Search Database ICD-O-3 Code Lists

Name

Acute panmyelosis with myelofibrosis (APMF)

ICD-O-3 Morphology

9931/3: Acute panmyelosis with myelofibrosis
Effective 2001 and later

Reportable

for cases diagnosed 1992 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Acute panmyelosis with myelofibrosis (APMF) is part of the Acute myeloid leukemia (AML) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B6)

This is primarily a disease of adults but has been reported in children. Bone marrow aspiration is usually unsuccessful; either no BM is obtained or the specimen is suboptimal. Bone marrow biopsy supplemented with immunohistology is required for a diagnosis.

If an AML and myeloid sarcoma (9930/3) occur during the same clinical workup, this is one primary, the leukemia.

If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.

See Multiple Primary Rule M3

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Acute myelosclerosis, NOS
Acute panmyelosis, NOS
Malignant myelofibrosis

Definition

Acute panmyelosis with myelofibrosis (APMF) is an acute panmyeloid proliferation with increased blasts (greater than or equal to 20% of cells in the bone marrow or peripheral blood) and accompanying fibrosis of the bone marrow.

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

-5/del(5q)
-7/del(7q)

Immunophenotyping

CD13+ (expression/positive)
CD33+ (expression/positive)
CD34+ (expression/positive)
KIT (CD117)+ (expression/positive)
Myeloid hematopoietic stem cell

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C94.4 Acute panmyelosis
C94.5 Acute myelofibrosis

Corresponding ICD-10-CM Codes (U.S. only)

C94.4 Acute panmyelosis with myelofibrosis (effective October 01, 2015 - September 30, 2024)
C94.4 Acute panmyelosis with myelofibrosis not having achieved remission (effective October 01, 2025)
C94.4 Acute panmyelosis with myelofibrosis, in remission (effective October 01, 2025)
C94.4 Acute panmyelosis with myelofibrosis, in relapse (effective October 01, 2025)

Signs and Symptoms

Bone pain
Easy bruising or bleeding
Fatigue
Fever
Pancytopenia
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Reverse transcription-polymerase chain reaction test (RT-PCR)

Progression and Transformation

None

Epidemiology and Mortality

Age: primarily in adults but does occur in children
Incidence: very rare form of AML
Survival: few months

Sources

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Acute Myeloid Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <02/12/2025>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq. Accessed <02/28/2025>. [PMID: 26389432]
Section: Acute Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Acute myeloid leukemia and related precursor neoplasms
Pages: 165-166
Glossary