Therapy-related myeloid neoplasms

Name
Therapy-related myeloid neoplasms
ICD-O-3 Morphology
9920/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 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
None
Alternate Names
t-AML
t-AML/t-MDS
t-MDS
t-MDS/MPN
Therapy-related acute myeloid leukemia, alkylating agent related
Therapy-related acute myeloid leukemia, epipodophyllotoxin-related
Therapy-related acute myeloid leukemia, NOS
Therapy-related acute promyelocytic leukemia (APL)
Therapy-related myelodysplastic syndrome
Therapy-related myelodysplastic/myeloproliferative neoplasm
Definition
Development of Acute myelogenous leukemia as a result of treatment for a previous malignancy with ionizing radiation or certain chemotherapy agents.

AML occurs after autologous bone marrow and stem cell transplantation; patients over 40 years at transplant who receive total body irradiation are at highest risk.

Therapy-related AML can result from chemotherapy or radiation therapy for benign or malignant diseases. Treatment options are the same as for other myelodysplastic syndromes.
Abstractor Notes
For cases abstracted prior to 2010, this code was only used for therapy-related Acute myeloid leukemia (t-AML).

In the 2008 WHO Manual, this code also includes therapy-related Myelodysplastic syndrome (t-MDS) and myelodysplastic/myeloproliferative neoplasms (t-MDS-MPN) occurring as late complications of prior cytotoxic chemotherapy and/or radiation therapy administered for a neoplastic or non-neoplastic disorder. Peripheral blood and bone marrow are the principle sites of involvement.

Although WHO groups therapy-related Myelodysplastic sydrome with the therapy-related AML MDS, MPN, and MDS-MPN, the ICD-O-3 has a separate code for therapy-related MDS 9987/3. WHO has grouped these therapy-related myeloid neoplasms into one category because current clinical practice recognizes these neoplasms as being similar in etiology (therapy-related) and groups them for the purpose of analysis. Therapy-related myeloid neoplasms represent coincidental disease and would be expected to behave like other de-novo disease.

For those cases diagnosed 2010 and later, Therapy-related myelodysplasic syndrome, NOS should be coded to the 9920/3 category.

DO NOT code therapy-related myeloid neoplasm simply because the patient has a history of radiation therapy or chemotherapy. There must be a physician's statement that says this is a therapy-related neoplasm (acute myeloid leukemia, MDS, MPN, or MDS/MPN.)

If a specific myeloid neoplasm that is described with a different specific histology term is also stated to be therapy related, code 9920/3 to capture the fact that this disease was therapy related. Document the other specific histology term in the text part of the abstract.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
Alk/RT: High incidence of clonal cytogenetic abnormalities
Alk/RT: Other chromosomes include 1, 4, 12, 14, and 18
Alk/RT: Unbalanced translocations or deletions involving chromosomes 5 and/or 7
del(13q), del (20q), del(11q)m dek*3o(m -17, -18, -21, +8
inv(16)
t(11;19)
t(3;21)
t(6;9)
t(6;11)
t(8;16)
t(8;21)
t(9;11)
Topo: 11q23 (MLL)
Immunophenotyping
CD7
CD13
CD33
CD34+
CD56
MDR-34+
Treatments
Bone marrow transplant
Chemotherapy
Immunotherapy
Radiation
Stem cell transplant
Transformations from
None
Same Primaries
Corresponding ICD-9 Codes
205.0 Acute myeloid leukemia
Corresponding ICD-10 Codes
C92.0 Acute myeloid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C92.0 Acute myeloblastic leukemia
Signs and Symptoms
Bone marrow failure
Easy bruising or bleeding
Shortness of breath
Weakness
Weight loss or loss of appetite
Progression and Transformation
None
Epidemiology and Mortality
Incidence: 10-20% of all cases of AML, MDS and MDS/MPN
Survival: 5 year survival less than 10%