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Produced: 11/23/2024 5:50 PM
Question 20100018
Inquiry Details
References:
Heme & Lymph Manual & DB, Appendix F
Question:
Discussion:
A patient was diagnosed in 2010 with light chain disease based on SPEP and urine testing. Bone marrow aspiration and biopsy were done. Flow cytometry, cytogenetic studies and FISH for plasma cell disorders are all normal. Medical oncologist states diagnosis is light chain disease. Patient was started on Revlimid, dexamethasone and Velcade.
In reviewing the case reportability instructions, this seems to fall under Instruction 1, note 1. Immunoglobulin deposition disease, preferred term for light chain disease, is coded as 9769/1. This is normally a non-reportable diagnosis, but the patient was given cancer-directed treatment. Would this case be accessioned using the above morphology code and primary site of bone marrow [C42.1]?
Answer:
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case is not reportable. The histology is 9769/1 [light chain disease] in the Heme DB.
Light chains are produced in neoplastic plasma cells (multiple myeloma) and are called Bence-Jones proteins. The physician did the cytogenetic studies and FISH to rule out plasma cell disease. 50-60% of people with Light-chain deposition disease (LCDD) have an associated lymphoproliferative disorder, most commonly multiple myeloma. The remaining patients develop LCDD in the setting of progression of monoclonal gammopathy of unknown significance (MGUS) with no evidence of neoplastic plasma cell proliferation. This patient falls in this category, MGUS, which is not reportable.
History:
This SINQ question has been updated to the Heme & Lymph Neo Manual & DB published January 2014.
The original answer below was written based on the rules in 2010.
This case is not reportable.
Light chains are produced in neoplastic plasma cells (multiple myeloma) and are called Bence-Jones proteins. That is why your physician did the cytogenetic studies and FISH to rule out plasma cell disease. 50-60% of people with Light-chain deposition disease (LCDD) have an associated lymphoproliferative disorder, most commonly multiple myeloma. The remaining patients develop LCDD in the setting of progression of monoclonal gammopathy of unknown significance (MGUS) with no evidence of neoplastic plasma cell proliferation. Your patient falls in this category, MGUS, which is not reportable.
The answers for SINQ questions with 2010 ID numbers were written using the 2010 Heme & Lymph Manual & DB. The instructions for using the 2010 Hematopoietic Database were written for the version of the software in use as of 5/24/2011. The user interface of the web-based 2010 Hematopoietic Database available from the SEER website varies slightly from the 5/24/2011 version in that the web-based version provides all the disease information in one scrollable window.
For cases diagnosed 2010-2011, access the 2010 Hematopoietic Database at http://seer.cancer.gov/tools/heme/. Click on Hematopoietic Project. Click on Hematopoietic and Lymphoid Database. For 2010-2011 diagnoses, click on the "use the 2010 database" label in the upper right corner of the screen. The 2010 Hematopoietic Coding Manual (PDF) button will appear to indicate the correct version of the program is available now for query.
