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Abstract two primaries. This is a transformation from a chronic disease (the Hairy Cell Variant) to an acute disease (DLBCL). Although this rare situation is not clearly covered in the Hematopoietic rules, the fact that this was originally a Hairy Cell Leukemia variant means that the DLBCL is a new primary.

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.

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.

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 prior to 1/1/2010:This is a single primary. The histology code is 9596/3 [composite Hodgkin and non-Hodgkin lymphoma].

According to the Single Versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table, 9596/3 followed by 9670/3 is one primary.

For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

Abstract this case as a single primary. Hematopoietic Multiple Primary Rule M2 applies.

Code to unknown primary, C809, based on rule PH27. There is no indication in the information provided of the site of origin; therefore, PH2 cannot be used. We recommend a thorough review of the case to determine if the site of origin is identified in the medical record.

Use Rule M2. Abstract as a single primary when there is a single histology.

When you have questions about how to code the primary site, start with the abstractor notes. If the answer isn't found there go to Module 7 (a specific module to help code primary site for lymphomas).

The abstractor notes for DLBCL in this case do not provide information you can use for this case. Go to Module 7 in the PH rules.

Use Rule PH25 Code the primary site to the organ when lymphoma is present in an organ and that organ’s regional lymph nodes. Code the primary site to colon (organ and regional lymph nodes involved). The fallopian tube is secondary involvement. As is common with lymphomas, there can be more than one organ involved. You can differentiate the primary site from the secondary site(s) because of the large colon mass with regional lymph node involvement.

Code Multiplicity Counter to 02 [Two tumors present] for an epithelial ovarian primary involving both ovaries. Do not count the peritoneal implants; they are regional metastasis and not included in the multiplicity counter. An example like this will be added to the manual in the next revision.

For cases diagnosed prior to 2018

Code the grade/differentiation field from the biopsy for this situation. According to experts consulted, hormone therapy does not alter the grade in this case and grade should be coded based on information after hormone therapy when that is the only grade information available.

Do not code aspirin as treatment for primary polycythemia (polycythemia vera).