Abstract:
:Mastocytosis is a heterogeneous disease caused by excessive mast cell (MC) proliferation. Diagnosis of systemic mastocytosis (SM) is based on the presence of major and minor criteria defined by the World Health Organization. Symptoms of MC activation can also occur in patients without SM or without allergic or inflammatory disease. These MC activation syndromes (MCAS) can be divided into primary (monoclonal) MCAS (MMAS) vs. secondary and idiopathic MCAS. In this single center study, the diagnostic work-up of 38 patients with a clinical suspicion of SM and/or with elevated basic tryptase levels is presented. Clinical symptoms, biochemical parameters, results of bone marrow investigation, flow cytometric immunophenotyping, and molecular analysis were retrospectively reviewed. Twenty-three patients were found to have a monoclonal MC disorder of which 19 were diagnosed with SM and 4 with MMAS. In 13/19 SM patients, multifocal MC infiltrates in the bone marrow were found (major criterion), while in 6 the diagnosis was based on the presence of ≥3 minor criteria. Flow cytometric analysis of bone marrow showed CD25 expression of MCs in all patients with SM and MMAS (range: 0.002-0.3% of cells). In bone marrow, the KIT D816V mutation was detected in all SM patients but in only 2 patients with MMAS (range: 0.007-9% mutated cells). Basic tryptase elevation was demonstrated in 16/19 patients with SM but also in 9/19 patients without SM. Our study reveals the heterogeneity of primary MC disorders and the importance of sensitive assays in patients suspected of having SM.
journal_name
Acta Clin Belgjournal_title
Acta clinica Belgicaauthors
Van den Poel B,Kochuyt AM,Del Biondo E,Dewaele B,Lierman E,Tousseyn T,de Hertogh G,Vandenberghe P,Boeckx Ndoi
10.1080/17843286.2017.1293312subject
Has Abstractpub_date
2017-04-01 00:00:00pages
123-129issue
2eissn
1784-3286issn
2295-3337journal_volume
72pub_type
杂志文章abstract::We present the updated Belgian guidelines for the use of non-occupational HIV post-exposure prophylaxis (NONOPEP). This document is inspired by UK guidelines 2015, adapted to the Belgian situation and approved by all AIDS reference centers in Belgium. When recommended, NONOPEP should be initiated as soon as possible, ...
journal_title:Acta clinica Belgica
pub_type: 杂志文章,实务指引
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journal_title:Acta clinica Belgica
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doi:10.1179/acb.2010.65.6.011
更新日期:2010-11-01 00:00:00
abstract::Primary sclerosing cholangitis (PSC) and idiopathic pulmonary fibrosis (IPF) were each separately described in association with a variety of chronic idiopathic inflammatory diseases such as retroperitoneal fibrosis, Sicca complex or Riedel's thyroiditis. We report a case which may be the first description of the assoc...
journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1080/17843286.1989.11717982
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abstract::The kidneys play a major role in the regulation of the salt balance and thereby regulate blood pressure. Salt sensitivity is acquired or genetically-induced and is noted in about 50% of patients with essential hypertension. This property leads to a high cardiovascular risk. In this situation, the benefit of salt restr...
journal_title:Acta clinica Belgica
pub_type: 杂志文章,评审
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abstract::Nonalcoholic steatohepatitis (NASH), along with other forms of nonalcoholic fatty liver disease, is an increasingly common clinico-pathological syndrome. It is frequently associated with obesity, especially visceral fat, and type 2 diabetes, and is intimately related to markers of the insulin resistance syndrome. Both...
journal_title:Acta clinica Belgica
pub_type: 社论
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abstract::Various treatments for essential hyperhidrosis are available. The aim of this study is to present our experience with a simplified thoracoscopic sympathicolysis technique in this disorder, and to confront our results with data in the literature, 35 consecutive patients (11 male, 24 female, age 12-44 years) with essent...
journal_title:Acta clinica Belgica
pub_type: 杂志文章,评审
doi:10.1080/22953337.1996.11718517
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abstract::We report a case of aneurysm of the abdominal aorta with spontaneous rupture in a 64-year-old man with emphysema and chronic alcoholism. Campylobacter fetus subspecies fetus was repeatedly recovered from the blood and from specimens of the excised aneurysm. The patient died in spite of early diagnosis, intravenous ery...
journal_title:Acta clinica Belgica
pub_type: 杂志文章,评审
doi:10.1080/17843286.1989.11717999
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1179/0001551213Z.00000000016
更新日期:2014-04-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1080/17843286.2020.1790867
更新日期:2020-07-04 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章,评审
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更新日期:2010-09-01 00:00:00
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journal_title:Acta clinica Belgica
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doi:10.2143/ACB.67.4.2062679
更新日期:2012-07-01 00:00:00
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pub_type: 杂志文章
doi:10.1179/acb.2010.009
更新日期:2010-01-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1080/17843286.1976.11717057
更新日期:1976-01-01 00:00:00
abstract:BACKGROUND:Automated insulin calculators (AICs) with carbohydrate counting (CHC) have been shown to be effective in improving glycated haemoglobin (HbA1c) levels. By contrast, use of AICs without CHC, with predetermined prandial insulin doses modified according to a correction factor and modulated as a function of glyc...
journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1080/17843286.2015.1116151
更新日期:2016-02-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:
更新日期:1999-01-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1080/17843286.2019.1680134
更新日期:2019-10-15 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章,评审
doi:10.1179/acb.2003.58.5.009
更新日期:2003-09-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1179/acb.2007.62.s1.007
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1179/acb.2001.053
更新日期:2001-11-01 00:00:00
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doi:10.1179/acb.2004.033
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journal_title:Acta clinica Belgica
pub_type: 杂志文章,评审
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更新日期:1991-01-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.2143/ACB.66.4.2062566
更新日期:2011-07-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1179/acb.2008.030
更新日期:2008-05-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.2143/ACB.66.4.2062576
更新日期:2011-07-01 00:00:00
abstract:OBJECTIVE AND IMPORTANCE:Sarcoidosis is a multisystem inflammatory disorder of unknown etiology. It is characterized by the presence of noncaseating granulomas and an inflammatory process in which T lymphocytes, especially type-1 helper T (Th1) cells, macrophages and different cytokines are involved. Different studies ...
journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1080/17843286.2016.1152671
更新日期:2016-12-01 00:00:00
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journal_title:Acta clinica Belgica
pub_type: 杂志文章,评审
doi:
更新日期:2007-01-01 00:00:00
abstract::We present a case of cutaneous mucormycosis in a patient with several important risk factors precipitating disease, namely underlying acute myeloid leukaemia and poorly controlled secondary diabetes. Inoculation was most likely caused by repeated minor trauma (insulin injection) at the site of infection. Treatment con...
journal_title:Acta clinica Belgica
pub_type: 杂志文章,评审
doi:10.1080/17843286.2016.1266802
更新日期:2017-10-01 00:00:00
abstract::The last decade an increase has been seen in drug smuggling. Body-packing and body-stuffing are the terms used for intracorporeal concealment of illicit drugs (mainly cocaine and heroine, but sometimes also amphetamines and cannabinoids). These body-packets are especially prone to rupture. In order to avoid systemic c...
journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.1080/17843286.1994.11718357
更新日期:1994-01-01 00:00:00
abstract::A tired 32-year-old woman complaining of tiredness was referred for work-up of a possible immune deficiency. She had a history of recurrent infections since birth, which usually responded to antibiotics within a few days. Her mother, a nurse, had reported that early charts had disappeared. Munchausen's by proxy was su...
journal_title:Acta clinica Belgica
pub_type: 杂志文章
doi:10.2143/ACB.66.2.2062531
更新日期:2011-03-01 00:00:00