Acute panmyelosis with myelofibrosis

Name
Acute panmyelosis with myelofibrosis
ICD-O-2 Morphology
9932/3
Effective 1992 - 2000
ICD-O-3 Morphology
9931/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
See abstractor notes
Alternate Names
Acute megakaryocytic leukemia
Acute myelosclerosis, NOS [OBS]
Acute panmyelosis, NOS
Malignant myelosclerosis [OBS]
Definition
Very rare form of acute myelogenous leukemia. This neoplasm is an acute panmyeloid proliferation with increased blasts and accompanying fibrosis of the bone marrow.

An acute panmyeloid proliferation with 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
CD33
CD34
CD117
Myeloid hematopoietic stem cell
Treatments
Chemotherapy
Stem cell transplant
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 (effective October 1, 2015 U.S. only)
C94.4 Acute panmyelosis with myelofibrosis
Signs and Symptoms
Bone pain
Easy bruising or bleeding
Pancytopenia
Shortness of breath
Weakness
Weight loss or loss of appetite
Diagnostic Exams
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