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

Code the primary site to C421 [bone marrow] per Rule PH5 when CLL/SLL [9823/3] involves the bone marrow.

In the later stages of CLL/SLL, there may be involvement of bone marrow AND lymph node(s), lymph node region(s), organ(s), or tissue(s). As long as the peripheral blood and/or bone marrow are involved, the primary site is bone marrow.

WHO states that the diagnostic criteria for CLL versus SLL is not clearly defined. According to WHO guidelines, it is better to code to CLL/SLL and code the primary site to bone marrow when the marrow is involved and to lymph nodes, organ, or tissue when there is no bone marrow involvement.

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.

Per Rule M15, accession two primaries. According to Rule M15, use the multiple primaries calculator to determine the number of primaries for all cases that do not meet the criteria of M1-M14. Per the calculator, this scenario represents two primaries.

Assuming the only area of involvement is the large intestine, code the histology to 9680/3 [diffuse Large B-Cell Lymphoma] and per Rule PH24 code the primary site to C189 [colon, NOS]. According to PH24, one is to code the primary site to the organ when lymphoma is present only in an organ.

Rule PH26 applies to the second primary. Assuming the only area of involvement is the bone marrow, code the histology to 9702/3 [peripheral T-cell lymphoma] and code the primary site to C421 [bone marrow]. According to PH26, one is to code the primary site to bone marrow (C421) when lymphoma is present only in the bone marrow.

Rule M4 does not apply for this case. Rule M4 applied when you have two or more types of non-Hodgkin lymphoma in the same anatomic location. That is not the case in this scenario.

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.

Polycythemia Vera (PV) [9950/3] is reportable. The Abstractor Notes section in the Hematopoietic Database for PV indicates there are three phases of PV. The third phase is referred to as the "spent" or "post-polycythemic myelofibrosis phase". This patient appears to be in the third phase of PV. This would not be reported as a new primary if PV has already been reported.

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.

Per the Heme DB, the histology B-cell acute lymphoblastic leukemia is synonymous with B lymphoblastic leukemia/lymphoma, NOS. Per Rule PH8, for a neoplasm that can manifest as either leukemia lymphoma or leukemia lymphoma, one is to code the primary site to the site of origin when lymph node(s) or lymph node region(s), tissue(s) or organs are involved. The Note 4 instruction states it is necessary to go to Module 7 (Rules PH18-PH27) to code the more specific primary site. In this case, use Rule PH22 to code primary site to C779 [lymph nodes, NOS] for the case you describe.

In this case, there is involvement of abdominal lymph nodes, spleen, bone marrow and bone. There is no indication of the primary site. Per the Heme DB, the most frequent sites of involvement for the lymphoma are bone and lymph nodes. This is a Stage IV lymphoma.

The now inactivated SINQ 20120047, stated that based on the sites of involvement, this histology could be coded as either leukemia or lymphoma. If the only involvement is the bone marrow, the site is coded to C421 [bone marrow]. The involvement of peripheral blood does not change the primary site because such involvement is part of the leukemic process.

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 1/1/2022

A low-grade appendiceal mucinous neoplasm (LAMN) is not reportable. The WHO classification designates LAMN with the behavior code /1 [uncertain whether benign or malignant].

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

Code the histology as therapy-related acute myeloid leukemia, NOS [9920/3] when the physician states this is a therapy-related AML.

The therapy-related diagnosis may be either clinically or pathologically stated to code the histology to 9920/3. In this case, the physician is aware of the previous chemotherapy, hormone therapy or radiation and adds that knowledge to the histologic findings of AML. The pathology report did not include this clinical, historical information as part of the final diagnosis. However, one can code therapy-related acute myeloid leukemia because clinically it was stated.

We recommend that you clearly document in the abstract that you are coding a clinical histology.

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.

Under the Alternate Names section of the Heme DB, systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD) is a synonym for systemic mastocytosis. Per Rule M2, this is one primary. Abstract a single primary when there is a single histology. Code the histology to 9741/3 [systemic mastocytosis].

Per the pathology report, the two diagnoses of systemic mastocytosis and mantle cell lymphoma meet the criteria for SM-AHNMD. The B-cell lymphoma is a symptom/marker of the AHNMD. In systemic mastocytosis with AHNMD, a myeloid or lymphatic malignancy is diagnosed with the SM. The prognosis is usually dominated by the non-mast cell malignancy.

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 following are not alternative names for any reportable disease process:

• Essential thrombocytopenia

The diagnosis of essential thrombocythemia is based on blood counts, but is usually a diagnosis made by excluding other myelodysplastic disorders. The following are reportable disease processes:

• Essential thrombocythemia (9962/3)

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 a single primary, chronic myeloid leukemia, NOS [9863/3] diagnosed 01/2008 per Rule M2. The patient was diagnosed with CML, NOS [9863/3] in 2008 and again in 2010 and 2012. Abstract a single primary when there is a single histology.

CML, Chronic phase; CML, Accelerated phase; and CML, Blast phase (Blast crisis) are listed under the Alternate Names section for CML, NOS in the Heme DB.

Not all histologies have transformations. If a transformation is not listed in the Heme DB, Rules M8 - M13 do not apply.

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.