Learn About Systemic Mastocytosis
Systemic mastocytosis is a blood disorder that can affect many different body systems. Individuals with the condition can develop signs and symptoms at any age, but it usually appears after adolescence.
Systemic mastocytosis occurs when white blood cells called mast cells, which are produced in bone marrow, abnormally accumulate in one or more tissues. In most cases of systemic mastocytosis, the accumulated mast cells have a mutation in a gene called KIT. The KIT gene provides instructions for making a protein that plays an important role in development and activity of mast cells. The KIT protein stimulates chemical signaling pathways that are involved in the growth and division (proliferation) of many types of cells, including mast cells. In systemic mastocytosis, KIT gene mutations are somatic, which means they are acquired during a person's lifetime. These mutations result in a KIT protein that is always turned on (activated). As a result, signaling pathways are overactive, leading to increased production and accumulation of mast cells.
Systemic mastocytosis is estimated to occur in 1 per 10,000 to 20,000 individuals worldwide.
This condition is generally not inherited but arises from a somatic mutation in the body's cells that occurs after conception.
Dana-Farber Cancer Institute, Inc.
Daniel Deangelo is a Hematologist Oncology specialist and a Hematologist in Boston, Massachusetts. Dr. Deangelo is rated as an Elite provider by MediFind in the treatment of Systemic Mastocytosis. His top areas of expertise are Systemic Mastocytosis, Acute Myeloid Leukemia (AML), Leukemia, Bone Marrow Aspiration, and Bone Marrow Transplant. Dr. Deangelo is currently accepting new patients.
Stanford Medicine Cancer Center
Jason Gotlib is a Hematologist in Palo Alto, California. Dr. Gotlib is rated as an Elite provider by MediFind in the treatment of Systemic Mastocytosis. His top areas of expertise are Systemic Mastocytosis, Mast Cell Activation Syndrome (MCAS), Myelofibrosis, Bone Marrow Aspiration, and Tissue Biopsy. Dr. Gotlib is currently accepting new patients.
Peter Valent practices in Vienna, Austria. Mr. Valent is rated as an Elite expert by MediFind in the treatment of Systemic Mastocytosis. His top areas of expertise are Mast Cell Activation Syndrome (MCAS), Systemic Mastocytosis, Leukemia, Chronic Myelogenous Leukemia (CML), and Bone Marrow Transplant.
Summary: This phase II trial studies how well naive T-cell depletion works in preventing chronic graft-versus-host disease in children and young adults with blood cancers undergoing donor stem cell transplant. Sometimes the transplanted white blood cells from a donor attack the body's normal tissues (called graft versus host disease). Removing a particular type of T cell (naive T cells) from the donor cell...
Summary: Systemic mastocytosis with an associated hematologic neoplasm (SM-AHN) is a challenging disease to treat. Targeted KIT inhibitors have been approved for this indication based on their ability to control the mastocytosis portion of the disease, but patients frequently experience progression of the concomitant myeloid malignancy (i.e. the AHN). Using a combination approach to treat both aspects of t...
Published Date: October 01, 2018
Published By: National Institutes of Health