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Question: 20120070 Status
Final

References
Source 1:   Heme & Lymph Neo Manual & DB
pgs:  
Notes:  
Source 2:  
pgs:  
Notes:  


Question
Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a bone marrow biopsy shows myelodysplastic syndrome - refractory anemia with excess blasts type 2 (RAEB-2) and myelofibrosis?



Discussion
Should the myelofibrosis be accessioned as a second primary? Or is it a descriptor of the MDS/RAEB-2? The multiple primaries calculator shows 9983/3 and 9961/3 represent two primaries.



Answer
For cases diagnosed 2010 and forward, access the Hematopoietic Database at Click on Hematopoietic and Lymphoid Database. In the search box, enter your histology or the ICD-O-3 code. Click on the link to that disease. In the "Help me code for dx year" field, enter the year of diagnosis. The Hematopoietic Coding Manual (PDF) button will appear.

Accession a single primary, refractory anemia with excess blasts type 2 (RAEB-2) [9983/3]. The steps used to arrive at this decision are:

Step 1: Enter myelofibrosis in the Heme DB to find the histology. Click on the SEARCH button. Click on the term “primary myelofibrosis” [9961/3] on the screen under the NAME heading.

Step 2: Scroll down to the ALTERNATIVE NAMES section. Review the alternative names for primary myelofibrosis. Note that myelofibrosis, NOS, is NOT a synonym for primary myelofibrosis. Myelofibrosis, NOS (not specified as primary myelofibrosis), therefore, is not reportable.

Step 3: Enter myelodysplastic syndrome in the Heme DB to find the histology. Click on the SEARCH button. Click on the term “myelodysplastic syndrome, unclassifiable” [9989/3] on the screen under the NAME area.

Step 4: Review the ABSTRACTOR NOTE section. MDS is a generic disease description with more specific Myelodysplastic syndromes that can be specified such as RAEB-2.

Step 5: Scroll to the SAME PRIMARY section. Refractory anemia with excess blasts type 2 (RAEB-2) [9983/3] and MDS is considered one primary.

Step 6: Determine the number of primaries. Click on the HEMATOPOIETIC CODING MANUAL (PDF) button. Once in the manual, locate the Multiple Primary rules. The rules are intended to be reviewed in consecutive order from Rule M1-M15. Stop at the first rule that applies to the case you are processing. Stop at Rule M2. Abstract a single primary when there is a single histology.

NOTE: Myelofibrosis means a proliferation of fibroblastic cells in bone marrow causing anemia and sometimes enlargement of the spleen and liver. Myelofibrosis may be associated with both benign and malignant disease processes.



History
This SINQ question has been updated to the Hematopoietic & Lymphoid Neoplasm Manual & Database published January 2014. The original answer below was written based on the rules in 2012 For cases diagnosed 2012 and later, access the Hematopoietic Database at

http://seer.cancer.gov/tools/heme/.

Click on Hematopoietic Project. Click on Hematopoietic and Lymphoid Database. The 2012 Hematopoietic Coding Manual (PDF) button will appear to indicate the correct version of the program is available now for query.

Accession a single primary, refractory anemia with excess blasts type 2 (RAEB-2) [9983/3]. The steps used to arrive at this decision are:

Step 1: Enter myelofibrosis in the Heme DB to find the histology. Click on the SEARCH button. The term “primary myelofibrosis” [9961/3] will be automatically highlighted on the screen.

Step 2: Scroll down to the ALTERNATIVE NAMES section. Review the alternative names for primary myelofibrosis. Note that myelofibrosis, NOS, is NOT a synonym for primary myelofibrosis. Myelofibrosis, NOS (not specified as primary myelofibrosis), therefore, is not reportable.

Step 3: Enter myelodysplastic syndrome in the Heme DB to find the histology. Click on the SEARCH button. The term “myelodysplastic syndrome, unclassifiable” [9989/3] will be automatically highlighted on the screen.

Step 4: Review the ABSTRACTOR NOTE section. MDS is a generic disease description with more specific Myelodysplastic syndromes that can be specified such as RAEB-2.

Step 5: Scroll to the SAME PRIMARY section. Refractory anemia with excess blasts type 2 (RAEB-2) [9983/3] and MDS is considered one primary.

Step 6: Click on the 2012 HEMATOPOIETIC CODING MANUAL (PDF) button. Once in the manual, go to the Multiple Primary Rules. The rules are intended to be reviewed in consecutive order from Rule M1 to Rule M16. Stop at the first rule that applies to the case you are processing. Stop at Rule M2. Abstract a single primary when there is a single histology.

NOTE: Myelofibrosis means a proliferation of fibroblastic cells in bone marrow causing anemia and sometimes enlargement of the spleen and liver. Myelofibrosis may be associated with both benign and malignant disease processes.



Last Updated
02/16/2014

Date Finalized
08/29/2012