Name

Acute panmyelosis with myelofibrosis

ICD-O-1 Morphology

9951/1: Acute panmyelosis
Effective 1978 - 1991

ICD-O-2 Morphology

Effective 1992 - 2000

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)

Grade

Not Applicable

Module Rule

See abstractor notes

Alternate Names

Acute panmyelosis, NOS
APMF
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.

Abstractor Notes

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 the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5:PH10.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

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

Immunophenotyping

CD13 expression
CD33 expression
CD34 expression
KIT (CD117) expression
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

Corresponding ICD-9 Codes

238.79 Other lymphatic and hematopoietic tissues

Corresponding ICD-10 Codes

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)

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

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

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

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary