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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.

Per the Abstractor Notes in the Heme DB, primary effusion lymphoma (PEL) is unusual in that the majority of cases arise in body cavities, such as the pleural, pericardial, and peritoneal cavities. Because there are no ICD-O-3 codes for the pleural space, pericardium, or peritoneal cavity, code the primary site to pleura C384 when the neoplasm arises in the pleural cavity, to pericardium C380 when it occurs in the pericardium, and to peritoneal cavity C482 when it occurs in the peritoneum.

Typically only one body cavity is involved. However, if multiple regions are positive for PEL as in this case, code the primary site to C809 per Rule PH27. Rule PH27 indicates one is to code the to primary site C809 when there is no evidence of lymphoma in lymph nodes AND the physician in the medical record that he/she that the lymphoma in an

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.

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.

Unless the disease is specified as primary, idiopathic, essential, or the physician states there is a myeloproliferative neoplasm, the term thrombocytosis, NOS is not reportable. Thrombocytosis, NOS, is the presence of high platelet counts in the blood. Thrombocytosis can be associated with chronic infections and other diseases as well as with myeloproliferative disease. Thrombocytosis, NOS is listed in Appendix F as a Non-Reportable Term.

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.

Thrombocytopenia NOS is not a reportable diagnosis per Appendix F. Thrombocytopenia and Refractory Thrombocytopenia are not the same disease.

Thrombocytopenia is caused by a decreased number of platelets in the blood. Non-malignant causes include disseminated intravascular coagulation (DIC), drug-induced non-immune thrombocytopenia, drug-induced immune thrombocytopenia, hypersplenism, immune thrombocytopenic purpura (ITP), thrombotic thrombocytopenic purpura, and infections of the 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.

Code the histology to 9591/3 [B-cell lymphoma, NOS].

After searching the Heme DB for the term , no B-cell leukemia/lymphoma NOS code is found. However, the diagnosis of B-cell lymphoblastic leukemia/lymphoma is found. This case scenario does not specify that this is a lymphoblastic leukemia/lymphoma; therefore, the histology code 9811/3 [B-cell lymphoblastic leukemia/lymphoma, NOS] cannot be applied.

A subsequent search of the Heme DB for the term returns "Non-Hodgkin lymphoma, NOS" [9591/3]. Under the Alternative Names section of the Heme DB, B-cell lymphoma, NOS, is a synonym for Non-Hodgkin lymphoma, NOS. Therefore, the B-cell lymphoma NOS code [9591/3] is the most appropriate histology code to use for this case.

This will be added to the next revision of the Heme DB and 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.

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 unknown primaries treated with a lymph node dissection and diagnosed 1/1/2003 and after, code:

1) Surgery to Primary Site: 98 [All unknown and ill-defined disease sites, WITH or WITHOUT surgical treatment].

2) Scope of Regional Lymph Node Surgery: 9 [Unknown or not applicable].

3) Surgical Procedure of Other Site: 1 [Surgery to other site(s) or node(s), NOS; unknown if regional or distant].

4) Radiation Sequence with Surgery: 3 [Radiation after surgery]. Any planned surgical treatment is used to code radiation/surgery sequence (per CoC I&R).

Code Rituxan as chemotherapy. Code 90-Yttrium as radioisotope. Code 131-Iodine as radioisotope when given with Rituxan as treatment for lymphoma.

Zevalin is a monoclonal antibody conjugated to Yttrium 90. Bexxar is a monoclonal antibody conjugated to Iodine 131. In both drugs, the monoclonal antibody is only the delivery agent for the radioisotope. Both drugs should be coded as radioisotopes. The one-two-three punch of Rituxan and zevalin followed by Rituxan and Bexxar should be coded as chemotherapy plus radioisotopes. Zevalin is also used by itself for people who have not responded to Rituxan.