Oncology & Hematology Coding Alert

Question: How do you code triple-hit lymphoma with MYC, BCL2 & BCL rearrangements? The provider selected diffuse ultimately as there is not a code for triple-hit lymphoma. I want to make sure I understand coding for triple-hit scenarios.

AAPC Forum Participant

Answer: You won’t find an entry for triple-hit lymphoma in the alphabetic index for ICD-10-CM. However, you can code this rare cancer easily once you know the World Health Organization (WHO) defines it as a “B-cell lymphoma unclassifiable with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL)” (Source: www.ncbi.nlm.nih.gov/pmc/articles/PMC3954668/). Additionally, your provider identified the lymphoma as diffuse, which will lead you to choose a code from C83.3- (Diffuse large B-cell lymphoma).

You will need to add a fifth digit to specify where the lymphoma is located, including lymph nodes of the head, face, and neck (fifth digit 1), interthoracic (2), intra-abdominal (3), axilla and upper limb (4), inguinal region and lower limb (5), intrapelvic (6), and multiple sites (8). For triple-hit lymphoma of the spleen, you’ll use fifth digit 7, and for extra-nodal and solid organ triple-hit lymphoma, you’ll use fifth digit 9. If your provider does not specify the site, you will use C83.30 (Diffuse large B-cell lymphoma, unspecified site).