Short answer · Medically reviewed summary · Last updated: 2026-05-08
The primary ICD-10-CM code for primary myelofibrosis is D47.4 (Osteomyelofibrosis). Under the older ICD-9-CM classification system, primary myelofibrosis was categorized as 289.83 (Myelofibrosis with myeloid metaplasia). What is the clinical classification of Myelofibrosis? Myelofibrosis is a rare type of chronic myeloproliferative neoplasm where the bone marrow is replaced by scar tissue, leading to abnormal blood cell production.
ICD10 code of Myelofibrosis and ICD9 code
ICD-10 and ICD-9 codes for Myelofibrosis, with classification details for clinicians, coders and patients.
The primary ICD-10-CM code for primary myelofibrosis is D47.4 (Osteomyelofibrosis). Under the older ICD-9-CM classification system, primary myelofibrosis was categorized as 289.83 (Myelofibrosis with myeloid metaplasia).
What is the clinical classification of Myelofibrosis?
Myelofibrosis is a rare type of chronic myeloproliferative neoplasm where the bone marrow is replaced by scar tissue, leading to abnormal blood cell production. Because myelofibrosis can be either primary or secondary (arising from other conditions like polycythemia vera or essential thrombocythemia), clinicians must be precise with coding to ensure accurate medical records and insurance coverage for treatments.
How is Myelofibrosis coded for billing and research?
While D47.4 is the standard code for primary myelofibrosis, medical coding can become complex if the disease is secondary to another condition. In the 16 members of our DiseaseMaps.org community living with myelofibrosis, we often see that tracking these specific codes is essential for patients navigating complex hematology-oncology care pathways.
What are the key diagnostic criteria for Myelofibrosis?
Diagnosis of myelofibrosis typically involves a combination of clinical, laboratory, and genetic assessments. The following elements are crucial for a definitive diagnosis:
- Bone Marrow Biopsy: Demonstrating grade 2 or 3 fibrosis of the marrow.
- Genetic Testing: Identifying mutations in genes such as JAK2, CALR, or MPL (found in approximately 90% of patients).
- Clinical Presentation: Presence of anemia, splenomegaly (enlarged spleen), and constitutional symptoms like fatigue or night sweats.
- Laboratory Findings: Peripheral blood smear showing leukoerythroblastosis and teardrop-shaped red blood cells.
Next steps
- Consult with a hematologist or an MPN (myeloproliferative neoplasm) specialist to review your current diagnostic codes.
- Request a copy of your pathology report to confirm the grading of your myelofibrosis.
- Join the myelofibrosis patient community at DiseaseMaps.org to connect with others who understand the diagnostic journey.
- Ask your care team about clinical trials for emerging JAK inhibitors or novel therapies.
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Myelofibrosis
- Orphanet: Primary Myelofibrosis (ORPHA: 602)
- World Health Organization (WHO) Classification of Tumours of Haematopoietic and Lymphoid Tissues
- MPN Research Foundation: Understanding Myelofibrosis
