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SEER Inquiry System - Report
Produced: 10/05/2025 2:32 PM
Question 20110066
Inquiry Details
References:
Heme & Lymph Manual & DB
Question:
Discussion:
Answer:
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.
"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (9680/3).
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.
History:
For cases diagnosed 2010-2011, access the Hematopoietic Database at http://seer.cancer.gov/tools/heme/. Click on Hematopoietic Project. Click on Hematopoietic and Lymphoid Database. For 2010-2011 diagnoses, click on the "use the 2010 database" label in the upper right corner of the screen. The 2010 Hematopoietic Coding Manual (PDF) button will appear to indicate the correct version of the program is available now for query.
Abstract a second primary, DLBCL [9680/3] for this patient. The steps you would use to determine the number of primaries are:
"Richter transformation" is a term that indicates CLL has transformed to DLBCL. Richter transformation will be added as a synonym for DLBCL in the next revision of the Heme DB.
Enter in the Heme DB to find the histology. Click on the SEARCH button. "Chronic lymphocytic leukemia/small lymphocytic lymphoma" [9823/3] is highlighted on the screen under the RESULTS FOR ALL TERMS area.
Scroll down to the TRANSFORMATIONS section information. Note that CLL (a chronic disease process) transforms into diffuse large B-cell lymphoma (an acute disease process). When a cell type is listed in the transformation section for a given malignancy, the disease process in the transformation section is considered the acute process and the disease listed at the top of the screen display is considered the chronic process. In this case, the chronic and acute disease processes were diagnosed at different times. Given that the case scenario indicated a "history of" CLL, the assumption in this response is that the acute neoplasm (DLBCL) occurred more than 21 days after the diagnosis of the chronic neoplasm (CLL).
Click on the 2010 HEMATOPOIETIC CODING MANUAL (PDF) button. Once in the manual, locate one of the three formats (i.e., flowchart, matrix or text) to check the Multiple Primary Rules. The rules are intended to be reviewed in consecutive order from Rule M1 to Rule M13. Stop at the first rule that applies to the case you are processing.
. Abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase occurs more than 21 days after the chronic disease diagnosis.
