D47.4
Osteomyelofibrosis
Clinical Classification Guidelines
Inclusion Terms
- Chronic idiopathic myelofibrosis
- Myelofibrosis (idiopathic) (with myeloid metaplasia)
- Myelosclerosis (megakaryocytic) with myeloid metaplasia
- Secondary myelofibrosis in myeloproliferative disease
Excludes Type 1
- acute myelofibrosis (C94.4-)
Medical Intelligence & Overview
Osteomyelofibrosis, also known as myelofibrosis, is a rare bone marrow disorder that disrupts normal blood cell production. This condition leads to the replacement of healthy marrow tissue with fibrous scar tissue, impairing the development of blood cells and causing a range of health issues. It is categorized under ICD-10 code D47.4 and can occur as a primary disease or develop secondary to other myeloproliferative disorders.
Causes & Symptoms
Clinical Causes: Primary idiopathic myelofibrosis with no known cause Secondary fibrosis due to other myeloproliferative diseases such as polycythemia vera or essential thrombocythemia Myeloid metaplasia, where abnormal blood cell growth occurs outside the marrow, particularly in the spleen and liver Chronic activation and fibrosis of marrow tissue over time
Key Symptoms: Fatigue and weakness Enlarged spleen and liver (splenomegaly and hepatomegaly) Bone pain or tenderness Unexplained weight loss Night sweats Easy bruising or bleeding Anemia-related symptoms such as pallor and shortness of breath Gout or painful joint swelling due to increased cell turnover
Diagnostic & Treatment
Diagnosis Path: Diagnosis involves a combination of blood tests, bone marrow biopsy, and imaging studies. Blood tests may reveal anemia, abnormal blood cell counts, or other irregularities. Bone marrow biopsy confirms the degree of fibrosis and the extent of marrow damage. Additional tests help distinguish primary myelofibrosis from secondary causes due to other myeloproliferative conditions.
Treatment Protocols: While there is no cure for osteomyelofibrosis, treatment aims to manage symptoms and improve quality of life. Approaches include medications such as corticosteroids, chemotherapy, and targeted therapies to reduce fibrosis and control blood counts. Blood transfusions may be necessary for anemia. Certain cases might require splenectomy or stem cell transplantation. Supportive care, including managing infections and preventing bleeding, is also essential.
Clinical Advice & FAQs
Billing Guidance
Is D47.4 a billable ICD-10 code?
Yes, D47.4 is a specific, billable code that can be used to indicate a diagnosis for reimbursement purposes.
Documentation
How do I report D47.4?
Clinical documentation must specify the nature of Osteomyelofibrosis and any associated comorbidities for accurate reporting.
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