Monoclonal mast cell activation syndrome
ICD-10 D89.41 is a billable code used to indicate a diagnosis of monoclonal mast cell activation syndrome.
Monoclonal mast cell activation syndrome (MMAS) is a hematologic condition characterized by an abnormal proliferation of mast cells that leads to excessive release of mediators, resulting in a range of symptoms. Patients may experience recurrent episodes of anaphylaxis, flushing, abdominal pain, and other systemic symptoms due to mast cell degranulation. The condition is often associated with other hematologic disorders, such as monoclonal gammopathy of undetermined significance (MGUS) or multiple myeloma. Diagnosis typically involves a combination of clinical evaluation, serum tryptase levels, and bone marrow biopsy to confirm the presence of abnormal mast cells. Management may include antihistamines, corticosteroids, and other medications to control symptoms and prevent severe reactions. Understanding the complexities of MMAS is crucial for accurate coding and appropriate patient management.
Detailed clinical notes, lab results, and treatment plans must be documented.
Patients presenting with recurrent anaphylaxis or unexplained systemic symptoms.
Ensure that the monoclonal aspect is clearly documented to avoid misclassification.
Comprehensive allergy history and response to treatments should be recorded.
Patients with suspected mast cell disorders presenting with allergic symptoms.
Documenting the response to antihistamines and other treatments is crucial.
Used to evaluate blood cell counts and assess for mast cell activation.
Document the reason for the CBC and any relevant findings.
Hematologists should ensure that the CBC is interpreted in the context of MMAS.
Key symptoms include recurrent anaphylaxis, flushing, abdominal pain, and other systemic reactions due to mast cell degranulation.