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Rule PH21 applies to this situation which states to code the primary site to multiple lymph node regions, NOS (C778) when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated. Axillary nodes are coded to C773 and inguinal nodes are coded to C774. There are two lymph node regions involved. Code the primary site to C778 [multiple lymph nodes].

If this patient had only bilateral axillary OR only bilateral inguinal nodes are involved, then PH20 would have applied and you would code to the specific lymph node region mentioned.

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.

This case is reportable if the patient was treated for plasmacytoma. When the physician calls the case plasmacytoma and treats the patient accordingly, report the case.

See Case Reportability Instructions #6: Report the case when there is a clinical diagnosis (physician's statement) of a reportable hematopoietic or lymphoid neoplasm.

Note 1: The clinical diagnosis may be a final diagnosis found within the medical record or recorded on a scan (CT, MRI for example)

Note 2: Report the case even if the diagnostic tests are equivocal. A number of hematopoietic neoplasms are "diagnoses of exclusion" in which the diagnostic tests are equivocal and the physician makes the clinical diagnosis based on the equivocal tests and the clinical picture. See the Heme DB for definitive diagnostic methods for the specific neoplasm being abstracted.

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.

Assign C269 for Gastrointestinal tract, NOS. Apply Rule PH24, code to the organ when only an organ is involved. This rule can be used for NOS sites such as GI tract, NOS.

Based on the information provided, this lymphoma is confined to the GI tract -- stomach and small bowel.

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

Per Rule M10, abstract two primaries. Per the Heme DB, polycythemia vera [9950/3] transforms to an acute myelogenous leukemia [9861/3]. According to Rule M10, one is to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (e.g., polycythemia vera) AND there is a second diagnosis of an acute neoplasm (e.g., acute myelogenous leukemia) more than 21 days after the chronic diagnosis.

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.

Without further information as to the type of acute myeloid leukemia, code the histology to 9861/3 [acute myeloid leukemia, NOS]. If further information on the specific acute myeloid leukemia becomes available, update the histology code.

Document that the pathology report states the acute myeloid leukemia is a "non-M3 type" in a text field. This documentation will help explain the choice of 9861/3 for this case. M3 refers to one of the eight FAB subtypes described by a group of French, American, and British leukemia experts in the 1970's who divided acute myeloid leukemias into subtypes, M0 through M7. They classified the disease based on the type of cell from which the leukemia developed and how mature the cells were. This was based largely on how the leukemia cells looked under the microscope after routine staining.

In this case, all we know is that the histology does not pathologically represent the M3 (acute promyelocytic leukemia (APL)) form of acute myeloid leukemia. We do not know which type of acute myeloid leukemia it does represent.

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 Abstractor Notes section, this case represents a single primary. Histology is coded to 9732/2 [multiple myeloma], which is now advanced.

Review the Abstractor Notes section in the Heme DB for multiple myeloma. It states that in multiple myeloma there is generalize bone marrow involvement. It further states that lytic bone lesions and bone tumor masses of plasma cells (plasmacytomas) are signs of advanced disease. According to the Discharge Summary, this patient had multiple lytic lesions and plasmacytoma which indicates advanced 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.

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.