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SEER Inquiry System - Report
Produced: 11/26/2024 1:13 PM
Question 20110155
Inquiry Details
References:
Subject matter expert
Question:
Discussion:
Per consultation: an 83 year old patient started on hydroxurea 18 years ago following a diagnosis of thrombocytosis and probable polycythemia. It appears the polycythemia progressed to probable myelofibrosis. The possibility of an MDS needs to be considered.
Problem list: Polycythemia with probable progression to myelofibrosis or MDS.
Bone marrow biopsy two weeks later shows some progression of dysmegakaryocytopoiesis. Patient has evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera.
On follow-up visit six weeks later: Continue to manage patient with hydroxyurea.
An additional six months later: Diagnosis is polycythemia with thrombocytosis.
Answer:
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case should be accessioned as a single primary. Code the histology to 9920/3 [therapy-related myelodysplastic syndrome].
The reportable diagnoses must first be separated from the non-reportable diagnoses mentioned in the consult. Thrombocytosis (NOS), polycythemia (NOS), and myelofibrosis (NOS) are not reportable terms. To verify this, look up each term in the Heme DB. No database matches list the preferred name or the alternative names as any of these NOS terms.
The reportable diagnoses are all from the post-bone marrow biopsy consult, "evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera." The subsequent notes in the consult again only refer to this as non-reportable polycythemia (NOS) or thrombocytosis (NOS). Keep in mind that this patient has been undergoing treatment with chemotherapy (hydroxyurea) for many years for polycythemia (NOS); the patient was diagnosed with polycythemia, "about 18 years ago."
According to the Subject Matter Experts, as MDS progresses, it may manifest as several different subtypes, this is a part of the disease process and abstracting each subtype would result in over-reporting this disease. This patient has a complicated history. The consult information does not adequately document whether this patient's initial diagnosis of "polycythemia" was primary polycythemia (reportable) or a secondary polycythemia (not reportable). If the patient was initially diagnosed with a primary polycythemia 18 years ago the current diagnosis of "JAK2 mutation positive polycythemia vera" would not be a new primary. The manifestation of ET may be due to the progression of MDS. In either case, this patient does have a therapy-related myelodysplastic syndrome which is the same primary as both PV and ET.
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, 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.
This case should be accessioned as a single primary. Code the histology as a therapy-related myeloid neoplasm (Therapy-related myelodysplastic syndrome) [9920/3]. The steps used to arrive at this decision are:
The reportable diagnoses must first be separated from the non-reportable diagnoses mentioned in the consult. Thrombocytosis (NOS), polycythemia (NOS), and myelofibrosis (NOS) are not reportable terms. To verify this, look up each term in the database. No database matches list the preferred name or the alternative names as any of these NOS terms. For example, polycythemia, NOS is not the preferred name or an alternative name for Polycythemia Vera. The reportable diagnoses left are all taken from the post-bone marrow biopsy consult, "evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera." The subsequent notes in the consult again only refer to this as non-reportable polycythemia (NOS) or thrombocytosis (NOS). Also keep in mind that this patient has been undergoing treatment with chemotherapy (hydroxyurea) for many years for polycythemia (NOS). The consult notes this patient was diagnosed with polycythemia, "about 18 years ago."
Enter in the Heme DB to find the histology. Click on the SEARCH button. "Myelodysplastic syndrome, unclassifiable" [9989/3] will be automatically highlighted on the screen under the RESULTS area.
Review the other RESULTS for MDS. Select "Therapy-related myelodysplastic syndrome, NOS" under RESULTS because this patient was treated with chemotherapy in the past. The screen will refresh.
Scroll down to the ABSTRACTOR NOTE section. This states that for cases diagnosed 2010 and later, the correct histology code for therapy-related MDS is 9920/3 [therapy-related myeloid neoplasms].
Go to the RESULTS area again click on "Therapy-related myeloid neoplasm." The screen will refresh. This disease process was returned as a potential match when the initial search for MDS was performed. For therapy-related myeloid neoplasm the histology code is 9920. Scroll down to the DEFINITION section which notes that therapy-related AML can result from chemotherapy or radiation therapy for benign or malignant diseases.
Enter in the Heme DB to find the histology. Click on the SEARCH button. "Polycythemia Vera" [9950/3] will be automatically highlighted on the screen under the RESULTS area.
Enter in the Heme DB to find the histology. Click on the SEARCH button. "Essential thrombocythemia" [9962/3] will be automatically highlighted on the screen under the RESULTS area.
These three histologies (MDS, PV, ET) were diagnosed at the same time per the limited consult information available. 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.
No other rules apply as this patient was simultaneously diagnosed with Therapy-related MDS [9920/3], ET [9962/3] and PV [9950/3].
From the Hematopoietic DB, click on the SHOW MULTIPLE PRIMARIES CALCULATOR link at the top of the page. Enter [therapy-related MDS] and [PV] and click the CALCULATE button. The result is "Same Primary."
Repeat Step 9, this time entering [therapy-related MDS] and [ET]. Again, the result is "Same Primary."
Abstract a single primary because this patient has a Therapy-related MDS which is not a separate primary from either the ET or PV. To verify this, Enter in the Heme DB, click SEARCH. Ensure the term "Therapy-related myeloid neoplasm" is highlighted in the RESULTS area. Scroll down to the SAME PRIMARIES section for Therapy-related MDS which indicates both histologies 9950 [Polycythemia Vera] and 9962 [Essential Thrombocythemia] are the same primaries as this neoplasm.
According to the Subject Matter Experts, as MDS progresses, it may manifest as several different subtypes, this is a part of the disease process and abstracting each subtype would result in over-reporting this disease. This patient has a complicated history. The consult information does not adequately document whether this patient's initial diagnosis of "polycythemia" was primary polycythemia (reportable) or a secondary polycythemia (not reportable). If the patient was initially diagnosed with a primary polycythemia 18 years ago the current diagnosis of "JAK2 mutation positive polycythemia vera" would not be a new primary. The manifestation of ET may be due to the progression of MDS. In either case, this patient does have a therapy-related myelodysplastic syndrome which is the same primary as both PV and ET.
