Recent advances in the diagnosis and management of primary myelofibrosis

Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) in which dysregulation of the Janus kinase/signal transducers and activators of transcription (JAK/STAT) signaling pathways is the major pathogenic mechanism. Most patients with PMF carry a driver mutation in the JAK2, MPL (myeloproliferative leukemia), or CALR (calreticulin) genes. Mutations in epigenetic regulators and RNA splicing genes may also occur, and play critical roles in PMF disease progression. Based on revised World Health Organization diagnostic criteria for MPNs, both screening for driver mutations and bone marrow biopsy are required for a specific diagnosis. Clinical trials of JAK2 inhibitors for PMF have revealed significant efficacy for improving splenomegaly and constitutional symptoms. However, the currently available drug therapies for PMF do not improve survival. Although allogeneic stem cell transplantation is potentially curative, it is associated with substantial treatment-related morbidity and mortality. PMF is a heterogeneous disorder and decisions regarding treatments are often complicated, necessitating the use of prognostic models to determine the management of treatments for individual patients. This review focuses on the clinical aspects and outcomes of a cohort of Japanese patients with PMF, including discussion of recent advances in the management of PMF.

Validation of Hospital Anxiety and Depression Scale as a screening tool for psychological distress in advanced cancer patients undergoing chemotherapy

Advanced cancer patients experience stress and are at risk for developing psychological problems. Early diagnosis and suitable intervention are very important for their quality of life and compliance with chemotherapy. The Hospital Anxiety and Depression Scale (HADS) is one of the most commonly used mood scales; however, HADS has not been validated for use with cancer patients undergoing chemotherapy. The purpose of this study was to validate HADS as a screening tool for psychological distress among cancer patients undergoing chemotherapy. We also identified possible factors contributing to psychological distress and explored the development of original screening tools. Fifty subjects agreed to complete HADS and consult with psychiatric specialists. Possible contributing factors to psychological distress were identified by HADS and logistic regression analysis. The mean score for patients with psychological distress was 20.0 ± 8.93, and for patients without distress was 9.67 ± 6.11; scores for patients with psychological distress were significantly higher. The optimal cutoff point of psychological distress appeared to be 17. This cutoff point was associated with 72.7% sensitivity and 82.1% specificity. The factor “recurrent cancer” (p=0.043) had an odds ratio of 7.24 (1.21-61.2). We verified that HADS is a useful screening tool for cancer patients undergoing chemotherapy. The factor “recurrent cancer” was shown to contribute to psychological distress. Palliat Care Res 2011; 6(2): 150-157