D47.4
BillableOsteomyelofibrosis
HCC Category Mapping
V28HCC 19 — Lymphoma and Other Cancers
0.105V24HCC 46 — Severe Hematological Disorders
0.666ESRDHCC 46 — Severe Hematological Disorders
0.000RxHCCHCC 16 — Multiple Myeloma and Plasma Cell Neoplasms
0.000What This Code Means
A condition where bone marrow is replaced by fibrous tissue, leading to scarring and reduced blood cell production.
Coding Tips
- •Distinguish from primary myelofibrosis (PMF) which has its own code; this code is for uncertain behavior classification
- •Bone marrow biopsy showing fibrosis is typically required for diagnosis confirmation
Clinical Significance
Osteomyelofibrosis is characterized by progressive replacement of bone marrow with fibrous tissue, leading to severe cytopenias and extramedullary hematopoiesis in the spleen and liver. This condition results in debilitating symptoms including fatigue, night sweats, bone pain, and massive splenomegaly, and carries risk of transformation to acute myeloid leukemia.
Documentation Requirements
- ✓Documentation must include bone marrow biopsy demonstrating fibrosis (reticulin and/or collagen fibrosis grading), peripheral blood counts showing cytopenias or leukoerythroblastic picture, and spleen size.
- ✓Document constitutional symptoms, transfusion requirements, and JAK2/CALR/MPL mutation status.
- ✓The Dynamic International Prognostic Scoring System risk score should be calculated and documented.
Excludes 1 — Do NOT code together
- acute myelofibrosis (C94.4-)
Commonly Confused Codes
D47.1 (chronic myeloproliferative disease) is a broader category that may include myelofibrosis.D75.81 (myelofibrosis) may be used for secondary myelofibrosis.D45 (polycythemia vera) or D47.3 (essential thrombocythemia) may evolve into secondary myelofibrosis, which should be coded to this code if the marrow demonstrates fibrosis.C94.40 (acute panmyelosis with myelofibrosis) is an acute presentation.