PCM1-JAK2 Fusion Tyrosine Kinase Gene-Related Neoplasia: A Systematic Review of the Clinical Literature.

Publication Title

The oncologist

Authors

Henry G Kaplan, Swedish Cancer InstituteFollow
Ruyun Jin, Center for Cardiovascular Analytics, Research, and Data Science; Providence St Joseph Heart Institute, Portland, ORFollow
Carlo Bifulco, Earle A. Chiles Research Institute, Robert W. Franz Cancer Research Center, Providence Portland Medical Center, Portland, ORFollow
James M Scanlan, Swedish Center for Research and Innovation, Swedish Medical Center, Seattle, WAFollow
David R Corwin, Swedish Medical Center, Seattle, WashingtonFollow

Document Type

Article

Publication Date

4-26-2022

Keywords

swedish cancer; swedish; seattle; portland; oregon; washington; cards; cards publication; genomics

Abstract

BACKGROUND: This review summarizes the case studies of PCM1-JAK2 fusion tyrosine kinase gene-related neoplasia. Recommended treatment includes JAK2 inhibitors and hematologic stem cell transplantation (HSCT), although the small number of patients has limited study of their efficacy. Herein, we present all available cases in the current searchable literature with their demographics, diagnoses, treatments, and outcomes.

METHODS: PubMed, ScienceDirect, Publons, the Cochrane Library, and Google were searched with the following terms: PCM1-JAK2, ruxolitinib and myeloid/lymphoid.

RESULTS: Sixty-six patients (mean age = 50, 77% male) had an initial diagnosis of myeloproliferative neoplasm (MPN) in 40, acute leukemia in 21 and T-cell cutaneous lymphoma in 5. Thirty-five patients (53%) had completed 5-year follow-up. The 5-year survival for the MPN, acute myelogenous leukemia (AML), acute lymphocytic leukemia, and lymphoma groups are 62.7, 14.9%, 40.0%, and 100%, respectively. Too few patients have been treated with ruxolitinib to draw conclusions regarding its effect on survival while the 5-year survival for MPN patients with or without HSCT was 80.2% (40.3%-94.8%) versus 51.5% (22.3%-74.6%), respectively. The T-cell cutaneous lymphoma patients have all survived at least 7 years.

CONCLUSION: This rare condition may be increasingly detected with wider use of genomics. Ruxolitinib can yield hematologic and molecular remissions. However, HSCT is, at this time, the only potentially curative treatment. Useful prognostic markers are needed to determine appropriate timing for HSCT in patients with MPN. Patients presenting with acute leukemia have a poor prognosis.

Area of Special Interest

Cancer

Area of Special Interest

Cardiovascular (Heart)

Specialty/Research Institute

Oncology