5 Αποτελέσματα
Systemic mastocytosis is a disorder caused by clonal mast cell proliferation and release of mast cell mediators including tryptase. As a result, mast cell numbers may increase and affect target organs including the dermis (maculopapular cutaneous mastocytosis/urticaria pigmentosa, flushing),
PRIMARY OBJECTIVE:
Evaluate the response rate to ibrutinib in patients with advanced systemic mastocytosis (SM) (aggressive systemic mastocytosis [ASM] or mast cell leukemia [MCL], or SM-associated hematologic non-mast cell disorder [AHNMD]) by the end of 6 cycles (6 months).
SECONDARY OBJECTIVES:
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The study will take place as double-blind placebo controlled study. After a first a run-period of 2 months for all participants randomization (1:1) in two group will take place:
Group A: With omalizumab treatment for 6 months; dosage and administration schedule according to body weight and total IgE
Mastocytosis in infants and children is an unusual disease characterized by an excess of mast cells in tissues. In pediatric onset mastocytosis, disease is usually localized to the skin and disease is considered to be of limited duration. However, a subset of children appear to develop a clinical
This protocol is designed to examine those growth potentiating and inhibiting factors which regulate mast cell number in patients with mastocytosis, and to explore the molecular basis of the disease process in hopes of improving therapy. Patients entered into the study are seen initially and may