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We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3).

The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern.

In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis.

We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns.

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

This case is accessioned as a single primary diagnosed on 5/14/12 as acute myeloid leukemia with myelodysplasia-related (e.g., del(5q)) changes [9895/3] per Rule M2. The patient was diagnosed with a single histology, acute myeloid leukemia with myelodysplasia-related changes per the submitted information.

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.

Assign grade code 1 for well-differentiated NET (8240/3). Well-differentiated is synonymous with NET, grade 1, according to WHO Classification of Tumors of the Digestive System.

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

This case should be accessioned as a single primary, non-Hodgkin lymphoma (previously called poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell) [9591/3] diagnosed in 1991.

Determining the number of primaries is based on the rules in effect at the time of each diagnosis. The original lymphoma was diagnosed in 1991 and the first transformation to follicular lymphoma in 2008. The pre-2010 rules for coding histology and determining multiple primaries must be applied first because the rules changed for diagnoses occurring 2010 or later. Per the Single Versus Subsequent Primaries Table, poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3] is the same primary as follicular lymphoma [9690].

The Heme DB and Manual are used to confirm that the 2010 recurrences of follicular lymphoma, grade 1 [9695/3], and follicular lymphoma, grade 3 [9698/3], are the same primary according to the Heme Calculator check required per Rule M15. Per the Heme DB page, the diagnoses follicular lymphoma, grade 3 [9698/3] and follicular lymphoma, grade 1 [9695/3] are comparable to follicular lymphoma [9690] as stated in the 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.

Rule M7 applies. Abstract the bladder diagnosis and the renal pelvis diagnosis as separate primaries.

The lung diagnosis is metastatic. The MP/H rules do not apply to metastatic tumors.

For cases diagnosed 1998-2003, do not code the EOD-Clinical Extension field based on elevated PSA alone. If a recognized practitioner states that there is metastasis, then metastasis should be coded.

In this case, code the EOD-Clinical Extension field to 85 [Metastasis] because it is Stage D. But if you had D1 or D2 staging based on the involvement of lymph nodes, then that involvement would be coded under EOD lymph nodes and not under the clinical extension field.

For cases diagnosed prior to 1/1/2010:Code the primary site to tonsil (C099). This advanced stage lymphoma involves an extranodal site (tonsil) and that site's regional lymph nodes (cervical). The lymphoma has also spread to non-regional lymph nodes (mediastinal and mesenteric). Code the tonsillectomy as surgery of primary site.

For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

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

Granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously (i.e., at the same time or within 21 days of on another).

Apply Rule M3 to this case which states to abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. Code the primary site to C421 [bone marrow] with histology coded to 9873/3 [acute myeloid leukemia, M1]. The FAB category is an older classification that is seldom used. Changes from FAB 1 to FAB 7 do not constitute a new primary.

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.

Code primary site to C445 [skin, back] and histology to 9718/3 [cutaneous anaplastic large cell lymphoma] .

Per Rule M6, abstract a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location. For this case, there is cutaneous follicular (follicle) center lymphoma (9597/3) and cutaneous anaplastic large cell lymphoma (9718/3).

Per Rule PH22, code the primary site to the site or origin (skin, back) and the histology to the NHL with the numerically highest ICD-O-3 code. In this case, that would be 9718/3.