Epidemiologic study of myelodysplastic syndromes in a multiethnic, inner city cohort

Little is known about the epidemiology of MDS in minority populations. The IPSS and newly released IPSS-R are important clinical tools in prognostication of patients with MDS. Therefore, we conducted a retrospective epidemiological analysis of MDS in an ethnically diverse cohort of patients. Demographics, disease characteristics, and survival were determined in 161 patients seen at Montefiore Medical Center from 1997 to 2011. We observed that Hispanics presented at a younger age than blacks and whites (68 vs. 73.7 vs. 75.6 years); this difference was significant (p = 0.01). A trend towards greater prevalence of thrombocytopenia in Hispanics was observed, but this was not significant (p = 0.08). No other differences between the groups were observed. Overall median survival after diagnosis was the highest among Hispanics (8.6 years) followed by blacks (6.2 years) and Caucasians (3.7). Adjusted hazard ratios however did not show significant differences in risk of death between the groups. The IPSS-R showed slightly better discrimination when compared to the IPSS in this cohort (Somers Dxy 0.39 vs. 0.35, respectively) but observed survival more was more closely approximated by IPSS than by IPSS-R. Our study highlights the possibility of ethnic differences in the presentation of MDS and raises questions regarding which prognostic system is more predictive in this population

Cerebral Venous Sinus Thrombosis (CVST): Long-Term Single-Center Experience

CVST is a rare location of thrombosis involving Dural/ cerebral venous sinuses. It affects around 5-10 people per million population annually. It is an uncommon but life-threatening form of stroke affecting younger individuals. Therefore, identifying and treating in a timely manner is critical. Rarer thrombotic disorders like paroxysmal nocturnal hemoglobinuria (PNH) or Janus Kinase 2 (JAK2) mutation positive myeloproliferative neoplasms (MPN) can rarely present with CVST. It can also present during pregnancy for the first time. Diagnosis is often established by Computed Tomography (CT) or Magnetic Resonance Imaging (MRI). Infections, certain medication use (asparaginase or birth control pills) could lead to CVST. Patients often present with headaches, seizures or neurological deficits. Management is often with systemic anticoagulation despite intraparenchymal hemorrhage. Reducing intracranial pressure by invasive approaches is sometimes needed.https://digitalcommons.unmc.edu/surp2022/1024/thumbnail.jp

Apixaban to Prevent Thrombosis in Adult Patients Treated With Asparaginase

The outcomes of pediatric acute lymphoblastic leukemia (ALL) have improved dramatically after intensification of the multi-agent chemotherapy backbone over a period of time starting from 1960’s, and asparaginase (ASP) is an important part of this success story. Adoption of such strategy in adult ALL and other high grade lymphoid neoplasms had been difficult due to higher anticipated toxicities including thrombosis. Many thrombosis prevention strategies including antithrombin (AT) replacement, Enoxaparin, unfractionated heparin (UFH) was studied but there is no firmly established evidence-based approach yet. We report the safety and efficacy of thrombosis prophylaxis with apixaban 2.5 mg orally twice daily for up to 3 weeks starting on the first day of receiving ASP. Characteristics of the 20 patients treated with ASP between the years 2017-2020 included a median age of 29.5 years (range 19-63 years), 80% male, 70% whites. Indication for ASP was ALL in 90%. A total of 75% received cryoprecipitate with a median of 10 units (range 0-105 units) in a 4-week period following ASP. The total incidence of thrombosis was 10% and 5% incidence while on anticoagulation. The incidence of major bleeding was 5% while on anticoagulation with apixaban. In conclusion, our study demonstrates the safety of using apixaban for thrombosis prophylaxis and concurrent cryoprecipitate

Association of insurance types and outcomes in acute promyelocytic leukemia

Understanding the association between insurance status and survival in an evolving US healthcare system remains a challenge but is essential to address healthcare disparities. We utilized National Cancer Database to evaluate the effects of insurance type on one-month mortality and overall survival (OS) in patients with acute promyelocytic leukemia. Among patients \u3c 65 years, one-month mortality was worse for uninsured patients and patients with Medicare compared to patients with private insurance. OS was similar between patients with private insurance and uninsured patients but worse for patients with Medicare and Medicaid/other government insurance. In multivariate analysis, older age and greater comorbidity burden conferred worse OS. For patients ≥ 65 years, insurance type did not affect one-month mortality and OS. Older age, greater comorbidity burden, and treatment at non-academic centers conferred worse one-month mortality and OS. Our results highlight healthcare disparities based on insurance types for both younger and older patients

Association of Insurance Types and Survival in Acute Promyelocytic Leukemia (APL)

Introduction: Health insurance, or lack thereof, is a significant barrier to health care access in the United States. Patients without insurance or with inadequate coverage are more likely to delay or forego treatment, even with acute illness or significant symptoms, leading to worse health outcomes. We aimed to analyze if insurance types impacted one-month mortality and overall survival (OS) in younger patients with APL

Associations of Budd-Chiari Syndrome and Factors Predicting Need for TIPS and Liver Transplantation: Long-Term Single-Center Experience

Background: Budd-Chiari Syndrome (BCS) is a rare thrombotic disorder that involves obstruction of hepatic venous outflow. It can result in increased sinusoidal pressure and portal hypertension. Patients may develop cirrhosis and liver failure from chronic injury, along with other complications such as ascites and varices. BCS is associated with thrombophilia and hematological disorders such as myeloproliferative neoplasms (MPNs) and other auto-immune diseases. Patients typically receive long-term anticoagulation to decrease the risk of recurrence of thrombosis along with supportive measures such as trans-jugular intrahepatic portosystemic shunt (TIPS) and liver transplant. Methods: We performed a retrospective study to describe the patient population with BCS at UNMC. We evaluated 23 patient medical records and collected data on demographics, risk factors, health history, BCS presentation, associated diseases, any acquired/inherited thrombophilia, laboratory results at the earliest available date from diagnosis, and treatments/procedures received. Model of End-Stage Liver Disease (MELD) and Child-Pugh scores were calculated and reported. Descriptive Findings: The median age for the 23 patients in our study was 36 years (range: 11-59 years). The percentage of females was 60.87%. The overall survival rate was 91.3%. Median follow-up duration was 89 months (range: 3-330 months). Of the total, 57% of the patients had myeloproliferative neoplasms (MPNs). Cirrhosis of liver was observed in 74% of the patients. 8.7% of patients developed BCS after receiving the COVID-19 vaccine (Ad26.COV2.S). 91.3% of patients received long-term anticoagulation. 65% of patients received TIPS and/or liver transplant. Conclusion: The most common primary cause of BCS in our study was MPNs (57%). Vaccine-induced thrombotic thrombocytopenia (VITT) after COVID-19 vaccine was found to be a newly described primary cause of BCS.https://digitalcommons.unmc.edu/surp2021/1046/thumbnail.jp