Salama, Mohamed

Lymphoproliferative disorders (LPD) are conditions characterized by the uncontrolled proliferation of B or T-cell lines. They encompass a wide spectrum of abnormalities, which may be broadly classified as reactive processes or malignant diseases, such as lymphoma, based on their cellular clonality and clinical behavior. While some of these disorders are rare, they may be encountered sporadically in clinical practice, causing diagnostic dilemmas owing to overlap in their clinical and imaging features with more common disorders. The updated 4th edition WHO classification of lymphoid neoplasms was released in 2016 to incorporate the rapid clinical, pathological, molecular biology and cytogenetic advances of some of these disorders. Despite these updates, very little information is presented in the literature from the radiology perspective. The aim of this article is to familiarize radiologists and other physicians with certain rare variants of B-cell lymphoproliferative disorders with a focus on imaging features of these disorders, as well as to provide an overview of some important updates contained within the new WHO classification of lymphoid neoplasms.

Understanding the pathogenesis and molecular biology of malignant lymphomas is challenging, given the complex nature and incongruity of these disorders. The classification of lymphoma is continually evolving to account for advances in clinical, pathological, molecular biology and cytogenetic aspects, which impact our understanding of these disorders. The latest fourth edition of the WHO classification of lymphoid malignancies was released in 2016 to account for these changes. Additionally, unlike B-cell lymphomas (BCL), T-cell lymphomas (TCL) are uncommon, and may be sporadically experienced in clinical practice. These disorders are rare, thus early diagnosis is challenging for both physicians and radiologists, owing to the overlap in clinical and imaging features with other, more common disorders. We aim to discuss some rare variants of T-cell lymphomas, including clinicopathologic and imaging features, as well as to give a glimpse of the updates contained within the new 2016 WHO classification.

Abstract Myelofibrosis (MF) is a clonal stem cell neoplasm characterized by abnormal JAK-STAT signaling, chronic inflammation, cytopenias, and risk of transformation to acute leukemia. Despite improvements in the therapeutic options for patients with MF, allogeneic hematopoietic stem cell transplantation remains the only curative treatment. We previously demonstrated multiple immunosuppressive mechanisms in patients with MF, including increased expression of programmed cell death protein 1 (PD-1) on T cells compared with healthy controls. Therefore, we conducted a multicenter, open-label, phase 2, single-arm study of pembrolizumab in patients with Dynamic International Prognostic Scoring System category of intermediate-2 or greater primary, post-essential thrombocythemia or post-polycythemia vera myelofibrosis that were ineligible for or were previously treated with ruxolitinib. The study followed a Simon 2-stage design and enrolled a total of 10 patients, 5 of whom had JAK2V617mutation, 2 had CALR mutation, and 6 had additional mutations. Most patients were previously treated with ruxolitinib. Pembrolizumab treatment was well tolerated, but there were no objective clinical responses, so the study closed after the first stage was completed. However, immune profiling by flow cytometry, T-cell receptor sequencing, and plasma proteomics demonstrated changes in the immune milieu of patients, which suggested improved T-cell responses that can potentially favor antitumor immunity. The fact that these changes were not reflected in a clinical response strongly suggests that combination immunotherapeutic approaches rather than monotherapy may be necessary to reverse the multifactorial mechanisms of immune suppression in myeloproliferative neoplasms. This trial was registered at www.clinicaltrials.gov as #NCT03065400.

Cesarean hysterectomy remains a life-saving surgery mostly performed under critical circumstances to control severe obstetric hemorrhage. Post-hysterectomy pelvic bleeding is a serious complication that may arise despite securing all accessible vascular pedicles. Pelvic compression presents a key last resort in the management of such challenging cases. No procedure-related intraoperative complications were encountered. One patient developed surgical site infection that was treated by antibiotics and secondary closure. In summary, we believe that the present balloon tamponade has employed a low-cost readily available equipment to provide a rapid and simple intervention which can be potentially lifesaving for women with pelvic hemorrhage post-hysterectom

Yacoub et al report excellent responses to pegylated interferon alfa-2a in patients with hydroxyurea-resistant/intolerant polycythemia vera or essential thrombocythemia.