Abstract:
:Mutation of the VHL tumor suppressor gene is a frequent genetic event in the carcinogenesis of renal-cell carcinoma (RCC). Circulating endothelial progenitor cells (EPCs) have important role in neoangiogenesis, and mobilization of these cells is induced by various growth factors including erythropoietin (EPO). With this regard, we analyzed a patient with EPO-producing clear-cell RCC and polycythemia. DNA extraction and sequencing analysis of the VHL gene were performed from the tumor and the adjacent normal renal tissue. Isolated and cultured circulating EPCs from the blood taken with phlebotomy were characterized by flow cytometry and immunofluorescence analysis. This RCC had two novel somatic mutations of the VHL gene, p.Leu128Pro and p.Asn131Lys. Culture of blood mononuclear cells revealed a strikingly high number of endothelial cell colonies derived from EPCs (nearly 10-fold more than in controls). Elevated number of circulating EPCs seems to be related to high EPO production from RCC with novel double somatic mutation of the VHL gene in this patient.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Rad FH,Ulusakarya A,Gad S,Sibony M,Juin F,Richard S,Machover D,Uzan Gdoi
10.1002/ajh.21019subject
Has Abstractpub_date
2008-02-01 00:00:00pages
155-8issue
2eissn
0361-8609issn
1096-8652journal_volume
83pub_type
杂志文章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
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更新日期:1999-03-01 00:00:00
abstract::We performed a laparoscopic splenectomy (LS) in 60 patients (age 9-83, 45 females) with idiopathic thrombocytopenic purpura (ITP) who did not achieve sustained remission on steroid therapy. Using a modified procedure, the mean duration of LS was 78 min (range 25-240 min) and surgery was associated with only 5% major a...
journal_title:American journal of hematology
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doi:10.1002/(sici)1096-8652(200001)63:1<7::aid-ajh2>3.
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abstract::Selective labeling of phagocytes in human blood can be achieved by incubation of unseparated, heparinized blood with 99mTc-sulfur colloid. We have developed a method for quantitation of granulocyte chemotaxis using granulocytes labeled by this technique. The method was found to be more accurate and less tedious to per...
journal_title:American journal of hematology
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doi:10.1002/ajh.2830030402
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journal_title:American journal of hematology
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journal_title:American journal of hematology
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doi:10.1002/ajh.2830410305
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abstract::Allogeneic hematopoietic stem cell transplantation (HSCT) represents the only curative option for primary hemophagocytic lymphohistiocytosis (HLH), a rare disease of infants and young children, characterized by recurrent fever, hepatosplenomegaly, and cytopenia. We report a case of successful engraftment and stable fu...
journal_title:American journal of hematology
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更新日期:2003-02-01 00:00:00
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journal_title:American journal of hematology
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journal_title:American journal of hematology
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更新日期:2005-06-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章,评审
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journal_title:American journal of hematology
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更新日期:1982-02-01 00:00:00
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更新日期:1991-09-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10142
更新日期:2002-07-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280115
更新日期:1988-05-01 00:00:00
abstract::Thrombospondin (TSP), a large protein found in platelet alpha-granules (as TSP-1), mediates adhesion of sickle reticulocytes to cultured vascular endothelium. To further explore the physiologic relevance of this observation, we have measured plasma TSP levels and platelet TSP-1 content in subjects with sickle cell dis...
journal_title:American journal of hematology
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doi:10.1002/(SICI)1096-8652(199604)51:4<296::AID-AJH8>
更新日期:1996-04-01 00:00:00
abstract::Iron studies were compared in 434 patients from 80 hemochromatosis families classified as putative homozygotes, heterozygotes, and normal by HLA typing. There were 28 of 255 (11%) heterozygotes with an elevated serum ferritin and 22 of 255 (8.6%) with an elevated transferrin saturation. Serum ferritin (140 +/- 10.2 mi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450210
更新日期:1994-02-01 00:00:00
abstract::It has been suggested that alterations of cell cycle genes probably contribute to the pathogenesis of endemic Burkitt's lymphoma (BL) in addition to c-MYC translocation. Mutations disrupting the normal nuclear localization signal of the retinoblastoma-related gene Rb2/p130 have been documented in BL cell lines and pri...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20749
更新日期:2007-03-01 00:00:00
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>
更新日期:1998-02-01 00:00:00
abstract::The Sicilian type of (delta beta) (0)-thalassemia characterized by a approximately 13 kb deletion, was present in a Turkish boy who is a homozygote and in his heterozygous parents who are first cousins. The father with approximately 21% Hb F had five alpha-globin genes (alpha alpha/alpha alpha alpha) and the mother wi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830340316
更新日期:1990-07-01 00:00:00
abstract::Conventional treatment of mantle cell lymphoma (MCL) yields modest responses and short remissions. We report 30 hematopoietic stem cell transplants (HSCT) for MCL: 13 autologous, 10 allogeneic myeloablative, and 7 nonablative. After a median 1.2 years from diagnosis (range 0.5 to 4.7) and a median of 2 pre-HSCT chemot...
journal_title:American journal of hematology
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更新日期:2006-07-01 00:00:00
abstract::Acute leukemias of ambiguous lineage (ALAL) comprise acute undifferentiated leukemias (AUL) and mixed-phenotype acute leukemias (MPAL). In the revised fourth edition of the World Health Organization (WHO) classification provided further refinements to the diagnostic criteria for ALAL. Molecular characterization of MPA...
journal_title:American journal of hematology
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doi:10.1002/ajh.25771
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pub_type: 杂志文章
doi:10.1002/ajh.20602
更新日期:2006-06-01 00:00:00
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
更新日期:2001-04-01 00:00:00
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
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doi:10.1002/ajh.21402
更新日期:2009-06-01 00:00:00
abstract::Six patients and their parents from five different families with Hb H have been evaluated clinically and hematologically. Previous studies using restriction endonuclease mapping technique indicated that alpha-thalassemia determinants in these cases are heterogeneous. Only one of the five cases have the usual genotype ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830090304
更新日期:1980-01-01 00:00:00
abstract::Clinical management of lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM) has changed considerably over recent years, reflected in the use of new therapeutic agents (purine analogs, monoclonal antibodies, thalidomide- and bortezomib-based therapies). No population-based studies and few randomized tria...
journal_title:American journal of hematology
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doi:10.1002/ajh.23351
更新日期:2013-01-01 00:00:00
abstract:DISEASE OVERVIEW:Chronic neutrophilic leukemia (CNL) is a rare, often aggressive myeloproliferative neoplasm (MPN) defined by persistent mature neutrophilic leukocytosis, bone marrow granulocyte hyperplasia, and frequent hepatosplenomegaly. The seminal discovery of oncogenic driver mutations in colony-stimulating facto...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25688
更新日期:2020-02-01 00:00:00
abstract::Several histone deacetylase inhibitors (HDACi), including vorinostat, have been approved for the therapy of cutaneous T-cell lymphoma (CTCL). Emerging data suggest that HDACi may exert immune suppressive effects which would be disadvantageous for therapy of CTCL. We describe a patient with Sezary syndrome who was moni...
journal_title:American journal of hematology
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更新日期:2012-02-01 00:00:00
abstract::The objective of this study is to establish a method to identify patients with primary immune thrombocytopenia (ITP) utilizing administrative data from diverse data sources that would be appropriate for epidemiologic studies of ITP, regardless of patients' age and source of health care. Medical records of the Oklahoma...
journal_title:American journal of hematology
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更新日期:2012-09-01 00:00:00
abstract::Extensive extralymphatic Hodgkin disease developed in a young man with common variable immunodeficiency manifested by hypogammaglobulinemia, recurrent sinopulmonary infections, and multiple autoimmune phenomena. Both humoral and cell-mediated immune dysfunction were present prior to treatment. After two cycles of chem...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830320212
更新日期:1989-10-01 00:00:00
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
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doi:10.1002/(sici)1096-8652(200005)64:1<64::aid-ajh11>
更新日期:2000-05-01 00:00:00