Myeloid leukemia associated with Down Syndrome

Myeloid leukemia associated with Down Syndrome
ICD-O-3 Morphology
Effective 2010 and later
for cases diagnosed 2010 and later
Primary Site(s)

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Code grade specified by pathologist. If no grade specified, code 9
Module Rule
M3 Module 5: PH10
Alternate Names
Acute myeloid leukemia associated with Down syndrome
AML associated with DS
Acute myeloid leukemia in DS is usually an acute megakaryoblastic leukemia. There are no biological differences in Down Syndrome's individuals between myelodysplastic syndrome (MDS) and AML. In the preleukemia phase, the disease has the features of Refractory cytopenia of childhood (RCC). Individuals with DS have a 50x increase in incidence of acute leukemia during the first 5 years of life compared to non-DS individuals. This is part of the myeloid proliferations with Down syndrome WHO group, which also includes transient abnormal myelopoiesis (see 9898/1).
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9860/3.) This disease is unique to children with Down's Syndrome (DS). Blood and bone marrow are the principle sites of involvement. Extramedullary involvement, mainly of spleen and liver, is almost always present. Acute myeloid leukemia in DS accounts for 50% of cases of acute leukemia in DS individuals. There is no biological differences between myelodysplastic syndrome (MDS) and overt acute myeloid leukemia (AML) in DS individuals. Because this type of disease is unique to children with DS, the term Myeloid leukemia (AML) of DS encompasses both neoplasms, MDS and AML.
Definitive Diagnostic Methods
Bone marrow bx
Peripheral blood
Genetics Data
Trisomy 8
Trisomy 21
CD4+ (most cases)
CD7+ (most cases)
CD13+ (most cases)
CD33+ (most cases)
CD36+ (most cases)
CD41+ (most cases)
CD42+ (most cases)
CD61+ (most cases)
CD71+ (most cases)
CD117+ (most cases)
IL-3R+ (most cases)
TPO-R+ (most cases)
Stem cell transplant
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
205.8 Other myeloid leukemia
Corresponding ICD-10 Codes
C92.7 Other myeloid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C92.Z Other myeloid leukemia
Signs and Symptoms
Diagnostic Exams
Recurrence and Metastases
Epidemiology and Mortality