SEER Inquiry System - Report
Produced: 11/26/2024 7:26 AM
Question 20100075
Inquiry Details
References:
Heme & Lymph Manual & DB
Question:
Discussion:
1/27/10 BM biopsy: CML BCR/ABL+ FISH positive for BCR/ABL and cytogenetics showing the t(9;22)q34q11.2 translocation. Treated with Imatinib. 4/15/10 BM biopsy: B-acute lymphoblastic leukemia (Blast phase of CML). Would the term "blast phase of CML" indicate the 4/15/10 bone marrow biopsy showed CML or would a new primary be abstracted with histology coded 9811/3 [B lymphoblastic leukemia/lymphoma, NOS]?
Applying rule M10, this is a new primary, but note 2 states transformations are defined in the Heme DB. The Abstractor Notes section indicates CML has three phases: chronic, accelerated, and the blastic phase or blast crisis. The accelerated phase can last weeks to months. In the chronic phase the involvement is usually limited to blood, bone marrow and spleen although the liver may be infiltrated. During the blastic phase, lymph nodes and tissue may be involved. The blastic phase is a disease progression from the chronic phase. The disease, however, remains the same histology, chronic myelogenous leukemia.
Answer:
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case represents a multiple primary per Rule M15 which states you are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries for all cases that do not meet the criteria of M1-M14.
The histology for the first primary is coded to 9875/3 [chronic myelogenous leukemia, BCR-ABL1 positive].
The histology for the second primary is 9811/3 [B lymphoblastic leukemia/lymphoma, NOS] in the absence of further documentation that the B-ALL was also positive for the t(9;22) translocation.
The histology code 9806/3 [Mixed phenotype acute leukemia with t(9;22)(q34;q11.2); BCR-ABL1] cannot be used for the second primary because there is no documentation that the B-ALL diagnosed on 04/15/2010 also had the t(9;22) translocation and this histology cannot be used in patients ." Per the Definition section in the Heme DB, in order to use histology code 9806/3 "This leukemia meets the criteria for mixed phenotype acute leukemia (MPAL) in which the blasts also have t(9;22) translocation of BCR-ABL1 rearrangement. Some patients with chronic myeloid leukemia may develop or even present with a mixed blast phase that would meet criteria for MPAL; however, this diagnosis should not be made in patients known to have had CML."
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:
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 2010
For cases diagnosed 2010-2011, this is a new primary. The information listed for CML is accurate because there is a blast phase which can be confusing. This case, however, does not meet the criteria of having the same histology, CML.
Enter the unique phrase or words into the 2010 Hematopoietic Database search mechanism (Do not enter the entire term). In this case, a "smart search" would be entering t(9;22) into the search mechanism. The first disease displayed is mixed phenotype acute leukemia with t(9;22)(q34;q11.2);BCR-ABL1 [9806/3]. This matches your case exactly.
Note 2 under M10 actually means that you use all of the information in the DB. When you do not see the disease in the transformation information, use the multiple primaries calculator. The MP calculator shows that the mixed phenotype acute leukemia is a new primary. The hematologists do not list this disease as a transformation. Instead, it was a new primary. Check both the transformation information and the MP calculator when you are questioning whether or not to code a new primary.
The answers for SINQ questions with 2010 ID numbers were written using the 2010 Heme & Lymph Manual & DB. The instructions for using the 2010 Hematopoietic Database were written for the version of the software in use as of 5/24/2011. The user interface of the web-based 2010 Hematopoietic Database available from the SEER website varies slightly from the 5/24/2011 version in that the web-based version provides all the disease information in one scrollable window.
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 2010
For cases diagnosed 2010-2011, access the 2010 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.