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Richter's Syndrome

Kelly Posted Tue 25th of September, 2012 19:51:43 PM

How would you code this? When I looked up the definition for Richter's (transformation) Syndrome, I was not sure I would even use 204.10 as the primary dx?

CLL (chronic lymphocytic leukemia) with Richter's transformation/DLCL

SuperCoder Answered Wed 26th of September, 2012 17:40:22 PM

Richter's transformation or Richter's syndrome (RS) is a term for transformation of B-cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) to diffuse large B-cell lymphoma (DLBCL).M10 says when an acute phase occurs more than 21 days after a chronic disease, it is a new primary. Check the DB(Hematopoietic Database ) for information on CLL, the transformation data item lists DLBCL, so you have confirmed that this is a chronic disease that has transformed to an acute disease. Make a new abstract for the DLBCL 9680/3.

To determine the primary site, Use module 7 in the PH rules. This is a special module set up to help with site coding for lymphomas. You note that the stomach and multiple lymph node regions are involved. If there are lymph node chain(s) involved that are regional to the stomach, use PH35 and code the primary site stomach C169. If none of the involved lymph node chains are regional to the stomach, use PH336 and code lymph nodes, NOS C779.

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