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

Help me code for dx year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
M3 Module 5: PH10
Alternate Names
Acute megakaryocytic leukemia
Acute myelodysplasia with myelofibrosis
Acute myelofibrosis
Acute myelosclerosis, NOS [OBS]
Acute panmyelosis, NOS
Malignant myelofibrosis
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. Patients present with acute and severe symptoms including weakness, fatigue, fever and bone pain. Pancytopenia is always present. There is no or minimal splenomegaly. The peripheral blood shows pancytopenia which is usually marked. 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.
Definitive Diagnostic Methods
Bone marrow biopsy
Immunohistology
Genetics Data
-5/del(5q)
-7/del(7q)
Immunophenotyping
CD13
CD33
CD34
CD117
Myeloid hematopoietic stem cell
Treatments
Chemotherapy
Stem cell transplant
Transformations to
None
Transformations from
None
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None