Refractory cytopenia with multilineage dysplasia

Name
Refractory cytopenia with multilineage dysplasia
ICD-O-3 Morphology
9985/3
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
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
RCMD
Refractory cytopenia of childhood
Definition
Myelodysplastic syndrome with bi-cytopenia or pancytopenia and dyplastic changes in 10% or more of cells in 2 or more of myeloid cell lines.
< 1% blasts in blood, < 5% blasts in marrow, <15% ringed sideroblasts (If > 15%, case should be classified as subtype RCMD-RS)
Auer rods not seen, Monocytes in blood < 1 x 109/L
Abstractor Notes
Blood and bone marrow are always involved. At least 2 types of blood counts are low and have an abnormal appearance under the microscope (dysplasia). The number of blasts is less than 5%.

This histology code also includes childhod MDS. MDS is very rare in children. Both the peripheral blood and bone marrow are involved.

For MDS diseases (9980, 9982, 9983, 9985, 9986, 9989, 9991, 9992), abstracting each of the subtypes would result in over-counting of the diseases.
1. Code only the first subtype that is diagnosed.
2. Do not change the histology code or create a new abstract for any subsequent specific MDS subtypes.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Genetics Data
del (5q)
del (7q)
del (20q)
Monosomy 5
Monosomy 7
Immunophenotyping
None
Treatments
Bone marrow transplant
Chemotherapy
Immunotherapy
Stem cell transplant
Transformations from
None
Corresponding ICD-9 Codes
238.72 Low grade myelodysplastic syndrome lesions
Corresponding ICD-10 Codes
D46.7 Other myelodysplastic syndromes
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
D46.A Refractory cytopenia with multilineage dysplasia
D46.B Refractory cytopenia with multilineage dysplasia and ring sideroblasts
Signs and Symptoms
Cytopenia (of two or more myeloid lines)
Easy bruising or bleeding
Petechiae (flat, pinpoint spots under the skin caused by bleeding)
Shortness of breath
Skin paler than usual
Weakness or feeling tired
Progression and Transformation
~10% of cases evolve to AML in 2 years
Epidemiology and Mortality
Age (Adult): 70 years median age
Age (Childhood): all age groups
Incidence (Adult): accounts for ~30% of MDS cases
Incidence (Childhood): most common MDS of childhood, ~50%
Sex (Adult): slight male predominance
Sex (Childhood): no male or female predominance
Survival: 30 month median survival (~1/2 of patients died within 2 years)