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When the latest WHO classification for heme neoplasms was written in 2008, there was a lot of controversy about whether or not the FL grading system was useful or not. A number of papers have been written stating that grades 1 and 2 do not have a statistically different survival or transformation rate. Given that the controversy had not been settled by those in the clinical world, the WHO recommended analyzing grades 1 and 2 together. They did not, however, remove either grade 1 or 2 from their classification. When the WHO intend to change their classification (have both grades classified under one histology number), they omit one code from their book (make it obsolete) and change the definition for the other code. The 2008 WHO book did not make either ICD-O-3 code obsolete. Therefore, we continue to collect the cases as designated by the pathologist. If the controversy is settled before the next WHO classification, you may see changes in the codes.

Additionally, since the 2008 WHO book was written, there have been some clinical papers challenging the designation of grade 3. They contend that grade 3 can be mistaken for low-grade.

The grades for follicular lymphoma are based on the number of centroblasts per high powered field (HPF). The number of centroblasts for grade 1 is 0-5; for grade 2 is 6-15, for grade 3a and 3b is >15 centroblasts. 3a has centrocytes and 3b has no centrocytes.

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

Aplastic anemia is not reportable and it is not an alternative name for refractory anemia.

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] per Rule PH5. Note 1 for Rule PH5 states CLL always has peripheral blood involvement. If the peripheral blood is positive for CLL/SLL and no bone marrow biopsy is done, code the primary site to C421 [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.

Reactive thrombocytosis is not reportable and is not an alternative name for essential thrombocythemia [9962/3].

Only the following are listed as alternate names for essential thrombocythemia in the Heme DB:

• Essential hemorrhagic thrombocythemia

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, this case should be accessioned as two primaries. According to Rule M10, one is to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm AND there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis.

The histology for the 1996 chronic neoplasm is coded to 9670/3 [small lymphocytic lymphoma]. The histology for the 2012 acute neoplasm is 9680/3 [diffuse large B-cell lymphoma].

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 following are not alternative names for any reportable disease process:

• Essential thrombocytopenia

The diagnosis of essential thrombocythemia is based on blood counts, but is usually a diagnosis made by excluding other myelodysplastic disorders. The following are reportable disease processes:

• Essential thrombocythemia (9962/3)

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.

Castleman disease, NOS, is not reportable for cases diagnosed 2010 and later. However, when Castleman disease is diagnosed in connection with large B-cell lymphoma [9738/3], it is 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.

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.

For cases diagnosed 2007 or later, accession a single primary, papillary transitional cell carcinoma of the bladder and ureter [C689, urinary system, NOS] diagnosed in 2010. The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Urinary MP Rules because site specific rules exist for this primary.

Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. This patient has urothelial tumors in two or more of the listed sites (bladder, ureter and renal pelvis) diagnosed within 3 years.

When C689 is assigned because tumors of the bladder and tumors of the ureter were determined to be a single primary and the site of origin is not known (as in this example), rule M8 is applied when a subsequent tumor is diagnosed in one of the listed sites. However, if site C689 [urinary system, NOS] was assigned for other unknown urinary primary site situations, rule M8 would not be used.

Rule M8 was written specifically for urothelial tumors in the renal pelvis, ureter, bladder and urethra. Paraurethral gland [C681] and overlapping lesions of urinary organs [C688] do not belong in rule M8.

We will add this issue to the list of possible revisions for the next edition of the MP/H Rules.

Code the histology to 8413/3 [eccrine adenocarcinoma]. This is the most specific code available for this diagnosis.

According to our expert pathologist advisor, "The adnexal glands in the skin, sweat (eccrine) glands and apocrine glands, all have ducts which connect the business portion of each gland to the skin surface. Some of the adnexal tumors have features of differentiation which appear to be duct-like, hence the designation 'ductal.'"

In addition, "The 'squamoid' simply indicates some degree of squamous differentiation, but doesn't alter the usefulness of [code 8413/3] because we have no way of coding anything more specific in this case anyway."