Understanding Systemic Mastocytosis ICD-10 Codes And Diagnosis

What is Systemic Mastocytosis ICD 10?

Systemic mastocytosis is a rare disorder characterized by the abnormal accumulation of mast cells in various tissues and organs of the body. Mast cells are a type of white blood cell that play a role in the body’s immune response. In systemic mastocytosis, these mast cells are overproduced and accumulate in higher numbers than normal, leading to a range of symptoms and potential complications.

Code Information

ICD--CM Diagnosis Code D
ICD–CM Diagnosis Code D

The ICD-10 code for systemic mastocytosis is C96.2. This code is used to classify and track cases of systemic mastocytosis in medical records and health databases.

Diagnostic Related Groups (MS-DRG)

Global Classification of Mast Cell Activation Disorders: An ICD-
Global Classification of Mast Cell Activation Disorders: An ICD-

Systemic mastocytosis falls under MS-DRG 014 – Allogeneic Bone Marrow Transplant. This DRG is used to group cases of systemic mastocytosis that require a bone marrow transplant for treatment.

Convert to ICD-9 Code

In the previous ICD-9 coding system, systemic mastocytosis was classified under code 975.1. This code has been updated to C96.2 in the current ICD-10 coding system.

Code History

The ICD-10 code for systemic mastocytosis was first introduced in 2016 as part of the tenth revision of the International Classification of Diseases. This update brought about changes in the coding and classification of various medical conditions, including systemic mastocytosis.

Approximate Synonyms

Systemic mastocytosis is also known by other names and synonyms, including systemic mast cell disease, mast cell leukemia, and systemic mast cell disorder. These terms are used interchangeably to describe the same condition of abnormal mast cell accumulation.

Clinical Information

Systemic mastocytosis can present with a wide range of symptoms and severity, depending on the extent of mast cell accumulation and involvement of different organs. Common symptoms include skin rashes, itching, flushing, abdominal pain, diarrhea, and anaphylaxis.


The exact cause of systemic mastocytosis is not fully understood, but it is believed to be related to genetic mutations in the mast cells, leading to their uncontrolled growth and accumulation. Risk factors for developing systemic mastocytosis include a family history of the condition and certain genetic predispositions.


Patients with systemic mastocytosis may experience a variety of symptoms, ranging from mild to severe. These symptoms can include skin lesions, bone pain, gastrointestinal issues, cardiovascular problems, and neurological symptoms. The severity of symptoms can vary between individuals and may worsen over time.


Diagnosing systemic mastocytosis involves a combination of clinical evaluation, laboratory tests, imaging studies, and bone marrow biopsy. Blood tests can reveal elevated levels of mast cells and certain markers associated with the condition. A bone marrow biopsy may be necessary to confirm the diagnosis and assess the extent of mast cell involvement.


Treatment for systemic mastocytosis focuses on managing symptoms, preventing complications, and controlling mast cell proliferation. Therapies may include medications to reduce mast cell activity, alleviate symptoms, and prevent allergic reactions. In severe cases, bone marrow transplantation may be considered to replace abnormal mast cells with healthy cells.


Systemic mastocytosis is a rare disorder characterized by the abnormal accumulation of mast cells in various tissues and organs. The condition can present with a wide range of symptoms and severity, requiring a multidisciplinary approach to diagnosis and treatment. By understanding the code information, diagnostic criteria, clinical features, and treatment options for systemic mastocytosis, healthcare providers can better manage and care for patients with this complex condition.


Can systemic mastocytosis be cured? Currently, there is no cure for systemic mastocytosis. Treatment focuses on managing symptoms and preventing complications.
Is systemic mastocytosis hereditary? While systemic mastocytosis can have a genetic component, not all cases are inherited. Some cases may arise sporadically without a family history of the condition.
What are the long-term complications of systemic mastocytosis? Long-term complications of systemic mastocytosis can include organ damage, anaphylaxis, and systemic

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