eJHaem,2022年
Jad Sibai, RuiQi Chen, Ibrahim Al Nabhani, Maria Agustina Perusini, Hassan Sibai
LicenseType:Unknown |
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy. It is associated with poor prognosis and heterogenous presentation. The CD123-directed cytotoxin, Tagraxofusp, is a targeted therapy for BPDCN. Here, we report an 81-year-old female diagnosed with BPDCN. The patient was treated with Tagraxofusp and underwent a remarkably long remission (>20 months) without stem-cell transplantation. She, however, experienced blue toe syndrome and left foot gangrene. We postulate that these previously unreported side effects were caused by microembolization. Characterization of the incidence of thrombo- and microembolizations in such a context, as well as prophylactic management options, are warranted.
eJHaem,2022年
Verna Cheung, James England, Dawn Maze, Hassan Sibai
LicenseType:Unknown |
Myeloproliferative neoplasms (MPNs) are a group of rare clonal disorders of hematopoietic progenitor cells that are associated with morbidity from disease-related symptoms, thrombotic events, and risk oftransformation to acute myeloid leukemia (AML) [1]. The three mostcommon MPNs are polycythemia vera (PV), essential thrombocytosis(ET), and myelofibrosis (MF). Patients with MPN can experience a constellation of debilitating symptoms that negatively impact their qualityof life [2–5]. In prior surveys of patients with MPNs, bone pain was thefourth most common symptom reported by 44% of patients and ratedas “very severe” in up to a third of MF patients [2–5].In very rare situations, patients with MPN can develop osteolyticlesions [6–21]. We report the first case of an osteolytic lesion in achronic-phase PV patient, and review published case reports of MPNpatients with osteolytic lesions to summarize the clinical characteristics and implications for patient care.
eJHaem,2023年
Ibrahim Al Nabhani, Claire Andrews, Jad Sibai, Eshetu Atenafu, Taylor Young, Steven M. Chan, Vikas Gupta, Dawn Maze, Aaron D. Schimmer, Andre C. Schuh, Karen Yee, Hassan Sibai
LicenseType:Unknown |
1000 mg/dl or 11.4 mmol/L). The incidence of hypertriglyceridemia associated with PEG-asparaginase in adult patients was high (67.5%). Therefore, checking TGs at baseline and monitoring levels while receiving PEG-asparaginase need to be considered and studied in prospective studies. However, in patients with hypertriglyceridemia not complicated by acute pancreatitis, re-challenging is safe once TG levels normalize.
eJHaem,2023年
Verna Cheung, Fotios V. Michelis, Hassan Sibai
LicenseType:Unknown |
To date, allogenic stem cell transplant (ASCT) remains the only potential curative option for patients with primary myelofibrosis (PMF). However, relapse rates and associated mortality remain a concern. A second ASCT may not be feasible due to advancing age, declined functional status, donor unavailability, toxicities associated with a second ASCT. Herein, we report the first case of utilizing initially azacitidine and subsequently oral decitabine + cedazuridine (decitabine), in the context of relapsed PMF post-ASCT. Utilizing both hypomethylating agents provided disease control and improved donor/myeloid lineage chimerism levels, and the patient also remained transfusion independent, with preserved functional status and quality of life.