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SEER Inquiry System - Report
Produced: 12/23/2025 10:46 PM
Question 20100037
Inquiry Details
References:
Heme & Lymph Manual & DB
Question:
Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries should be accessioned for a patient diagnosed with essential thrombocythemia [9962/3] in 2002 who had a 2010 biopsy consistent with the fibrotic stage for a chronic myeloproliferative disorder that "suggests the patient is transforming to an acute myeloid leukemia"? See Discussion.
Discussion:
Patient had a diagnosis of essential thrombocythemia [9962/3] in 2002 and was treated with Hydroxyurea. In 2010, the patient was admitted with severe bone pain and a diagnosis described as, "The overall features of the biopsy are consistent with a fibrotic stage of a chronic myeloproliferative disorder. The presence of up to 15% CD34+ immature cells seen in the biopsy suggests that the patient is transforming to an acute myeloid leukemia." In addition, cytogenetic studies and molecular testing for JAK2 were ordered. These findings confirmed a myeloproliferative disorder. JAK2 mutation was not detected. The patient died within 2 weeks. Is this a new primary?
Was this patient diagnosed with AML (which requires 20% or more blasts and this is only 15%)? If this is a new primary, is the histology 9861/3 [AML, NOS] or 9895/3 [AML with myelodysplasia-related changes]? Was the second diagnosis of AML definitively diagnosed?
Answer:
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case is a single primary, essential thrombocythemia [9962/3] in 2002. The 2010 diagnosis is chronic myeloproliferative disorder [9960/3].
According to Rule M15, the Multiple Primaries Calculator is to be used to first determine the number of primaries. Per the calculator, essential thrombocythemia and chronic myeloproliferative disorder are the same primary. (Acute myeloid leukemia is not used as the second histology because it is preceded by a non-reportable ambiguous term, "suggests." "Suggests" is not on the list of reportable ambiguous terms in the Hematopoietic and Lymphoid Neoplasm Coding Manual.
In 2010, this patient was in a late stage of ET. When any of the specific MPN neoplasms such as ET are in the late stage of disease, the characteristics of the specific disease (ET) will no longer be detectable. Accordingly, for this patient the diagnostic testing was positive for MPN, unclassifiable. In this case, do not change the diagnosis from the more specific disease (ET) to the NOS (MPN, unclassifiable).
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 (or 2012)
For cases diagnosed 2010-2011, this case is a single primary, essential thrombocythemia [9962/3] in 2002. The 2010 diagnosis is chronic myeloproliferative disorder [9960/3]. According to the multiple primaries calculator, essential thrombocythemia and chronic myeloproliferative disorder are the same primary. Acute myeloid leukemia is not reportable in this case because it is preceded by a non-reportable ambiguous term "suggests." "Suggests" is not on the list of reportable ambiguous terms. See page 12 of the 2010 Hematopoietic and Lymphoid Neoplasm manual. Note: Ambiguous terminology is used to determine reportability; it is NEVER used to determine histology for heme and lymphoid neoplasms.
In 2010, this patient was in a late stage of ET. When any of the specific MPN neoplasms such as ET are in the late stage of disease, the characteristics of the specific disease (ET) will no longer be detectable. Accordingly for this patient, the diagnostic testing was positive for MPN, unclassifiable. In this case, do not change the diagnosis from the more specific disease (ET) to the NOS (MPN, unclassifiable).
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.
