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Case Reports in Hematology
Volume 2015, Article ID 737302, 4 pages
http://dx.doi.org/10.1155/2015/737302
Case Report

Serum Total Tryptase Level Confirms Itself as a More Reliable Marker of Mast Cells Burden in Mast Cell Leukaemia (Aleukaemic Variant)

1Department of Internal Medicine, Faenza Hospital, Viale Stradone 9, 48018 Faenza, Italy
2Department of Clinical Pathology, Centro Servizi Pievesestina, AUSL Romagna, Piazzale della Liberazione 60, Pievesestina, 47522 Cesena, Italy
3Department of Experimental, Diagnostic and Specialty Medicine, Institute of Hematology “L. e A. Seràgnoli”, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy

Received 16 August 2014; Revised 7 January 2015; Accepted 13 January 2015

Academic Editor: Stephen Langabeer

Copyright © 2015 P. Savini et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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.