D75.81

Billable

Myelofibrosis

HCC Category Mapping

V28HCC 19 — Lymphoma and Other Cancers

0.105

V24HCC 46 — Severe Hematological Disorders

0.666

ESRDHCC 46 — Severe Hematological Disorders

0.000

RxHCCHCC 16 — Multiple Myeloma and Plasma Cell Neoplasms

0.000

What This Code Means

A condition where bone marrow becomes scarred and fibrotic, leading to reduced blood cell production and potential anemia or bleeding problems.

Coding Tips

•Myelofibrosis can be primary or secondary; verify if this is primary myelofibrosis or secondary to another condition
•Document the stage of myelofibrosis if available, as this affects treatment and prognosis

Clinical Significance

Myelofibrosis is a chronic myeloproliferative neoplasm characterized by progressive bone marrow fibrosis, extramedullary hematopoiesis (splenomegaly, hepatomegaly), and peripheral blood cytopenias. It may be primary (PMF) or secondary to polycythemia vera or essential thrombocythemia. The disease carries risk of transformation to acute myeloid leukemia. JAK2, CALR, or MPL driver mutations are found in approximately 90% of cases.

Documentation Requirements

✓Document bone marrow biopsy findings showing fibrosis grading (MF-0 to MF-3), driver mutation status (JAK2 V617F, CALR, MPL), complete blood count with differential, peripheral blood smear findings (leukoerythroblastic picture, teardrop cells), spleen size, and prognostic risk score (DIPSS, MIPSS70).
✓Record current treatment (JAK inhibitors like ruxolitinib, transfusion support, hydroxyurea).

Excludes 1 — Do NOT code together

acute myelofibrosis (C94.4-)
idiopathic myelofibrosis (D47.1)
leukoerythroblastic anemia (D61.82)
myelofibrosis with myeloid metaplasia (D47.4)
myelophthisic anemia (D61.82)
myelophthisis (D61.82)
primary myelofibrosis (D47.1)

Use Additional Code

code, if applicable, for associated therapy-related myelodysplastic syndrome (D46.-)
code for adverse effect, if applicable, to identify drug (T45.1X5)

Code First

the underlying disorder, such as:
malignant neoplasm of breast (C50.-)

Commonly Confused Codes

C94.4x (Acute panmyelosis with myelofibrosis) — acute presentation, not chronic D47.1 (Chronic myeloproliferative disease, not classified elsewhere) — broader category C91-C95 (Leukemia codes) — if transformation to AML occurs D61.9 (Aplastic anemia) — may mimic fibrotic-phase myelofibrosis.

Code Hierarchy

└D75Other and unspecified diseases of blood and blood-forming organs └D75.8Other specified diseases of blood and blood-forming organs └D75.81Myelofibrosis

└D75.81Myelofibrosis