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Question: 20120064 Status
Final

References
Source 1:   Heme & Lymph Manual & DB, Appendix F
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Notes:  
Source 2:  
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Question
Reportability--Heme & Lymphoid Neoplasms: If hemophagocytic lymphohistiocytosis treated with several rounds of chemotherapy is considered reportable, what is the primary site?

Discussion
Patient was diagnosed with hemophagocytic lymphohistiocytosis on blood and bone marrow biopsy. This was also referred to in the chart as hemophagocytosis and hemophagocytic syndrome. Hemophagocytic syndrome is listed in the Heme DB as 9724/3. The patient had several rounds of fairly aggressive chemotherapy. Would the correct primary site for histology 9724/3 be C421 [bone marrow], or C779 [lymph nodes, NOS]? See SINQ 20100113.



Answer
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.

History
This SINQ question has been updated to the Hematopoietic & Lymphoid Neoplasm Manual & Database published January 2014. The original answer below was written based on the rules in 2012 For cases diagnosed 2012 and later, access the Hematopoietic Database at

http://seer.cancer.gov/tools/heme/.

Click on Hematopoietic Project. Click on Hematopoietic and Lymphoid Database. The 2012 Hematopoietic Coding Manual (PDF) button will appear to indicate the correct version of the program is available now for query.

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is not reportable. The steps used to arrive at this decision are:

Step 1: Enter hemophagocytic in the Heme DB to find the histology. Click on the SEARCH button. Only one result is returned, "Systemic EBV-positive T-cell lymphoproliferative disease of childhood" [9724/3] is highlighted on the screen.

Step 2: Scroll down to the ALTERNATIVE NAMES information. Note that neither hemophagocytic syndrome (NOS) nor hemophagocytic lymphohistiocytosis (HLH) are given as synonyms and, therefore, this case is not reportable. Fulminant hemophagocytic syndrome in children is not the same as hemophagocytic syndrome (NOS).

HLH is caused by an over-stimulated immune system (infection, etc.). It is a clinical syndrome that is associated with a variety of underlying conditions. Systemic EBV-positive T-cell lymphoproliferative disease of childhood [9724/3] is not applicable. To use histology code 9724/3, the patient must be a child, and there must be documentation of FULMINANT hemophagocytic syndrome in the patient.



Last Updated
06/30/2014

Date Finalized
08/29/2012