BJH - volume 5, issue Abstract Book BHS, january 2014
J. Caers MD, PhD, M.C. Vekemans MD, I. Vande Broek MD, PhD, V. Maertens MD, P.H. Mineur , G. Bries MD, PhD, E. Vandeneste , G. Vanstraelen , K. Beel MD, PhD, F. Leleu , H. Demuynck MD, C. Scheurmans , A. Van De Velde MD, PhD, W. Schroyens MD, PhD, K.L. Wu MD, PhD, N. Meuleman MD, PhD, R. Schots MD, PhD, M. Delforge MD, PhD, C. Doyen MD
BJH - volume 4, issue 3, september 2013
G. Deslypere MD, T. Devos MD, PhD, M. Delforge MD, PhD, G. Verhoef MD, PhD
Systemic mastocytosis is an orphan myeloproliferative disease characterised by an excessive mast cell accumulation. Benign forms present with urticaria pigmentosa while aggressive subtypes or leukaemic variants lead to organ dysfunction. In patients with unexplained hypotensive syncope’s or anaphylaxis, flushing and angio-oedema with a basal tryptase >20 ng/mL, one should think of systemic mastocytosis. Pathophysiology is based on mutations in KIT, encoding the c-kit receptor (CD117) on the surface of mast cells. Diagnosis is based on bone marrow biopsies with clusters of atypical mast cells and co-expression of CD2 or CD25 and/or a KIT mutation. Treatment consists of avoiding triggers of mast cell release and antihistaminic drugs. Patients with aggressive subtypes are candidates for cytoreductive therapies. CD30 is thought to be a novel predictor of prognosis.
(BELG J HEMATOL 2013;4(3):85–89)
Read moreBJH - 2013, issue BHS Abstractbook, january 2013
J. Caers MD, PhD, M.C. Vekemans MD, I. Vande Broek MD, PhD, P.H. Mineur , K. Beel MD, PhD, V. Maertens MD, C. Schuermans MD, F. Leleu , G. Vanstraelen , H. Demuynck MD, W. Schroyens MD, PhD, E. Van den Neste MD, PhD, G. Bries MD, PhD, A. Van De Velde MD, PhD, M. Delforge MD, PhD, C. Doyen MD
BJH - 2013, issue BHS Abstractbook, january 2013
H. Maes MD, G. Verhoef MD, PhD, D. Kuypers , P. Schöffski , T. Tousseyn MD, PhD, M. Delforge MD, PhD, T. Devos MD, PhD, A. Janssens MD, PhD, J. Maertens MD, PhD, H. Schoemans MD, PhD, D. Dierickx MD, PhD
Top
To provide the best experiences, we and our partners use technologies like cookies to store and/or access device information. Consenting to these technologies will allow us and our partners to process personal data such as browsing behavior or unique IDs on this site and show (non-) personalized ads. Not consenting or withdrawing consent, may adversely affect certain features and functions.
Click below to consent to the above or make granular choices. Your choices will be applied to this site only. You can change your settings at any time, including withdrawing your consent, by using the toggles on the Cookie Policy, or by clicking on the manage consent button at the bottom of the screen.
