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

Accession a single primary, acute myeloid leukemia [9861/3] diagnosed in 2004 per Rule M3.

When there is a myeloid sarcoma diagnosed simultaneously or after a leukemia of the same lineage, it is a single primary. The myeloid sarcoma is actually caused by the AML progressing. The myeloid cells in the bone marrow or blood literally "seep out" and implant in the tissue.

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.

This is a single primary, essential thrombocythemia [9962/3] diagnosed in 2007. The 2010 Heme DB and Manual should not have been used to determine the number of primaries in this case. The Heme DB applies only to cases diagnosed 2010 and later.

In order to determine the number of primaries, use the rules in place at the time of the subsequent 2009 diagnosis of primary myelofibrosis. Per the Single Versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table, a diagnosis of essential thrombocythemia [9962/3] followed by a diagnosis of primary myelofibrosis [9961/3] is a single primary.

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

Accession a single primary per Rule M2 which indicates you are to abstract a single primary when there is a single histology. Code the histology to 9983/3 [refractory anemia with excess blasts type 2 (RAEB-2)].

Per Appendix F, myelofibrosis, NOS, is NOT a synonym for primary myelofibrosis. Myelofibrosis, NOS, if not specified to be myelofibrosis, therefore, is not reportable.

Per PH29, code the specific histology when the diagnosis is one non-specific (NOS) histology (MDS) and one specific histology (RAEB-2) AND the Multiple Primary Calculator confirms the specific histology and NOS histology are the same primary (which it does).

Myelodysplastic syndrome, NOS is a generic disease description. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. The more specific myelodysplastic syndromes are: refractory anemia; refractory neutropenia; refractory thrombocytopenia; refractory anemia with ring sideroblasts; refractory cytopenia with multilineage dysplasia; refractory anemia with excess blasts; and refractory cytopenia of childhood. If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific 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.

Code the histology to 9680/3 [diffuse large B-cell lymphoma (DLBCL)].

For CLL (and CLL/SLL), Richter's transformation represents when CLL changes into DLBCL. In this case, there was a biopsy that demonstrated a diagnosis of the chronic disease (CLL/SLL) transforming (Richter's transformation) into an acute disease DLBCL.

Per Rule M8, one is instructed to abstract the acute neoplasm as a single primary when both a chronic (CLL/SLL) and an acute neoplasm (diffuse large B-cell lymphoma (DLBCL)) are diagnosed simultaneously there is documentation of only one positive bone marrow biopsy, lymph node biopsy or tissue biopsy.

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 2007 or later, code the histology as 8213/2 [carcinoma in situ in a serrated adenoma].

The steps used to arrive at this decision are:

: Apply ICD-O-3 rule F (Matrix principle) and assign the behavior code /2 when the behavior assigned by the pathologist differs from the usual behavior as given in the ICD-O-3.

: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text) and go to the Colon Histology rules.

: Start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at rule H4. Code the histology as 8213/2.

Note: The histology 8213 (adenocarcinoma in serrated adenoma) will be added to rule H4 in the next revision.

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

Per Appendix F, monoclonal B-lymphocytosis of uncertain significance (MLUS) is not reportable.

Some papers point out that a lymphocyte count less than five thousand is equivalent to monoclonal B-lymphocytosis of uncertain significance (MLUS) or monoclonal B-cell lymphocytosis (MBL). A lymphocyte count of five to thirty thousand could be smoldering chronic lymphocytic leukemia (CLL). The diagnosis of MLUS is a benign process that does not meet the criteria for CLL.

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.

Venous angiomas (9122/0) are not reportable wherever they arise. The primary site for venous angioma arising in the cerebellum is C490.  The combination of 9122/0 and C490 is not reportable. Venous angioma is a venous abnormality, currently referred to as a developmental venous anomaly (DVA).

For cases diagnosed 2007 or later, accession a single primary, left upper lobe squamous cell carcinoma diagnosed 7/27/2007.

The steps used to arrive at this decision are:

Go to the General Information notes for Determining Multiple Primaries for Solid Malignant Tumors in the Multiple Primary and Histology Coding Rules Manual.

General Information Rule 7 states "Use the multiple primary rules as written unless a pathologist compares the present tumor to the "original" tumor and states that this tumor is a recurrence of cancer from the previous primary."

Accession a single primary. Do not apply the Multiple Primary rules because the pathologist compared the 2007 and 2010 slides and determined this was a recurrence and not a new primary.

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

This case should be accessioned as two primaries per Rule M13. Code the histology for the 7/31/08 diagnosis to 9680/3 [diffuse large B-cell lymphoma] and the code the histology for the 10/14/2010 diagnosis to 9698/3 [follicular lymphoma, grade 3A of 3]. Rule M13 applies to this case because the neoplasm was originally diagnosed in the blast or acute phase (DLBCL) and reverted to a less aggressive or chronic phase (follicular lymphoma) after treatment.

Per the "Transformations to" section in the Heme DB for follicular lymphoma, grade 3 transforms to diffuse large B-cell lymphoma [9680/3]. This means that the follicular lymphoma is the chronic neoplasm and that DLBCL is the acute neoplasm. In this case, the chronic neoplasm was diagnosed after the acute neoplasm was diagnosed and treated (with chemotherapy).

Do not Stop at Rule M4 because diffuse large B-cell lymphoma and follicular lymphoma (both NHL's) were not present in the same node(s) AT THE SAME TIME.

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.