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SEER Inquiry System - Report
Produced: 09/16/2026 1:13 AM
Question 20260017
Inquiry Details
References:
Heme & Lymph Manual & DB. October 2025 update
Question:
Multiple Primaries/Histology--Heme & Lymphoid Neoplasms: Should histology be coded as 9895 when the pathologist's diagnosis on the bone marrow biopsy is "consistent with transformation of the patient's known polycythemia vera to acute myeloid leukemia?" See Discussion.
Discussion:
The patient has a known history of polycythemia vera (PV). The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) entry for myelodysplasia-related acute myeloid leukemia ("acute myeloid leukemia post myelodysplastic-myeloproliferative neoplasm") states, "The pathologist must make the diagnosis of being myelodysplasia-related." However, it's unclear if the pathologist's statement of an acute myeloid leukemia (AML) "transforming from" counts as the AML being myelodysplasia-related since AML can arise/transform from PV. The Heme DB does indicate histology 9895 should not automatically be used just because the patient has a history of MPN.
The bone marrow did not identify any of the specific cytogenetic or molecular abnormalities listed in the Heme DB. However, next generation sequencing identified mutations in BCOR, DNMT3A, IDH1, JAK2, EZH2. The managing physician's clinical diagnosis was "AML arising from polycythemia vera."
Answer:
Assign a second primary as Acute Myeloid Leukemia, NOS (9861/3), per Rule M10, the first being polycythemia vera (9950/3). The physician states “consistent with transformation of the patient's known polycythemia vera to acute myeloid leukemia.” This is not the same thing as “Acute myeloid leukemia with myelodysplasia-related changes.”
The Heme DB states:
Do not automatically assign this histology (Acute myeloid leukemia with myelodysplasia-related changes (9895/3) if patient has a history of a myeloproliferative neoplasm (Myelodysplastic Syndrome [MDS], or Myeloproliferative Neoplasm [MPN]). Note: Polycythemia Vera is a Myeloproliferative Neoplasm (MPN). The pathologist must make the diagnosis of being myelodysplasia-related.
