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

Code the histology to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)] per Rule PH15. Code the histology to the non-Hodgkin lymphoma (NHL) with the numerically highest ICD-O-3 code when two or more NHLs are present in the same present in the same lymph node(s) or lymph node region(s), tissue(s), organ(s), or bone marrow. Both follicular lymphoma [9690/3] and SLL [9823/3] are types of NHL. Therefore, the histology is coded to 9823/3.

This composite histology represents a single primary per Rule M4. The rule states to abstract 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).

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.

Code the primary site to C419 [bone, NOS] per Rule PH30.

This patient has widely metastatic disease. Per Rule PH30, one needs to reference the Heme DB to determine the primary site and histology for this case. Per the Abstractor Notes section, Langerhans cell histiocytosis arises in the bone and many times can involve multiple bones, along with other organs and lymph nodes. Although the right femur was biopsied, this does not prove that the primary site is the femur [C402] because the patient has what was described as extensive bony metastatic disease.

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.

When the latest WHO classification for heme neoplasms was written in 2008, there was a lot of controversy about whether or not the FL grading system was useful or not. A number of papers have been written stating that grades 1 and 2 do not have a statistically different survival or transformation rate. Given that the controversy had not been settled by those in the clinical world, the WHO recommended analyzing grades 1 and 2 together. They did not, however, remove either grade 1 or 2 from their classification. When the WHO intend to change their classification (have both grades classified under one histology number), they omit one code from their book (make it obsolete) and change the definition for the other code. The 2008 WHO book did not make either ICD-O-3 code obsolete. Therefore, we continue to collect the cases as designated by the pathologist. If the controversy is settled before the next WHO classification, you may see changes in the codes.

Additionally, since the 2008 WHO book was written, there have been some clinical papers challenging the designation of grade 3. They contend that grade 3 can be mistaken for low-grade.

The grades for follicular lymphoma are based on the number of centroblasts per high powered field (HPF). The number of centroblasts for grade 1 is 0-5; for grade 2 is 6-15, for grade 3a and 3b is >15 centroblasts. 3a has centrocytes and 3b has no centrocytes.

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.

Per Rule PH27, code the primary site to C809 [unknown primary]. According to Rule PH27 one is to code the primary site to unknown primary site C809 when there is no evidence of lymphoma in lymph nodes AND the physician documents in the medical record that he/she suspects that the lymphoma originates in an organ(s) OR multiple organ involvement without any nodal involvement.

If further workup is done and a primary site is determined, update the primary site for this case.

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.

If the bone is involved, code the primary site to bone. Langerhans more commonly starts in the bone and extends to the soft tissue.

If bone is not involved, code primary site to C492, Connective, subcutaneous and other soft tissues of lower limb and hip.

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.

Code the histology to diffuse large B-cell lymphoma [9680/3] because the physician states this is a DLBCL and is treating the patient accordingly. Although the pathology report was only compatible with DLBCL, there was a subsequent clinical diagnosis that confirmed a diagnosis of DLBCL. In addition, the patient was treated for DLBCL.

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 PH24, code primary site to orbit. According to Rule PH24, one is to code the primary site to the organ when lymphoma is present only in an organ. Note 2 under this rule also instructs one to capture the secondary involvement of distant lymph nodes and/or bone marrow in CS extension fields.

If the physician had not confirmed the primary site as orbit, you would have used Rule PH22 when the primary site is not indicated.

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 M2, this is a single primary because there is a single histology. The bone marrow initially showed a non-specific B-cell lymphoma. WM is a type of B-cell neoplasm. After immunophenotyping, a more specific histologic diagnosis of WM was made. In this case a single histology (WM) is diagnosed by the definitive diagnostic method (serum paraprotein demonstrating IgM), so it accessioned as a single primary.

Per PH16, code the histology to 9761/3 [Waldenstrom Macroglobulinemia (WM)] and the primary site to C420 [blood].

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.