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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]. Our hematopoietic specialty physicians state that involvement of peripheral blood is equivalent to bone marrow involvement because the marrow produces blood. In the absence of any other involvement, per Module 7 (Coding primary sites for lymphomas) Rule PH26, it states to code the primary site to bone marrow when the only involvement is bone marrow.

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.

For this case, code primary site to C189 [colon, NOS] per Rule PH24.

Mantle cell lymphoma usually begins with lymph node involvement and spreads to other tissue. However, it can begin in a lymphocyte such as those in the GI tract. Per the Abstractor Notes section in the Heme DB, patients usually present with advanced disease. About half will have some combination of B symptoms. Swelling of lymph nodes and spleen are usually present. Bone marrow, liver and GI tract involvement occurs in a very high percentage

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 histology to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Per the Alternate Names section in the Heme DB, B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma is one of the synonyms for 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..

Code the primary site to the involved lymph nodes [C770-C779]. Per Rule PH 8, it indicates you are 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. Note 2 further states that the bone marrow may or may not be involved. If the bone marrow is involved, code this information in the CS Extension field.

Per the Abstractor Notes section in the Heme DB, this is a systemic disease with widespread lymph node involvement as well as involvement of the peripheral blood. In addition, systemic involvement of extranodal sites (including lung) are often involved.

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 M10, 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. Two primaries should be accessioned for this case: refractory anemia with excess blasts (RAEB) [9983/3] (a chronic neoplasm), and acute myeloid leukemia [9861/3] (an acute neoplasm).

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 PH30, use the Heme DB to determine the primary site and histology when rules PH1-PH29 do not apply. Code the primary site to C419 [bone, NOS], assuming there are multiple bones involved in this case. If only one bone is involved, code the primary site to the specified bone.

In the Abstractor Notes section in the Heme DB, it indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen).

Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site to C419 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the Abstractor Notes.

The spleen and liver are typically not primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. This information will be added to the ABSTRACTOR NOTE 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.

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

Myeloproliferative syndrome and the myeloproliferative diseases were used in the past to describe myeloproliferative neoplasms. For cases diagnosed 2010 and forward, although the term "myeloproliferative syndrome" is not currently used to describe this disease, the synonyms "myeloproliferative syndrome" and "myeloproliferative disease" were added to the database for myelodysplastic/myeloproliferative neoplasm, unclassified [9975/3].

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 is accessioned as one primary and the histology is coded to 9732/3 [multiple myeloma].

To arrive at this answer, it is important to first try to determine how many different unique neoplasms there are to correctly identify the number of primaries to report. Per the Heme DB, plasma cell leukemia is an obsolete term. The current term and histology code for this diagnosis is 9732/3 [plasma cell myeloma]. Plasma cell myeloma and multiple myeloma are synonyms per the Heme DB. Therefore, per Rule M2 a single primary exists when there is a single histology. That takes care of the multiple myeloma/plasma cell myeloma and plasma cell leukemia, but not the plasmacytoma.

In checking the Heme DB, the terms plasma cell myeloma and multiple myeloma are not synonyms for plasmacytoma. Therefore, we are left to determine whether the multiple myeloma/plasma cell myeloma vs the plasmacytoma represents one or two primaries.

Under the Transformation section of the Heme DB, it indicates that plasmacytoma (a chronic disease process) transforms to multiple myeloma (an acute disease process). Per Rule M9, abstract a single primary and code the acute histology when both a chronic and an acute neoplasm are diagnosed simultaneously. The histology is coded to the acute neoplasm when there is no information on the biopsy regarding which is the "later" histology. This update will be added to the Heme Manual.

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 9837/3 [T lymphoblastic leukemia/lymphoma].

To determine the primary site for leukemia/lymphoma histologies, first go to Module 4. Per Rule PH8, code the primary site to the site of origin when lymph nodes, tissue or organs are involved. To determine a more specific histology, go to Module 7, rules for coding primary site for lymphomas. Per Rule PH20, code the lymph node region when multiple lymph node chains within the same region are involved. Mediastinal and hilar lymph nodes are intrathoracic lymph nodes. The substernal mass is also intrathoracic and is presumed to be a lymph node mass which involved the pericardium. For this case, code the primary site to C771 [Intrathoracic lymph nodes].

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.