SEER Inquiry System - View
Source 1: Heme & Lymph Manual & DB, Appendix F
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? See 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.
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Accession a single primary per Rule M2 which indicates you are to abstract a single primary when there is a single histology. Code the histology to 9983/3 [refractory anemia with excess blasts type 2 (RAEB-2)].
Per Appendix F, myelofibrosis, NOS, is NOT a synonym for primary myelofibrosis. Myelofibrosis, NOS, if not specified to be primary myelofibrosis, therefore, is not reportable.
Per PH29, code the specific histology when the diagnosis is one non-specific (NOS) histology (MDS) and one specific histology (RAEB-2) AND the Multiple Primary Calculator confirms the specific histology and NOS histology are the same primary (which it does).
Myelodysplastic syndrome, NOS is a generic disease description. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. The more specific myelodysplastic syndromes are: refractory anemia; refractory neutropenia; refractory thrombocytopenia; refractory anemia with ring sideroblasts; refractory cytopenia with multilineage dysplasia; refractory anemia with excess blasts; and refractory cytopenia of childhood. If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific diagnosis.
SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.
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 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. myelofibrosis
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 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. myelodysplastic syndrome
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. Abstract a single primary when there is a single histology. Stop at Rule M2.
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.