[Successful treatment with brentuximab vedotin for MYC positive syncytial variant nodular sclerosis Hodgkin lymphoma].

A 25-year-old female patient was referred to our department due to fever and cervical lymphadenopathy. Computed tomography showed bulky mediastinal lesions and bilateral supraclavicular and axillary lymphadenopathies. Cervical lymph node biopsy revealed nodular sclerotic Hodgkin's lymphoma (NS-HL) comprised of large Hodgkin/Reed-Sternberg (HRS) cells with sheet-like aggregates. The patient was diagnosed with a syncytial variant (SV). Immunohistochemical staining of HRS cells was positive for CD30, PD-L1, and MYC and negative for Epstein-Barr virus encoding region (EBER). The patient was assigned clinical stage IIB according to the Lugano classification and the poor prognosis group according to the National Comprehensive Cancer Network (NCCN). Furthermore, treatment with AVD (adriamycin/vinblastine/dacarbazine) in combination with brentuximab vedotin (BV) was initiated to achieve a complete metabolic response. Histopathologically, SV has a high proportion of HRS cells, with high CD30-positive and low EBER-positive rates. Therefore, CD30-targeted therapies such as BV may be preferable for SV, even in localized NS-HL patients, thereby improving patient prognosis.