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Produced: 09/12/2024 2:34 PM
Question 20230033
Inquiry Details
References:
#1: Heme & Lymph Manual & DB
#2: WHO Class Hem & Lymph Tumors. 2017, 4th Ed.
Question:
Histology--Heme and Lymphoid Neoplasms: What is the histology code for a final diagnosis of features compatible with EBV-positive nodal T-/NK-cell lymphoma? See Discussion.
Discussion:
This patient has only nodal involvement for this EBV positive NK/T cell lymphoma. There is no extranodal involvement, specifically no nasal involvement. Additionally, the immunophenotyping does not match the immunophenotyping listed for histology 9719. Therefore, histology 9719 (Extranodal NK/T cell lymphoma, nasal type) does not seem applicable to this case. This patient is an adult, so histology 9724 (Systemic EBV-positive T-cell lymphoma of childhood) also does not seem to be a match. The most recent WHO Classification seems to classify these as 9702. Is this the appropriate histology for an EBV-positive nodal NK/T-cell lymphoma in an adult?
Answer:
Assign code 9702/3 for EBV+ nodal T- and NK-cell lymphoma. Primary nodal EBV-positive T- or NK-cell lymphoma is a rare lymphoma type introduced in the 2017 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (4th Ed.) as a variant of peripheral T-cell lymphoma (PTCL). It presents more commonly in elderly and/or immunodeficient patients, lacks nasal involvement, and is more often of T-cell rather than NK-cell lineage.
