Refractory anemia with ring sideroblasts

Name
Refractory anemia with ring sideroblasts
ICD-O-3 Morphology
9982/3
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
Acquired idiopathic sideroblastic anemia
Pure sideroblastic anemia
RARS
Refractory anemia with hemochromatosis
Refractory anemia with ring sideroblasts associated with marked thrombocytosis
Refractory anemia with ringed sideroblasts
Refractory anemia with sideroblasts
Sideroblastic anemia
Definition
The peripheral blood may contain a minor population of hypochromic cells but will not show blasts. The bone marrow aspirate smear shows an increase in erythroid precursors with erythroid lineage dysplasia.

> or equal to 15% ringed sideroblasts in marrow
< 5% myeloblasts in marrow, not in blood
Erythroid dysplasia
Abstractor Notes
People with RA with ring sideroblasts are similar to those with refractory anemia except that many of the red blood cells in the bone marrow contain characteristic ring-shaped iron deposits which are called ring sideroblasts.

The principle sites of involvement are the peripheral blood and bone marrow.

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
Peripheral blood smear
Genetics Data
None
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
D46.1 Refractory anemia with sideroblasts
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
D46.1 Refractory anemia with sideroblasts
Signs and Symptoms
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
~1-2% of cases evolve to acute myeloid leukemia
Epidemiology and Mortality
Age: 60-73 years median age
Incidence: 3-11% of MDS cases
Sex: no male or female predominance
Survival: 69-108 months median survival