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Report Produced: 04/01/2025 22:04 PM

Report Question ID Question Discussion Answer Year
20100065

Reportability--Heme & Lymphoid Neoplasms: Is "myeloproliferative syndrome, NOS" synonymous with "myeloproliferative syndrome" and "myeloproliferative disease" and, therefore, reportable under the new hematopoietic rules?

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.

2010
20130013

Reportability--Heme & Lymphoid Neoplasms: Is Mast Cell Activation Syndrome (MCAS) reportable?

MCAS has been given an ICD-9 code of 202.60 by our medical record coders. In the Progress Notes, the physicians state this is not the same as systemic mastocytosis. There is no listing for MCAS in the Hematopoietic and Lymphoid Database.

Mast Cell Activation Syndrome (MCAS) is not a reportable neoplasm unless it is specifically stated to be a result of a mast cell proliferative disorder that is reportable.

Per our expert pathologist, MCAS is a relatively new term used for conditions in which patients experience the symptoms of mast cell mediators in the absence of an increase/proliferation of mast cells. The diagnosis of this group of disorders is based in part on a complex of symptoms and on the demonstration of no increase in mast cells. Some of these diseases are difficult to separate from mastocytosis (which is reportable). Currently, this group of disorders is not part of the systemic mastocytosis/mast cell leukemia/mast cell sarcoma spectrum.

 2013
20170042

Reportability--Heme & Lymphoid Neoplasms: Is a diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with large cell transformation equivalent to a diagnosis of diffuse large B-cell lymphoma (DLBCL) without mention of Richter transformation or Richter Syndrome? See Discussion.

The patient has a history of CLL/SLL dating back to 2007, but has had progressive disease with development of a new left frontal brain tumor. The brain tumor resection proved CLL/SLL with large cell transformation, but neither the pathologist nor the managing physician called this a Richter transformation, Richter syndrome or provided a diagnosis of DLBCL. However, a large cell transformation of CLL/SLL is a Richter transformation. Can this be accessioned as a new acute neoplasm per Rule M10?

Accession as multiple primaries according to Hematopoietic and Lymphoid Neoplasm Coding Manual Rule M10. Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) followed by CLL/SLL with large cell transformation is multiple primaries because it is a chronic neoplasm followed by an acute neoplasm, more than 21 days in this case.

 2017
20130183

Reportability--Heme & Lymphoid Neoplasms: Is a peripheral blood finding consistent with involvement by monoclonal, lambda-restricted mature B cell population with co-expression of CD5 and CD23 reportable if, immunophenotypically, the case is consistent with a chronic lymphocytic leukemia/small lymphocytic lymphoma? See Discussion.

Peripheral blood: Final diagnosis: Leukocytosis absolute lymphocytosis monoclonal, lambda restricted B-cell population w/co-expression of CD5 and CD23 absolute increase in CD4=helper T cells. See comment.

Comment: Peripheral blood findings are consistent with involvement by monoclonal, lambda-restricted mature B cell population with co-expression of CD5 and CD23, which is immunophenotypically consistent with a chronic lymphocytic leukemia/small lymphocytic lymphoma immunophenotype. However, the absolute monoclonal population is only 3.02k/ul.

According to WHO criteria, in the absence of extramedullary tissue involvement, the monoclonal lymphocyte population must be greater than or equal to 5.0 k/ul. Therefore, in the absence of clinical evidence of extramedullary tissue involvement, the diagnosis is most consistent with a monoclonal B cell lymphocytosis. Review of initial analysis reveals well-defined groups of cells within lymphocyte, monocyte and granulocyte gates as defined by CD45 and sid-scatter characteristics (%'s are listed). Overall, peripheral blood findings are consistent with involvement by monoclonal, lambada-restricted B cell population with a chronic lymphocytic leukemia/small lymphocytic lymphoma immunophenotype.

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

This case is reportable. Code histology to 9823/3 [CLL/SLL].

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 on peripheral blood that is "consistent with" involvement by chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) immunophenotype.

The ambiguous terminology "consistent with" in the flow cytometry report is acceptable to determine reportability. Given that it is the only reportable histology mentioned in the scenario, it is also used to code histology. The instruction "Do not code histology based on ambiguous terminology" is intended to be used when there is a reportable NOS histology and reportable more specific histology stated in the diagnosis. Ambiguous terminology cannot be used to report the more specific diagnosis in cases of Heme & Lymphoid neoplasms.

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.

 2013
20140039

Reportability--Heme & Lymphoid Neoplasms: Is a statement of "JAK-2 positive polycythemia" reportable? See discussion.

Polycythemia, NOS is not reportable. However, there is a statement in the Heme Manual Glossary for JAK2 that states, "When JAK2 is positive, the MPN is definitely reportable." Does a positive JAK 2 always mean there is a reportable myeloproliferative disorder or must there also be an associated statement of a reportable neoplasm (e.g., myeloproliferative disorder, polycythemia vera, or essential thrombocythemia)?

A positive JAK 2 does not always mean there is a reportable myeloproliferative disorder. There must also be an associated statement of a reportable neoplasm (e.g., myeloproliferative disorder, polycythemia vera, or essential thrombocythemia). The glossary entry will be clarified.

2014
20130215

Reportability--Heme & Lymphoid Neoplasms: Is hemophagocytic lymphohistiocytosis synonymous with an EBV-associated lymphoproliferative disorder in children reportable? See Discussion.

Pathology report states: Prominent T-cell infiltrate with frequent immunoblast-like cells. COMMENT: Findings consistent with an acute EBV-associated hemophagocytic process. In addition, there is a prominent CD8 + T-cell infiltrate with many large, activated forms. This T-cell process may represent an EBV-associated lymphoproliferative disorder in children.

EBV-associated lymphoproliferative disorder in children is listed in the Heme database. However, throughout multiple admissions, the oncologist states the diagnosis as "hemophagocytic lymphohistiocytosis". Are the two the same condition?

The patient is being treated with Etoposide.

Per Appendix F, do not report this case based on the information provided.

The oncologist likely used the pathology report and clinical factors to determine the diagnosis of hemophagocytic lymphohistiocytosis, which is not reportable. Hemophagocytic lymphohistiocytosis is caused by an over stimulated immune system (infection, etc.). This clinical syndrome is associated with a variety of underlying conditions. To be reportable, it must state "fulminant hemophagocytic syndrome" (in a child) to be reportable (9724/3).

The pathology report for this case is not definitive. It states that the process "may" represent the EBV-associated lymphoproliferative disorder in children.

Follow back on this case to confirm reportability if possible.

 2013
20150008

Reportability--Heme & Lymphoid Neoplasms: Is idiopathic hypereosinophilia reportable? Must the diagnosis include the word 'syndrome'?

Idiopathic hypereosinophilia is not reportable.

Hypereosinophilic syndrome is a different entity and is a synonym for chronic eosinophilic leukemia.

2015
20190040

Reportability--Heme & Lymphoid Neoplasms: Is peripheral blood with a diagnosis of monoclonal B-cell lymphocytosis (MBL) with chronic lymphocytic leukemia (CLL) phenotype reportable for any year? See Discussion.

SINQ 20180050 and 20130041 appear to have conflicting answers regarding the reportability of MBL with CLL (immuno)phenotype.

While the question content of SINQ 20180050 does not reference the CLL phenotype, it is included in the Discussion as part of the oncologist's assessment. The answer does not address the clinical diagnosis of MBL with CLL-phenotype and simply states that monoclonal B-cell lymphocytosis is not reportable.

SINQ 20130041 does include the CLL phenotype information in the primary question and it is expanded on in the discussion as present in peripheral blood. Based on that information, the answer is that it should be reportable and coded as CLL (9823/3).

The description in the question is for 9823/1 per WHO blue book 2016. This description and code are not reportable. We will review the other SINQ questions and revise if necessary.

 2019
20130079

Reportability--Heme & Lymphoid Neoplasms: Is plasma cell dyscrasia reportable and synonymous with multiple myeloma? See Discussion.

Bone marrow biopsy and aspirate: Plasma cell dyscrasia with IgG kappa expression with FISH (+) for the following abnormalities: 3 copies of 1q21 (25/30 plasma cells) and an extra CCND1 signal (25/34 plasma cells) which is indicative of the presence of other chromosome 11 abnormalities possibly trisomy 11, a change known to occur in plasma cell neoplasms. Flow cytometry: A monoclonal plasma cell population is present, co-expressing cIgG, cKappa, CD56, & CD117 (up to 14% of analyzed cells).

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.

 2013
20130043

Reportability--Heme & Lymphoid Neoplasms: Is reactive plasmacytosis a reportable diagnosis that is equivalent to plasmacytoma?

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.

 2013