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

Help me code for dx year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Childhood myelodysplastic syndrome
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. Most patients present with evidence of bone marrow failure with cytopenia of two or more myeloid lines. 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%. About 25% of people with MDS have this type. It will change into leukemia in about 10% of patients. Having this type of MDS will shorten a person's survival. One estimate is that half of patients will die within 2 years of diagnosis.

This histology code also includes childhod MDS. MDS is very rare in children. Both the peripheral blood and bone marrow are involved. Hepatosplenomegaly is generally not a feature of RCC and lymph nodes are not the initial site of involvement.

MDS is a group name for a number of specific diseases. As the MDS progresses, it may manifest as several subtypes. This is a part of the disease process. Abstracting each of the subtypes would result in over-counting these diseases. Code the first subtype that is diagnosed. Do not change the histology code or create a new abstract for any subsequent specific MDS subtypes.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetics Data
del (5q)
del (7q)
del (20q)
Monosomy 5
Monosomy 7
Trisomy 8
Immunophenotyping
None
Treatments
Chemotherapy
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None