SEER Inquiry System - View
Source 1: 2012 Heme & Lymph Manual & DB
Reportability--Heme & Lymphoid Neoplasms: What is the primary site and is hemophagocytic lymphohistiocytosis treated with several rounds of chemotherapy considered reportable?
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.
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 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. hemophagocytic
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.