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Blood & Immune

Mastocytosis

Also called mast cell disease, systemic mastocytosis

Mastocytosis is a clonal myeloproliferative disorder characterized by accumulation and proliferation of abnormal mast cells in bone marrow, skin, and other organs. Most cases involve KIT proto-oncogene mutations (D816V) that drive mast cell expansion.

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About trials for Mastocytosis

Clinical trials are evaluating tyrosine kinase inhibitors (KIT inhibitors) and other targeted therapies that inhibit mast cell proliferation and mediator release. Multiple agents are in development for different mastocytosis forms.

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About Mastocytosis

Mastocytosis is a clonal myeloproliferative disorder characterized by accumulation and proliferation of abnormal mast cells in bone marrow, skin, and other organs. Most cases involve KIT proto-oncogene mutations (D816V) that drive mast cell expansion. Cutaneous mastocytosis (urticaria pigmentosa) is the most common form, particularly in children, characterized by brown lesions on skin that urticariate when irritated. Systemic mastocytosis involves bone marrow and organ involvement and is more common in adults. Patients experience symptoms from mast cell mediator release (histamine, tryptase, etc.) including flushing, pruritus, diarrhea, anaphylaxis, and bone disease from marrow infiltration. Anaphylactic reactions can be triggered by foods, insect venom, NSAIDs, opioids, and other medications, making treatment challenging.

Common Symptoms

  • Flushing, pruritus (itching), and urticaria
  • Abdominal pain, diarrhea, and nausea
  • Anaphylactic reactions triggered by food, insect venom, or medications
  • Headaches
  • Bone pain or pathologic fractures from bone marrow involvement
  • Hepatosplenomegaly and lymphadenopathy

Who It Affects

Can present at any age; pediatric-onset cutaneous mastocytosis is most common, while systemic disease more common in adults. Affects males and females approximately equally. Sporadic disease is most common.

Getting Involved in Clinical Trials

Clinical trials are evaluating tyrosine kinase inhibitors (KIT inhibitors) and other targeted therapies that inhibit mast cell proliferation and mediator release. Multiple agents are in development for different mastocytosis forms. The Mastocytosis Society and patient organizations provide information on trials and disease management. If you have systemic mastocytosis with KIT D816V mutation, discuss with your hematologist about tyrosine kinase inhibitor therapy, which can be highly effective. Strict avoidance of known triggers (foods, medications, temperature extremes) is important. Prophylactic medications (antihistamines, mast cell stabilizers) help prevent symptoms and anaphylaxis.

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