Report of a factor VIII inhibitor in a patient with autoimmune lymphoproliferative syndrome.

Abstract:

:The occurrence of factor VIII inhibitors in non-hemophilic patients is a rare event with a potentially lethal outcome. Despite its infrequent occurrence, the association of this inhibitor with multiple autoimmune diseases is well recognized. We report the case of a patient with the recently described autoimmune lymphoproliferative syndrome (ALPS) who developed an inhibitor to factor VIII. ALPS is a disease characterized by defective lymphocyte apoptosis due to inherited mutations in genes that regulate apoptosis, with the resulting enlargement of lymphoid organs and a variety of autoimmune manifestations. Published 2000 Wiley-Liss, Inc.

journal_name

Am J Hematol

authors

Fang BS,Sneller MC,Straus SE,Frenkel L,Dale JK,Rick ME

doi

10.1002/1096-8652(200007)64:3<214::aid-ajh14>3.0.c

keywords:

subject

Has Abstract

pub_date

2000-07-01 00:00:00

pages

214-7

issue

3

eissn

0361-8609

issn

1096-8652

pii

10.1002/1096-8652(200007)64:3<214::AID-AJH14>3.0.C

journal_volume

64

pub_type

杂志文章
  • Enhanced botrocetin-induced type IIB von Willebrand factor binding to platelet glycoprotein Ib initiates hyperagglutination of normal platelets.

    abstract::Botrocetin, a protein isolated from the venom of the snake Bothrops jararaca, induces platelet aggregation/agglutination by von Willebrand factor (vWF) binding to the membrane glycoprotein (GP) Ib, an action resembling that of ristocetin. However, some differences in the interaction between vWF and platelet GPIb induc...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830330409

    authors: Nishio K,Fujimura Y,Niinomi K,Takahashi Y,Yoshioka A,Fukui H,Usami Y,Titani K,Ruggeri ZM,Zimmerman TS

    更新日期:1990-04-01 00:00:00

  • R147W mutation of PROC gene is common in venous thrombotic patients in Taiwanese Chinese.

    abstract::We analyzed the genetic defects of 21 unrelated patients with venous thrombosis in whom hereditary protein C deficiency was diagnosed. Eleven mutations were detected in 18 families, while no mutation was detectable in the other three families. Among these mutations, a common genetic mutation of protein C (PROC) gene r...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20043

    authors: Tsay W,Shen MC

    更新日期:2004-05-01 00:00:00

  • Primary myelofibrosis: 2019 update on diagnosis, risk-stratification and management.

    abstract:DISEASE OVERVIEW:Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by stem cell-derived clonal myeloproliferation that is often but not always accompanied by JAK2, CALR, or MPL mutations; additional disease features include bone marrow stromal reaction including reticulin fibrosis, abnorm...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.25230

    authors: Tefferi A

    更新日期:2018-12-01 00:00:00

  • Study of fibrinolytic parameters in different types of polycythemia.

    abstract::Polycythemia vera (PV) is a myeloproliferative disorder characterized by thrombotic and, less often, bleeding complications. Many mechanisms have been advanced to explain the occurrence of these complications, none of them satisfactory. We examined a cohort of 27 patients with PV, secondary erythrocytosis, and essenti...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199903)60:3<196::aid-ajh5>

    authors: Lugassy G,Filin I

    更新日期:1999-03-01 00:00:00

  • Trends in anti-D immune globulin for childhood immune thrombocytopenia: usage, response rates, and adverse effects.

    abstract::In 2010, the Food and Drug Administration (FDA) added a black box warning to anti-D immune globulin (Rho(D) immune globulin, anti-D) for immune thrombocytopenia (ITP) to warn of the complications related to severe hemolysis. The objective of this retrospective medical record review was to examine recent trends in anti...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.22261

    authors: Long M,Kalish LA,Neufeld EJ,Grace RF

    更新日期:2012-03-01 00:00:00

  • In vitro exposure to hydroxyurea reduces sickle red blood cell deformability.

    abstract::Hydroxyurea is a drug that is used to treat some patients with sickle cell disease. We have measured the deformability of sickle erythrocytes incubated in hydroxyurea in vitro and found that hydroxyurea acts to decrease the deformability of these cells. The deformability of normal erythrocytes was not significantly af...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.1098

    authors: Huang Z,Louderback JG,King SB,Ballas SK,Kim-Shapiro DB

    更新日期:2001-07-01 00:00:00

  • The first two Japanese cases of severe type I congenital plasminogen deficiency with ligneous conjunctivitis: successful treatment with direct thrombin inhibitor and fresh plasma.

    abstract::A 71-year-old woman and her elder sister developed ligneous conjunctivitis after ocular surgery. Laboratory tests demonstrated that the proband and her sister had 6.6% and 8.1% of plasminogen activity, and 1.2 and 1.4 mg/dl of antigen, respectively. Thus, they were diagnosed as having severe type I plasminogen deficie...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.21402

    authors: Suzuki T,Ikewaki J,Iwata H,Ohashi Y,Ichinose A

    更新日期:2009-06-01 00:00:00

  • Prednisone-responsive aplastic anemia associated with T-lymphocyte proliferation.

    abstract::In some cases, bone marrow aplasia has been thought to result from immunologic abnormalities. Our patient had severe transfusion-dependent aplastic anemia, which responded to treatment with prednisone on two occasions. The exacerbations of aplastic anemia were associated with lymphocytic proliferation which on one occ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830110310

    authors: Bodner S,Cohen HJ

    更新日期:1981-11-01 00:00:00

  • CD20-positive adult T-cell leukemia.

    abstract::A 67-year-old woman was admitted to our hospital because of lymphadenopathy and lymphocytosis. Monoclonal integration of HTLV-I provirus DNA was detected, and a diagnosis of adult T-cell leukemia (ATL) was made. Flow cytometry revealed that the ATL cells expressed CD20 as well as T-cell-associated antigens, and expres...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/1096-8652(200101)66:1<39::AID-AJH1005>3.0.

    authors: Yasukawa M,Arai J,Kakimoto M,Sakai I,Kohno H,Fujita S

    更新日期:2001-01-01 00:00:00

  • Inhibitory effect of short-chain aliphatic acids on deoxythymidine incorporation into DNA in mitogen-stimulated and leukemic lymphocytes.

    abstract::The effect of short-chain aliphatic acids on [3H]deoxythymidine incorporation into DNA was studied in human mitogen-stimulated lymphocytes. Butyric acid at 1-2 mM level was strongly inhibitory; however, its hydroxy or amino derivatives were ineffective. Valeric and propionic acids were less inhibitory. Formic, acetic,...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830130203

    authors: Stolc V

    更新日期:1982-09-01 00:00:00

  • Danazol for the treatment of thrombocytopenia in patients with myelodysplastic syndrome.

    abstract::Thrombocytopenia is a poor prognostic indicator in the myelodysplastic syndromes (MDS). Treatment options for patients with symptomatic thrombocytopenia are limited. Danazol, an attenuated androgen, may have some efficacy in increasing the platelet count of patients with MDS. We retrospectively reviewed 33 patients wi...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.10209

    authors: Chan G,DiVenuti G,Miller K

    更新日期:2002-11-01 00:00:00

  • Waldenström macroglobulinemia: 2011 update on diagnosis, risk stratification, and management.

    abstract:DISEASE OVERVIEW:Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, and lymphadenopathy. DIAGNOSIS:Presence of IgM monoclonal protein associated with 10% clonal lymphoplasmacytic cell...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.22014

    authors: Gertz MA

    更新日期:2011-05-01 00:00:00

  • Evidence that circulating immune complexes remove transfused platelets from the circulation.

    abstract::Fifteen recently diagnosed patients with acute leukemias admitted for induction chemotherapy were selected for study. When thrombocytopenic (venous platelet count less than 20 X 10(9)/l) these patients received prophylactic platelet transfusions. A total of 67 platelet transfusion therapies were administered and evalu...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830110305

    authors: Kutti J,Zaroulis CG,Safai-Kutti S,Dinsmore RE,Day NK,Good RA

    更新日期:1981-11-01 00:00:00

  • A multi-institutional comparison of mitoxantrone, etoposide, and cytarabine vs high-dose cytarabine and mitoxantrone therapy for patients with relapsed or refractory acute myeloid leukemia.

    abstract::Relapsed or refractory acute myeloid leukemia (R/R AML) has a poor prognosis and is best treated with salvage chemotherapy as a bridge to allogeneic stem cell transplant (alloSCT). However, the optimal salvage therapy remains unknown. Here we compared two salvage regimens; mitoxantrone, etoposide, and cytarabine (MEC)...

    journal_title:American journal of hematology

    pub_type: 杂志文章,多中心研究

    doi:10.1002/ajh.25838

    authors: Christian S,Arain S,Patel P,Khan I,Calip GS,Agrawal V,Sweiss K,Griffin S,Cahill K,Konig H,Esen A,Shergill A,Odenike O,Stock W,Quigley JG

    更新日期:2020-08-01 00:00:00

  • Surgery in patients with hemoglobin SC disease. Preoperative Transfusion in Sickle Cell Disease Study Group.

    abstract::While surgery is commonly required for complications related to hemoglobin SC (HbSC) disease, little is known about the perioperative complications or the indications for preoperative transfusion in this group. We describe the patient characteristics, preoperative transfusion regimens, and outcome in 92 patients with ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199802)57:2<101::aid-ajh2>

    authors: Neumayr L,Koshy M,Haberkern C,Earles AN,Bellevue R,Hassell K,Miller S,Black D,Vichinsky E

    更新日期:1998-02-01 00:00:00

  • Single agents vs combination chemotherapy in relapsed and refractory peripheral T-cell lymphoma: Results from the comprehensive oncology measures for peripheral T-cell lymphoma treatment (COMPLETE) registry.

    abstract::Single agents have demonstrated activity in relapsed and refractory (R/R) peripheral T-cell lymphoma (PTCL). Their benefit relative to combination chemotherapy remains undefined. Patients with histologically confirmed PTCL were enrolled in the Comprehensive Oncology Measures for Peripheral T-cell Lymphoma Treatment (C...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1002/ajh.25463

    authors: Stuver RN,Khan N,Schwartz M,Acosta M,Federico M,Gisselbrecht C,Horwitz SM,Lansigan F,Pinter-Brown LC,Pro B,Shustov AR,Foss FM,Jain S

    更新日期:2019-06-01 00:00:00

  • Neutrophil elastase in patients with homozygous beta-thalassemia and pseudoxanthoma elasticum-like syndrome.

    abstract::In this study we investigated the possible role of neutrophil (PMN) elastase and its natural inhibitor, alpha1-proteinase inhibitor (alpha1-PI) in the pathogenesis of the pseudoxanthoma elasticum (PXE)-like syndrome which is found in patients with homozygous beta-thalassemia. We studied 30 beta-thalassemia homozygotes...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1002/(sici)1096-8652(200002)63:2<63::aid-ajh1>3

    authors: Samarkos M,Aessopos A,Fragodimitri C,Karagiorga M,Kalotychou V,Voskaridou E,Kavouklis E,Loukopoulos D

    更新日期:2000-02-01 00:00:00

  • Quantitative flow cytometry for the differential diagnosis of leukemic B-cell chronic lymphoproliferative disorders.

    abstract::We have investigated whether the quantitative flow cytometry is an useful tool to better characterize B-cell chronic lymphoproliferative disorders (CLDs). Peripheral blood samples from 104 patients with leukemic B-cell disorders and 20 healthy donors were analyzed. Directly phycoerythrin-conjugated CD19, CD20, CD22, C...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/1096-8652(200008)64:4<275::aid-ajh7>3.0.co

    authors: D'Arena G,Musto P,Cascavilla N,Dell'Olio M,Di Renzo N,Carotenuto M

    更新日期:2000-08-01 00:00:00

  • Serum from outdated human platelet concentrates: an alternative supplement for tissue (fibroblast) culture media.

    abstract::Fetal calf serum (FCS) is used as a component of the media for cells grown in culture. However, the high cost of FCS has stimulated a search for a possible alternative. Approximately 10% of human platelet concentrates prepared for clinical transfusions are outdated. Sera prepared from platelet concentrates were compar...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830170104

    authors: Schwartz KA,Lu G,Trosko JE,Chang CC

    更新日期:1984-07-01 00:00:00

  • Persistency with zoledronic acid is associated with clinical benefit in patients with multiple myeloma.

    abstract::Zoledronic acid (ZOL), an intravenous bisphosphonate, has been shown to reduce and delay the incidence of skeletal-related events (SREs) in multiple myeloma (MM) patients with bone disease. A retrospective claims-based analysis was conducted that used two distinct US managed care databases to examine the relationship ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.23164

    authors: Henk HJ,Teitelbaum A,Perez JR,Kaura S

    更新日期:2012-05-01 00:00:00

  • The choice of second-line therapy in steroid-resistant immune thrombocytopenia: role of platelet kinetics in a single-centre long-term study.

    abstract::Splenectomy is a time-honoured well established approach for patients with steroid-resistant immune thrombocytopenia (ITP). However, due to the more recent availability of therapeutic options alternative to splenectomy, such as rituximab and agonists of the thrombopoietin-receptor, the choice of second-line therapy is...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.23823

    authors: Palandri F,Polverelli N,Catani L,Sollazzo D,Romano M,Levorato M,Vianelli N

    更新日期:2014-11-01 00:00:00

  • Eosinophilia in routine blood samples and the subsequent risk of hematological malignancies and death.

    abstract::Eosinophilia may represent an early paraclinical sign of hematological malignant disease, but no reports exist on its predictive value for hematological malignancies. From the Copenhagen Primary Care Differential Count (CopDiff) Database, we identified 356,196 individuals with at least one differential cell count (DIF...

    journal_title:American journal of hematology

    pub_type: 杂志文章,随机对照试验

    doi:10.1002/ajh.23515

    authors: Andersen CL,Siersma VD,Hasselbalch HC,Lindegaard H,Vestergaard H,Felding P,de Fine Olivarius N,Bjerrum OW

    更新日期:2013-10-01 00:00:00

  • Clonality of cold agglutinins in patients with hemolytic anemia: an analysis by high-resolution two-dimensional gel electrophoresis.

    abstract::High-resolution two-dimensional gel electrophoresis (2-DGE) was used to analyse plasma samples and partially purified cold agglutinins (CA) obtained from two selected patients. Both presented an acute hemolytic anemia with CA of high thermal amplitude, normal immunoglobulin levels, no detectable paraproteinemia, and n...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830400304

    authors: Tissot JD,Clément F,Schifferli JA,Frei PC,Hochstrasser DF,Schneider P

    更新日期:1992-07-01 00:00:00

  • The prognostic value of monosomal karyotype (MK) in higher-risk patients with myelodysplastic syndromes treated with 5-Azacitidine: A retrospective analysis of the Hellenic (Greek) Myelodysplastic syndromes Study Group.

    abstract::In this study, we investigated the incidence and prognostic impact of monosomal karyotype (MK) in 405 higher-risk Myelodysplastic Syndromes (MDS) patients treated with 5-AZA. The MK was present in 66 out of 405 (16.3%) patients, most of whom had complex karyotype (CK). MK was strongly associated with CK and the cytoge...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.25111

    authors: Papageorgiou SG,Vasilatou D,Kontos CK,Kotsianidis I,Symeonidis A,Galanopoulos AG,Hatzimichael E,Megalakaki A,Poulakidas E,Diamantopoulos P,Vassilakopoulos TP,Zikos P,Papadaki H,Mparmparousi D,Bouronikou E,Panayiotidis P,V

    更新日期:2018-07-01 00:00:00

  • Retrospective review of the management of elective surgery with desmopressin and clotting factor concentrates in patients with von Willebrand disease.

    abstract::Limited data are available regarding optimal treatment with desmopressin (DDAVP) or intermediate-purity FVIII concentrates rich in VWF (CFCs) in patients with von Willebrand disease (VWD) who undergo planned surgery. We undertook a retrospective review over 10 years (1988-1997) and identified 27 patients treated with ...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.1058

    authors: Nitu-Whalley IC,Griffioen A,Harrington C,Lee CA

    更新日期:2001-04-01 00:00:00

  • Thiotepa-based conditioning for allogeneic stem cell transplantation in acute lymphoblastic leukemia-A survey from the Acute Leukemia Working Party of the European Society for Blood and Marrow Transplantation.

    abstract::In this study, we analyzed a thiotepa-based conditioning regimen for allogeneic stem cell transplantation in adults with acute lymphoblastic leukemia, using the EBMT database. A total of 323 patients were identified. The median age was 43 years. Disease status at transplant was first complete remission (CR1) in 48.9%,...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1002/ajh.24567

    authors: Eder S,Beohou E,Labopin M,Sanz J,Finke J,Arcese W,Or R,Bonifazi F,Aljurf M,Socié G,Passweg J,Giebel S,Mohty M,Nagler A

    更新日期:2017-01-01 00:00:00

  • Febrile transfusion reaction following initial transfusion in a man with immunoblastic lymphadenopathy and granulocyte autoantibodies.

    abstract::A 69-year-old man with immunoblastic lymphadenopathy and autoimmune hemolytic anemia who had no previous exposure to blood products developed a severe febrile nonhemolytic transfusion reaction following the initial infusion of packed red blood cells. The reaction recurred with transfusion of packed red blood cells, bu...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830140112

    authors: Barnes HM,Poon MC,Huang ST,Conrad ME,Lin J,McGowan EI

    更新日期:1983-02-01 00:00:00

  • Sequential administration of cyclophosphamide and granulocyte-colony stimulating factor relieves impaired myeloid maturation in Felty's syndrome.

    abstract::A patient with Felty's syndrome (FS) and persistent profound neutropenia developed recurrent infections and sepsis syndrome. No impairment of granulocyte-macrophage colony development was observed in vitro. Marrow morphology revealed an absence of mature neutrophil forms despite administration of granulocyte-colony st...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830430415

    authors: Pixley JS,Yoneda KY,Manalo PB

    更新日期:1993-08-01 00:00:00

  • Phase I study of oral clofarabine consolidation in adults aged 60 and older with acute myeloid leukemia.

    abstract::Clofarabine has shown activity and tolerability in older patients with acute myeloid leukemia (AML). We investigated the safety and tolerability of an oral formulation of clofarabine for consolidation therapy of patients aged 60 and older with AML. In this phase I study, twenty-two patients older than 60 years with AM...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.23663

    authors: Jacoby MA,Martin MG,Uy GL,Westervelt P,Dipersio JF,Cashen A,Stockerl-Goldstein K,Vij R,Luo J,Reineck T,Bernabe N,Abboud CN

    更新日期:2014-05-01 00:00:00

  • Elevated plasma endothelin-1 levels in sickle cell anemia: relationships to oxygen saturation and left ventricular hypertrophy.

    abstract::Endothelin-1 (Et-1) is a vasoconstrictor produced by endothelial and vascular smooth muscle cells in response to hypoxia, which induces hypertrophy in cultured cardiac myocytes. We measured plasma Et-1 levels and left ventricular dimensions in 13 patients with sickle cell anemia (SCD) and in 12 African-American contro...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199807)58:3<195::aid-ajh6>

    authors: Werdehoff SG,Moore RB,Hoff CJ,Fillingim E,Hackman AM

    更新日期:1998-07-01 00:00:00