen. Immunoelectrophoretic analysis of protein C antigen gave an abnormal pattern in all affected members, suggesting that the disorder is related to the presence of a structurally and functionally abnormal form of protein C. The propositus developed simultaneous warfarin-induced skin necrosis and bilateral adrenal hemorrhage. This is the first reported instance of warfarin-induced skin necrosis associated with a dysfunctional protein C molecule and the first reported instance of simultaneous warfarin-induced skin necrosis and bilateral adrenal hemorrhage.

journal_name

Am J Hematol

authors

Conlan MG,Bridges A,Williams E,Marlar R

doi

10.1002/ajh.2830290410

subject

Has Abstract

pub_date

1988-12-01 00:00:00

pages

226-9

issue

4

eissn

0361-8609

issn

1096-8652

journal_volume

29

pub_type

杂志文章
  • 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

  • Hydroxyurea treatment decreases glomerular hyperfiltration in children with sickle cell anemia.

    abstract::Glomerular hyperfiltration and microalbuminuria/proteinuria are early manifestations of sickle nephropathy. The effects of hydroxyurea therapy on these renal manifestations of sickle cell anemia (SCA) are not well defined. Our objective was to investigate the effects of hydroxyurea on glomerular filtration rate (GFR) ...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1002/ajh.23365

    authors: Aygun B,Mortier NA,Smeltzer MP,Shulkin BL,Hankins JS,Ware RE

    更新日期:2013-02-01 00:00:00

  • Evolution of sickle cell disease from a life-threatening disease of children to a chronic disease of adults: The last 40 years.

    abstract::Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimisti...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.24235

    authors: Chaturvedi S,DeBaun MR

    更新日期:2016-01-01 00:00:00

  • Genetic polymorphisms in cytochrome P450s, GSTs, NATs, alcohol consumption and risk of non-Hodgkin lymphoma.

    abstract::The aim of this study was to investigate whether genetic polymorphisms in cytochrome P450s (CYPs), glutathione S-transferases (GSTs), and N-acetyltransferases (NATs) genes modify the relationship between alcohol consumption and risk of non-Hodgkin's lymphoma (NHL) in a population-based, case-control study including 1,...

    journal_title:American journal of hematology

    pub_type: 信件

    doi:10.1002/ajh.21608

    authors: Li Y,Zheng T,Kilfoy BA,Lan Q,Holford T,Han X,Zhao P,Dai M,Leaderer B,Rothman N,Zhang Y

    更新日期:2010-03-01 00:00:00

  • Intensive short-term chemotherapy regimen induces high remission rate (over 90%) and event-free survival both in children and adult patients with advanced sporadic Burkitt lymphoma/leukemia.

    abstract::The optimal treatment of advanced sporadic Burkitt lymphoma in adults is still a matter of debate. The salutary results of pediatric therapies did open the road for improving the adult outcome. Between May 1988 and March 2009, 71 consecutive patients-46 adults, 25 children-affected by Burkitt lymphoma/leukemia were tr...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.22189

    authors: Todeschini G,Bonifacio M,Tecchio C,Balter R,Carli G,Stefani PM,Adami F,Zamò A,Dei Tos AP,Marino F,Gherlinzoni F,Marradi P,Semenzato G,Pizzolo G

    更新日期:2012-01-01 00:00:00

  • High serum lactate dehydrogenase level predicts short survival after vincristine-doxorubicin-dexamethasone (VAD) salvage for refractory multiple myeloma.

    abstract::We evaluated possible prognostic factors just before salvage therapy with vincristine, doxorubicin, and dexamethasone (VAD) for 36 patients with refractory multiple myeloma. The median duration from diagnosis to the first VAD salvage was 14 months (range 2-76 months). Among parameters that have been shown to be associ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/1096-8652(200010)65:2<132::aid-ajh7>3.0.co

    authors: Suguro M,Kanda Y,Yamamoto R,Chizuka A,Hamaki T,Matsuyama T,Takezako N,Miwa A,Togawa A

    更新日期:2000-10-01 00:00:00

  • Cytogenetic findings in 21 cases of peripheral T-cell lymphoma.

    abstract::Although numerous publications have described the chromosome abnormalities in B-cell non-Hodgkin lymphoma and their significance, sparse literature exists pertaining to the chromosome abnormalities in T-cell lymphoma. We did cytogenetic analyses in 21 cases of peripheral T-cell lymphoma (PTCL). Chromosomally abnormal ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830350205

    authors: Inwards DJ,Habermann TM,Banks PM,Colgan JP,Dewald GW

    更新日期:1990-10-01 00:00:00

  • HTLV-1 unrelated adult T-cell leukemia/lymphoma with unique phenotype and karyotype.

    abstract::We describe a unique case of adult T-cell leukemia/lymphoma (ATL). The patient had typical clinicohematological features as ATL, but showed a lack of antibody to human T-cell leukemia virus type-1 (HTLV-1) and was negative for HTLV-1 proviral DNA in the peripheral mononuclear cells by means of polymerase chain reactio...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(200005)64:1<64::aid-ajh11>

    authors: Nakase K,Hasegawa M,Tsuji K,Ikeda T,Tamaki S,Tanigawa M,Miyanishi E,Shiku H

    更新日期:2000-05-01 00:00:00

  • Inhibition of the activation of hageman factor (factor XII) by eosinophils and eosinophilic constituents.

    abstract::Several syndromes characterized by striking eosinophilia may be complicated by thrombosis. The experiments described indicate that, paradoxically, eosinophils and certain of their constituents inhibit the activation of Hageman factor (HF, factor XII). In earlier studies, suspensions of mixed types of granulocytes, oth...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830420127

    authors: Ratnoff OD,Gleich GJ,Shurin SB,Kazura J,Everson B,Embury P

    更新日期:1993-01-01 00:00:00

  • Immunological abnormalities in splenic marginal zone cell lymphoma.

    abstract::The clinical features of patients with splenic marginal zone cell lymphoma (SMZCL) have rarely been reported. In the present study, immunological abnormalities, particularly hematological abnormalities, observed in SMZCL were described. Autoimmune hemolytic anemia, immune thrombocytopenia, and appearance of lupus anti...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/(sici)1096-8652(199711)56:3<173::aid-ajh7>

    authors: Murakami H,Irisawa H,Saitoh T,Matsushima T,Tamura J,Sawamura M,Karasawa M,Hosomura Y,Kojima M

    更新日期:1997-11-01 00:00:00

  • Final results of a single institution experience with a pediatric-based regimen, the augmented Berlin-Frankfurt-Münster, in adolescents and young adults with acute lymphoblastic leukemia, and comparison to the hyper-CVAD regimen.

    abstract::Several studies reported improved outcomes of adolescents and young adults (AYA) with acute lymphoblastic leukemia (ALL) treated with pediatric-based ALL regimens. This prompted the prospective investigation of a pediatric Augmented Berlin-Frankfurt-Münster (ABFM) regimen, and its comparison with hyper-fractionated cy...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.24419

    authors: Rytting ME,Jabbour EJ,Jorgensen JL,Ravandi F,Franklin AR,Kadia TM,Pemmaraju N,Daver NG,Ferrajoli A,Garcia-Manero G,Konopleva MY,Borthakur G,Garris R,Wang S,Pierce S,Schroeder K,Kornblau SM,Thomas DA,Cortes JE,O'Brie

    更新日期:2016-08-01 00:00:00

  • Deformability characteristics of sickle cells by microelastimetry.

    abstract::Deformability of normal and sickle erythrocytes was measured by means of micropipette elastimetry with determination of intrinsic membrane rigidity (P) and total cell deformability (Pt). In the elastimetric technique employed, negative pressure at the pipette tip was generated and measured continuously. Membrane rigid...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830040103

    authors: Havell TC,Hillman D,Lessin LS

    更新日期:1978-01-01 00:00:00

  • A phase 2 study of lenalidomide, rituximab, cyclophosphamide, and dexamethasone (LR-CD) for untreated low-grade non-Hodgkin lymphoma requiring therapy.

    abstract::Patients with indolent non-Hodgkin lymphoma (NHL) have multiple treatment options yet there is no consensus as to the best initial therapy. Lenalidomide, an immunomodulatory agent, has single agent activity in relapsed lymphoma. This trial was conducted to assess feasibility, efficacy, and safety of adding lenalidomid...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.24693

    authors: Rosenthal A,Dueck AC,Ansell S,Gano K,Conley C,Nowakowski GS,Camoriano J,Leis JF,Mikhael JR,Keith Stewart A,Inwards D,Dingli D,Kumar S,Noel P,Gertz M,Porrata L,Russell S,Colgan J,Fonseca R,Habermann TM,Kapoor P,B

    更新日期:2017-05-01 00:00:00

  • Inhibitory effects of TTP sera on binding of anti-platelet glycoprotein IIb-IIIa monoclonal antibodies to human vascular endothelial cells.

    abstract::The binding of anti-human platelet glycoprotein (GP) IIbIIIa monoclonal antibodies to human umbilical vein endothelial cells (HUVE) was studied. Scatchard analysis using 125I-anti-platelet GPIIb-IIIa monoclonal antibody showed that the maximum binding capacity (B max) was 8 X 10(4)/cell and Kd was 40.2 nM. The binding...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830250113

    authors: Nakajima T,Koyama T,Kakishita E,Nagai K

    更新日期:1987-05-01 00:00:00

  • Just-in-time rescue plerixafor in combination with chemotherapy and granulocyte-colony stimulating factor for peripheral blood progenitor cell mobilization.

    abstract::Plerixafor, a recently approved peripheral blood progenitor cell mobilizing agent, is often added to granulocyte-colony stimulating factor (G-CSF) to mobilize peripheral blood progenitor cells in patients with lymphoma or myeloma who cannot mobilize enough CD34+ cells with G-CSF alone to undergo autologous stem cell t...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.23499

    authors: Smith VR,Popat U,Ciurea S,Nieto Y,Anderlini P,Rondon G,Alousi A,Qazilbash M,Kebriaei P,Khouri I,de Lima M,Champlin R,Hosing C

    更新日期:2013-09-01 00:00:00

  • Azacitidine treatment for patients with myelodysplastic syndrome and acute myeloid leukemia with chromosome 3q abnormalities.

    abstract::Acute Myeloid Leukemia (AML) and myelodysplasia (MDS) with chromosome 3q abnormalities have a dismal outcome either untreated or with conventional treatments. Azacitidine (AZA) is now considered as the standard of care in high-risk MDS and oligoblastic AML patients. The objective of this study was to evaluate the impa...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.24099

    authors: Wanquet A,Prebet T,Berthon C,Sebert M,Roux C,Kulasekararaj A,Micol JB,Esterni B,Itzykson R,Thepot S,Recher C,Delaunay J,Dreyfus F,Mufti G,Fenaux P,Vey N

    更新日期:2015-10-01 00:00:00

  • Laboratory evaluation of clopidogrel responsiveness by platelet function and genetic methods.

    abstract::Clopidogrel is a widely used antiplatelet agent that irreversibly inhibits platelet P2Y12 ADP receptors after conversion to an active metabolite. There are a number of laboratory tests capable of detecting clopidogrel-induced platelet inhibition and published literature correlates suboptimal clopidogrel response to ad...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.22112

    authors: Smock KJ,Saunders PJ,Rodgers GM,Johari V

    更新日期:2011-12-01 00:00:00

  • Effect of novel 1-alkyl-3-hydroxy-2-methylpyrid-4-one chelators on uptake and release of iron from macrophages.

    abstract::The effect of several iron chelators on iron uptake and release by mouse peritoneal macrophages has been investigated. The 1,2-dimethyl (L1) and 1-ethyl-2-methyl (L1NEt) derivatives of 3-hydroxypyrid-4-one markedly enhanced iron mobilisation from macrophages pulsed with 59Fe-transferrin-antitransferrin immune complexe...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830340106

    authors: Brock JH,Licéaga J,Arthur HM,Kontoghiorghes GJ

    更新日期:1990-05-01 00:00:00

  • Modulation of responsiveness of chronic myelogenous leukemia granulocyte-macrophage colony-forming cells to growth regulation following in vivo treatment with recombinant gamma-interferon.

    abstract::A patient with Philadelphia chromosome (Ph) chronic myelogenous leukemia (CML), in chronic phase, was treated with recombinant gamma-interferon (r gamma-IFN) in a phase I clinical trial. Prior to treatment, analysis of in vitro agar culture parameters indicated hyporesponsiveness of granulocyte-macrophage colony-formi...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830280105

    authors: Pelus LM,Vadhan-Raj S

    更新日期:1988-05-01 00:00:00

  • Chronic lymphoproliferative disorder with regulatory T-cell phenotype.

    abstract::We report a case of T-cell chronic lymphoproliferative disorder (CLPD) that shows neither features of T-cell prolymphocytic leukemia nor disease progression for more than 34 months. Flow cytometric analyses of the lymphocytes revealed high expression of CD4 and CD25. Up-regulation of Foxp3, a master regulatory gene fo...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20688

    authors: Kikuchi T,Katayama Y,Kubonishi S,Watanabe T,Watanabe Y,Matsuoka K,Maeda Y,Namba N,Masunari T,Nasu R,Ikeda K,Tanimoto M

    更新日期:2006-09-01 00:00:00

  • all-trans-Retinoic acid-induced expression and regulation of retinoic acid 4-hydroxylase (CYP26) in human promyelocytic leukemia.

    abstract::all-trans-Retinoic acid (ATRA) induces complete remission in majority of patients with acute promyelocytic leukemia (APL). However, accelerated metabolism of ATRA that is induced by chronic daily administration of oral ATRA has been implicated as one of the mechanisms leading to a reduced sensitivity or resistance to ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.10099

    authors: Ozpolat B,Mehta K,Tari AM,Lopez-Berestein G

    更新日期:2002-05-01 00:00:00

  • Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management.

    abstract:DISEASE OVERVIEW:Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms primarily characterized by erythrocytosis and thrombocytosis, respectively. Other disease features include leukocytosis, splenomegaly, thrombohemorrhagic complications, vasomotor disturbances, pruritus, and a sma...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.21946

    authors: Tefferi A

    更新日期:2011-03-01 00:00:00

  • Prevalence of factor V G1691A (factor V-Leiden) and prothrombin G20210A gene mutations in a recurrent miscarriage population.

    abstract::Factor V G1691A (FV-Leiden) and prothrombin G20210A mutations are major inherited risk factors for venous thrombosis. Recently, it was suggested that both mutations, through stimulation of venous and placental thrombosis events, were strongly associated with recurrent idiopathic miscarriages, although other studies di...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.10223

    authors: Finan RR,Tamim H,Ameen G,Sharida HE,Rashid M,Almawi WY

    更新日期:2002-12-01 00:00:00

  • Genomic Characteristics and Prognostic Significance of Co-mutated ASXL1/SRSF2 Acute Myeloid Leukemia.

    abstract::ASXL1 and SRSF2 mutations in AML are frequently found in patients with preexisting myeloid malignancies and are individually associated with poor outcomes. In this multi-institutional retrospective analysis, we assessed the genetic features and clinical outcomes of 43 patients with ASXL1mut SRSF2mut AML and compared o...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.26110

    authors: Richardson DR,Swoboda DM,Moore DT,Johnson SM,Chan O,Galeotti J,Esparza S,Hussaini MO,Van Deventer H,Foster MC,Coombs CC,Montgomery ND,Sallman DA,Zeidner JF

    更新日期:2021-01-27 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

  • Vasoactive factors in sickle cell disease: in vitro evidence for endothelin-1-mediated vasoconstriction.

    abstract::While systemic plasma endothelin-1 (ET-1) levels are increased during acute crisis in sickle cell disease, the relative levels of potent vasoactive factors that contribute to the regulation of vascular function, such as ET-1, NO, and cell-free hemoglobin, during the course of periodic vaso-occlusive episodes remain un...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20107

    authors: Ergul S,Brunson CY,Hutchinson J,Tawfik A,Kutlar A,Webb RC,Ergul A

    更新日期:2004-07-01 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

  • Relative iron deficiency in hereditary spherocytosis.

    abstract::Seventy-three patients with hereditary spherocytosis (HS) (58 nonsplenectomized, 15 splenectomized) were studied to evaluate iron status and the adequacy of iron availability for erythropoiesis. Splenectomized patients, who had hemoglobin levels in the normal or upper normal range, had higher levels of serum iron, tra...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830310202

    authors: Zanella A,Barosi G,Berzuini A,Colombo MB,Quaglini S,Volpes D,Sirchia G

    更新日期:1989-06-01 00:00:00

  • Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia.

    abstract::Fetal hemoglobin (HbF) inhibits the polymerization of sickle hemoglobin, modulating the clinical features of sickle cell anemia (SCA). Hydroxyurea (HU) therapy can increase the HbF level, although its production can be influenced by genetic determinants. Twenty-two Brazilian SCA patients were evaluated over 5 years be...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20293

    authors: Vicari P,Barretto de Mello A,Figueiredo MS

    更新日期:2005-03-01 00:00:00

  • Geographic variability of hemophilia-associated AIDS in the United States: effect of population characteristics. Hemophilia-Associated AIDS Study Group.

    abstract::National AIDS surveillance suggests that approximately 3.5% of all hemophilic patients in the United States have developed AIDS as of February 1988; however, the cumulative incidence of AIDS among seropositive patients at individual hemophilia treatment centers (HTCs) has been reported to be as high as 12%. The purpos...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830320304

    authors: Stehr-Green JK,Jason JM,Evatt BL

    更新日期:1989-11-01 00:00:00