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

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is not reportable. Per Appendix F, HLH is caused by an over stimulated immune system (infection, etc.). It is a clinical syndrome associated with a variety of underlying conditions. To be reportable, a child's diagnosis must state "fulminant hemophagocytic syndrome" to be reportable (9724/3). This is not the situation in this case.

"Hemophagocytic lymphohistiocytosis" is also listed in Appendix F: Non-Reportable List for Hematopoietic Diseases.

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.

Idiopathic and autoimmune types of thrombocytopenia are not reportable. Thrombocytopenia and thrombocythemia are not synonyms. Cytopenia and cythemia have different definitions. See Appendix F: Non-Reportable List for Hematopoietic Diseases.

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.

Indolent myeloma [9732/3] and smoldering myeloma [9732/2] are reportable terms synonymous with plasma cell myeloma. Monoclonal gammopathy of undetermined significance (MGUS) [9765/1] is not reportable.

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 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.

Per Rule M2, this is a single primary because there is a single histology. Code histology to 9823/3 [CLL/SLL]/

The distinction of CLL vs. SLL cannot be made on bone marrow biopsy in isolation. The pathologist cannot make a diagnosis of CLL vs SLL without having peripheral blood counts available for review. If the patient was treated for CLL in the past, that may alter the peripheral counts seen in 2010 (e.g., lymphocytosis). The distinguishing feature is peripheral lymphocytosis in CLL (not seen in SLL). The disease looks the same and both will often have bone marrow involvement and lymph node involvement. If the patient had true CLL in 2005, then any subsequent lymph node (or other) biopsy consistent with CLL/SLL remains consistent with the original diagnosis of CLL. I would not change the original CLL code.

I agree with the previous response. We have to assume the 2005 diagnosis included a peripheral blood supporting that diagnosis. Otherwise, CLL and SLL look the same in nodes and marrow. The interplay between the two "diseases" is expected. This is why they are considered a single 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 PH25, code the primary site of the diffuse large B-cell lymphoma to C649 (kidneys) and laterality to 4 (bilateral). Per PH25, code the primary site to the organ when a lymphoma is present in an and that . This patient had involvement of an organ (bilateral kidneys) as well as regional lymph nodes for that organ. The retroperitoneal lymph nodes are regional for the kidney.

The diffuse large B-cell lymphoma is an acute transformation of the chronic lymphocytic leukemia. Because the DLBCL occurred more than 21 days after the CLL, it is a new primary per Rule M10.

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.

Plasma cell dyscrasia and multiple myeloma are not synonymous terms. Plasma cell dyscrasia is not listed in the Alternate Names section of the Heme DB for plasma cell myeloma (multiple myeloma). Plasma cell dyscrasia is listed in the Alternate Names section of the Heme DB for MGUS [9765/1], which is not a reportable disease.

Plasma cell dyscrasia (PCD) is not reportable. PCD is a diverse group of neoplastic diseases that produces a serum M component (monoclonal immunoglobulin).

Usually these patients have a plasma cell morphology such as multiple myeloma or heavy chain disease. However, the registrar cannot diagnose multiple myeloma or heavy chain disease (or any other plasma cell neoplasm). There must be a physician statement and/or a positive biopsy to confirm a reportable 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.

Reactive plasmacytosis is not reportable unless there is another indication of a reportable neoplastic disease. Reactive plasmacytosis is "a well known pathological process described as occurring in a variety of situations including infections, autoimmune disease, diabetes mellitus, sideropenia, liver cirrhosis and neoplastic conditions including leukemia. This process, by definition, is assumed to be a reaction of the immune system to an unknown or poorly defined stimulus." Based on this definition, reactive plasmacytosis is not the same as a plasmacytoma, although it may indicate the presence of a neoplastic process, such as leukemia.

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 histology is coded as Plasma cell myeloma [9732/3]. The diagnostic confirmation is coded to 1 [positive histology].

Under the Definitive Diagnostic Methods section in the Heme DB it indicates that a bone marrow aspiration and bone marrow biopsy are procedures used to diagnose this disease process. This patient's diagnosis was based on the pathology (presumably from a bone marrow biopsy).

NOTE: This is a reportable case. Ambiguous terminology is used to accession cases (determine reportability) because it has been used for over 30 years to do so. Any deviation from using ambiguous terminology to determine case reportability would cause the reporting of incidence counts to vary. In this case, there was a reportable, ambiguous terminology diagnosis of plasma cell myeloma on the pathology report; as well as a reportable physician's statement/diagnosis of plasma cell myeloma.

Ambiguous terminology, however, is not used to report a more specific diagnosis for the Heme & Lymphoid neoplasms. For example, if the pathology report final diagnosis was "Myeloproliferative neoplasm, probably Polycythemia Vera" the histology would be coded as myeloproliferative neoplasm, unclassifiable [9975/3]. The ambiguous terminology indicates that the genetic testing, immunophenotyping, etc., probably are not complete or are not diagnostic of the more specific disease. Wait to code the histology until there is a definite diagnosis given.

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.