Systemic mastocytosis
ICD-10 D47.02 is a billable code used to indicate a diagnosis of systemic mastocytosis.
Systemic mastocytosis is a rare hematologic disorder characterized by the abnormal proliferation and accumulation of mast cells in various tissues, including the bone marrow, skin, liver, spleen, and gastrointestinal tract. This condition can present with a variety of symptoms, including skin lesions, flushing, abdominal pain, and anaphylaxis due to mast cell degranulation. The neoplastic behavior of systemic mastocytosis is classified as uncertain, as it can range from indolent forms with minimal symptoms to aggressive variants that may lead to organ dysfunction and systemic complications. Diagnosis typically involves a combination of clinical evaluation, serum tryptase levels, and bone marrow biopsy to confirm the presence of mast cell infiltration. Surveillance is crucial due to the potential for progression to more severe forms of mastocytosis, including mast cell leukemia. Management strategies may include symptomatic treatment, such as antihistamines and corticosteroids, and in some cases, targeted therapies like imatinib or midostaurin. Regular follow-up is essential to monitor for disease progression and manage symptoms effectively.
Detailed clinical notes on symptoms, diagnostic tests, and treatment plans.
Diagnosis confirmation through bone marrow biopsy, management of symptoms, and monitoring for progression.
Ensure accurate staging and classification of mastocytosis type for appropriate coding.
Records of allergic reactions, symptom management, and response to antihistamines.
Management of anaphylactic reactions and chronic urticaria related to mast cell activation.
Documenting the relationship between mastocytosis and allergic symptoms is crucial for accurate coding.
Used to monitor blood parameters in patients with systemic mastocytosis.
Document the reason for the CBC and any abnormal findings.
Hematology specialists should ensure comprehensive lab results are included.
The primary treatment focuses on managing symptoms, which may include antihistamines for flushing and itching, corticosteroids for inflammation, and in some cases, targeted therapies like imatinib for more aggressive forms.
