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For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

The histology is coded as Plasma cell myeloma [9732/3]. The diagnostic confirmation is coded to 1 [positive histology].

Under the Definitive Diagnostic Methods section in the Heme DB it indicates that a bone marrow aspiration and bone marrow biopsy are procedures used to diagnose this disease process. This patient's diagnosis was based on the pathology (presumably from a bone marrow biopsy).

NOTE: This is a reportable case. Ambiguous terminology is used to accession cases (determine reportability) because it has been used for over 30 years to do so. Any deviation from using ambiguous terminology to determine case reportability would cause the reporting of incidence counts to vary. In this case, there was a reportable, ambiguous terminology diagnosis of plasma cell myeloma on the pathology report; as well as a reportable physician's statement/diagnosis of plasma cell myeloma.

Ambiguous terminology, however, is not used to report a more specific diagnosis for the Heme & Lymphoid neoplasms. For example, if the pathology report final diagnosis was "Myeloproliferative neoplasm, probably Polycythemia Vera" the histology would be coded as myeloproliferative neoplasm, unclassifiable [9975/3]. The ambiguous terminology indicates that the genetic testing, immunophenotyping, etc., probably are not complete or are not diagnostic of the more specific disease. Wait to code the histology until there is a definite diagnosis given.

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.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

The patient only has two primaries: cutaneous T-cell lymphoma diagnosed in the 1980s and diffuse large B-cell lymphoma of the bowel diagnosed in 1991.

The DLBCL of the brain does not represent a new primary. It is progression of the 1991 disease process with the same histology. Under the Alternate Names section in the Heme DB, one synonym for DLBCL is "Primary DLBCL of the CNS." The histology code for both the 1991 bowel neoplasm and the current CNS neoplasm is 9680/3. Per Rule M2, a single histology is a single primary.

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.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

There are two reportable primaries for this case -- follicular lymphoma in 2010 and DLBCL in 2012.

First determine the histologies needed to to determine the number of primaries to report. We determined the histologies are follicular lymphoma, grade 1 for 2010 and DLBCL for 2012 as follows:

• Rule M4, a mix of DBLCL and follicular lymphoma, grade 3 is a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s). Additionally, the instruction per Rule PH11 indicates one is to code the histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma (i.e., follicular lymphoma, grade 3) are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.

Per the Hematopoietic database, follicular lymphoma (all types are chronic) transforms to DLBCL (acute). Per Rule M 10 instructions, "Abstract as multiple primaries when a neoplasm is as a neoplasm there is a of an neoplasm after the chronic diagnosis." Therefore, abstract the DLBCL as a second primary.

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.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics."

Abstract a single primary per M2, a single histology represents a single primary. Code the histology to 9983/3 [MDS/RAEB-2.]

The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability; it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML.

It is important to understand the implication of incorrectly assigning histology codes for hematopoietic and lymphoid neoplasm using ambiguous terminology. Using this case as an example, the patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML, if left untreated, is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML.

The other issue with this case is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." This means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the second primary.

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.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm.

Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [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.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

This case is accessioned as two primaries: Plasmacytoma diagnosed in 2010 and plasma cell myeloma diagnosed in 2011 per Rule M10. The patient has a diagnosis of a solitary plasmacytoma (chronic neoplasm) followed by a diagnosis of plasma cell myeloma (acute neoplasm) diagnosed greater than 21 days later.

The physician is calling this a progression to plasma cell myeloma even though the bone marrow has less than 10% plasma cells, take this statement as progression or a clinical diagnosis of plasma cell myeloma.

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.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

This case is reportable. Code histology to 9823/3 [CLL/SLL].

Ambiguous terminology is used to accession cases (determine reportability) because it has been used for over 30 years to do so. Any deviation from using ambiguous terminology to determine case reportability would cause the reporting of incidence counts to vary. In this case, there was a reportable, ambiguous terminology diagnosis on peripheral blood that is "consistent with" involvement by chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) immunophenotype.

The ambiguous terminology "consistent with" in the flow cytometry report is acceptable to determine reportability. Given that it is the only reportable histology mentioned in the scenario, it is also used to code histology. The instruction "Do not code histology based on ambiguous terminology" is intended to be used when there is a reportable NOS histology and reportable more specific histology stated in the diagnosis. Ambiguous terminology cannot be used to report the more specific diagnosis in cases of Heme & Lymphoid neoplasms.

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.

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, acute myeloid leukemia [9861/3].

MDS diagnosed prior to 1/1/2001 is not a reportable disease process. However, because MDS is currently a reportable disease process, it must be considered when trying to determine whether the AML represents a separate primary.

• If the Heme DB indicates MDS and AML represent different (separate) disease processes, only one primary is reported (i.e., AML) because the 1999 diagnosed MDS is not reportable.

• If the Heme DB indicates MDS and AML represent the same disease process, then no primaries are reported because MDS was not reportable in 1999.

Rule M2 does not apply to this case because more than one histology is mentioned in the scenario. According to the Heme DB, MDS can transform to AML. Rules M8-M13 apply to cases involving transformation. In this case, Rule M10 applies because the patient was diagnosed with a chronic neoplasm (myelodysplastic syndrome) followed greater than 21 days later by an acute neoplasm (AML).

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.

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, diffuse large B-cell lymphoma diagnosed in 2011 per Rule M7. Note 4 for Rule M7 states to change the histology code on the original abstract to the more specific histology, diffuse large B-cell lymphoma in this case. There is no time restriction for rule M7. Apply rule PH11 and code the histology as 9680/3 [DLBCL] when both DLBCL and follicular lymphoma are present in the same lymph node(s).

Ambiguous terminology is not used to code a more specific histologic type per the Heme Manual. The information submitted states only that this low grade B-cell lymphoma was "most consistent with follicular lymphoma." The term "consistent with" is an ambiguous term per SEER and cannot be used to code the histology of the 2011 neoplasm as follicular lymphoma. There was no subsequent clinical statement that this patient was diagnosed with follicular lymphoma in 2011.

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.

Although the ambiguous terminology on the pathology report is not used to code the histology to follicular lymphoma, had there been a subsequent clinical statement that this patient had follicular lymphoma, the histology would be coded to follicular lymphoma [9690/3]. A diagnosis of follicular lymphoma followed by a diagnosis of DLBCL more than 21 days later is a new primary per rule M12.

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, non-Hodgkin lymphoma (previously called poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell) [9591/3] diagnosed in 1991.

Determining the number of primaries is based on the rules in effect at the time of each diagnosis. The original lymphoma was diagnosed in 1991 and the first transformation to follicular lymphoma in 2008. The pre-2010 rules for coding histology and determining multiple primaries must be applied first because the rules changed for diagnoses occurring 2010 or later. Per the Single Versus Subsequent Primaries Table, poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3] is the same primary as follicular lymphoma [9690].

The Heme DB and Manual are used to confirm that the 2010 recurrences of follicular lymphoma, grade 1 [9695/3], and follicular lymphoma, grade 3 [9698/3], are the same primary according to the Heme Calculator check required per Rule M15. Per the Heme DB page, the diagnoses follicular lymphoma, grade 3 [9698/3] and follicular lymphoma, grade 1 [9695/3] are comparable to follicular lymphoma [9690] as stated in the section.

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.