In simple words: Aggressive systemic mastocytosis (ASM) is a rare disease where too many mast cells (a type of white blood cell) grow in the body. These cells build up in tissues and organs like the spleen, liver, and bone marrow. They release a substance called histamine, causing allergic reactions like itching, rashes, and stomach problems.

Aggressive systemic mastocytosis (ASM) involves the uncontrolled multiplication of mast cells, which accumulate in body tissues and may impact organs such as the spleen, small intestine, liver, and bone marrow. These mast cells release excessive histamine, leading to allergic reactions in the affected tissues.

Example 1: A patient presents with severe itching, abdominal pain, recurring rashes, and flushing. After laboratory tests and a bone marrow biopsy, they are diagnosed with aggressive systemic mastocytosis., A patient experiences persistent diarrhea, nausea, vomiting, and abdominal discomfort. Further investigation reveals organ enlargement and dysfunction, leading to a diagnosis of ASM., A patient with known mastocytosis develops worsening symptoms and organ involvement, indicating a progression to aggressive systemic mastocytosis.

Documentation should include patient history, physical exam findings, laboratory results (CBC, peripheral smear, blood chemistries, serum tryptase levels), bone marrow biopsy or fine needle aspiration findings, and imaging study results (DEXA scan, ultrasound). Details of symptom management, including medications like topical steroids and antihistamines, should also be documented.

** ASM carries a poor prognosis. There is no specific cure, and treatment focuses on managing symptoms and complications.