دورية أكاديمية
أعرض في EDS

Serum total tryptase level confirms itself as a more reliable marker of mast cells burden in mast cell leukaemia (aleukaemic variant).

التفاصيل البيبلوغرافية
العنوان: Serum total tryptase level confirms itself as a more reliable marker of mast cells burden in mast cell leukaemia (aleukaemic variant).
المؤلفون: Savini P; Department of Internal Medicine, Faenza Hospital, Viale Stradone 9, 48018 Faenza, Italy., Rondoni M; Department of Clinical Pathology, Centro Servizi Pievesestina, AUSL Romagna, Piazzale della Liberazione 60, Pievesestina, 47522 Cesena, Italy., Poletti G; Department of Clinical Pathology, Centro Servizi Pievesestina, AUSL Romagna, Piazzale della Liberazione 60, Pievesestina, 47522 Cesena, Italy., Lanzi A; Department of Internal Medicine, Faenza Hospital, Viale Stradone 9, 48018 Faenza, Italy., Quercia O; Department of Internal Medicine, Faenza Hospital, Viale Stradone 9, 48018 Faenza, Italy., Soverini S; Department of Experimental, Diagnostic and Specialty Medicine, Institute of Hematology 'L. e A. Seràgnoli', University of Bologna, Via Massarenti 9, 40138 Bologna, Italy., De Benedittis C; Department of Experimental, Diagnostic and Specialty Medicine, Institute of Hematology 'L. e A. Seràgnoli', University of Bologna, Via Massarenti 9, 40138 Bologna, Italy., Musardo G; Department of Internal Medicine, Faenza Hospital, Viale Stradone 9, 48018 Faenza, Italy., Martinelli G; Department of Experimental, Diagnostic and Specialty Medicine, Institute of Hematology 'L. e A. Seràgnoli', University of Bologna, Via Massarenti 9, 40138 Bologna, Italy., Stefanini GF; Department of Internal Medicine, Faenza Hospital, Viale Stradone 9, 48018 Faenza, Italy.
المصدر: Case reports in hematology [Case Rep Hematol] 2015; Vol. 2015, pp. 737302. Date of Electronic Publication: 2015 Feb 10.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Hindawi Pub. Corp Country of Publication: United States NLM ID: 101576456 Publication Model: Print-Electronic Cited Medium: Print ISSN: 2090-6560 (Print) Linking ISSN: 20906579 NLM ISO Abbreviation: Case Rep Hematol Subsets: PubMed not MEDLINE
أسماء مطبوعة: Original Publication: New York, NY : Hindawi Pub. Corp.
مستخلص: Mast cell leukemia (MCL) is a very rare form of systemic mastocytosis (SM) with a short median survival of 6 months. We describe a case of a 65-year-old woman with aleukaemic variant of MCL with a very high serum total tryptase level of 2255 μg/L at diagnosis, which occurred following an episode of hypotensive shock. She fulfilled the diagnostic criteria of SM, with a bone marrow smear infiltration of 50-60% of atypical mast cells (MCs). She tested negative for the KIT D816V mutation, without any sign of organ damage (no B- or C-findings) and only few mediator-related symptoms. She was treated with antihistamine alone and then with imatinib for the appearance of anemia. She maintained stable tryptase level and a very indolent clinical course for twenty-two months; then, she suddenly progressed to acute MCL with a serum tryptase level up to 12960 μg/L. The patient died due to haemorrhagic diathesis twenty-four months after diagnosis. This clinical case maybe represents an example of the chronic form of mast cell leukemia, described as unpredictable disease, in which the serum total tryptase level has confirmed itself as a reliable marker of mast cells burden regardless of the presence of other signs or symptoms.
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تواريخ الأحداث: Date Created: 20150311 Date Completed: 20150310 Latest Revision: 20201001
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC4338389
DOI: 10.1155/2015/737302
PMID: 25755899
قاعدة البيانات: MEDLINE
الوصف
تدمد:2090-6560
DOI:10.1155/2015/737302