Hodgkin Lymphoma and Cutaneous T-cell Lymphoma Sharing the PCM1-JAK2 Fusion and a Common T-cell Clone

Gregory M. Riedlinger, MD, PhD; Aleksander Chojecki, MD; Hana Aviv, PhD; David Weissmann, MD; Sonali Joshi, MS; Susan M. Murphy, MD; Kim M. Hirshfield, MD, PhD; Shridar Ganesan, MD, PhD


JCO Precis Oncol. 2019;2019(3) 

In This Article

Abstract and Introduction


The 2016 revision to the WHO classification of myeloid neoplasms and acute leukemia added myeloid or lymphoid neoplasms with PCM1-JAK2 as provisional entities.[1] The t(8;9)(p22;p24.1) translocation was first described in a patient in 1990,[2] and this cytogenetic abnormality was subsequently found to result in a PCM1-JAK2 fusion gene in 2005.[3] Approximately 50 patients with lymphoid or myeloid neoplasms associated with t(8;9)(p22;p24.1) PCM1-JAK2 have been reported, and these neoplasms have had an array of presenting diagnoses.[4,5] Common features associated with this translocation are a male predominance, hepatosplenomegaly, and eosinophilia. Diagnoses reported include Philadelphia chromosome–negative myeloproliferative neoplasms, myelodysplastic or myeloproliferative neoplasms, B-cell lymphoblastic leukemia, and rarely, T-cell lymphoma. One of the initial reports in the literature describing the t(8;9)(p22; p24) translocation was found in the cutaneous T-cell lymphoma of a patient who initially was reported to have lymphomatoid papulosis, followed by a diagnosis of Hodgkin disease, with evidence that all three of these diseases were derived from a common T-cell clone.[6] Here, we report what we believe is the second patient harboring the PCM1-JAK2 fusion with a history of cutaneous T-cell lymphoma who was later diagnosed with classic Hodgkin lymphoma. Additional immunophenotypic and molecular studies indicate that these are not two separate diseases but different manifestations of the same disease.