ALBERT

Description: Abstract 96 Introduction: Recently, a number of novel therapies have been under investigation in PTCLs, however, it remains a challenge to compare these agents and determine the impact on outcome. The purpose of this population-based study was to determine the spectrum of survival in PTCL patients (pts) following relapse and to explore factors influencing survival. The three most common subtypes encountered in North America were evaluated: PTCL-not otherwise specified (PTCL-NOS), angioimmunoblastic T-cell lymphoma (AILT), anaplastic large cell lymphoma, ALK positive (ALK-pos) and ALK-negative (ALK-neg). Material and Methods: The Centre for Lymphoid Cancer Database was screened to identify all pts ≥ 16 y with the above histologies that relapsed or had progressive disease (PD) following primary therapy. In addition to the timing of relapse (very early 〈 9 mos from diagnosis; early 9–24 mos and late 〉 24 mos), clinical information at the time of relapse was collected where possible. Responses were determined by the treating physician: Response = any clinical and/or radiographic response; PD = no change or disease progression. Results: In total, 276 pts diagnosed between 1976 and 2010 were identified with primary progressive or relapsed PTCL with initial diagnoses by the WHO classification. 68 were excluded: unconfirmed pathology (6), CNS disease (5), complications during primary CHT (17), no primary chemotherapy (CHT) (27), incomplete chart or lost follow-up (13). 208 pts were analyzed (PTCL-NOS = 109 (52%), ALCL = 54 (26%) (ALK-pos = 18; ALK-neg = 33; ALK status unknown = 3), AILT = 45 (22%). The majority of pts received anthracyclines as part of their primary CHT (89%). The median age at the time of relapse was 62.5 y (range 20 to 86 y) (PTCL-NOS 62 y, ALK-neg 62 y, ALK-pos 40.5 y, AILT 68 y). 129 (62%) pts were refractory to initial CHT. 53 pts were planned for HDC/SCT, however, only 38 (72%) ultimately received it (allo 17, auto 21). 37 pts (18%) were either too ill for any therapy (34) or refused therapy (3). The remaining pts received some type of treatment for their relapsed PTCL: systemic CHT (gemcitabine, CHOP(like), alkylators alone) (103) radiation alone (19) or steroids (11). The median follow-up for surviving pts after relapse/progression was ∼ 4 y. The median overall survival (OS) following relapse for the whole cohort was 7.0 mos (HDC/SCT 47.1 mos; no HDC/SCT 5.4 mos). The median progression free survival (PFS) following relapse was 4.6 mos (HDC/SCT 27.7 mos, no HDC/SCT 2.8 mos). The 3 y OS and PFS following relapse were 55% and 48%, respectively in the HDC/SCT group and similar whether an autologous or allogeneic SCT was performed. The corresponding 3 y OS and PFS estimates in the no HDC/SCT group were19% and 13.5 %, respectively. There was no difference in OS and PFS amongst the histologic subtypes. Considering the no HDC/SCT pts who received CHT (n=103), 55% were determined to have to have had a response to chemotherapy, and the median OS and PFS was 7.0 mos and 4.0 mos, respectively (responders vs non-responders/PD OS 16.7 mos vs 3.0 mos, p

Description: Introduction: Non-transfusion dependent thalassemia (NTDT) includes HbH disease, β-thalassemia intermedia (β-TI) and HbE/β-thalassemia. The transfusion requirements of patients with NTDT are variable and they are all at risk of developing iron overload and other complications. The prevalent genetic changes in thalassemia are different across geographical territories, accounting largely for the diverse clinical outcomes. The complication profile of NTDT in Southern China is less well studied than other areas. Data on age-related complications is sparse. The present study aims to describe the clinical-pathological features of adult NTDT patients in Hong Kong, and to evaluate the risk factors associated with complications. Method: A single-center observational study was performed during Jan 2017 to Jun 2018. Data collection included review of medical records for demographics; globin genotypes; hepatitis B and C status; transfusion requirement; splenectomy; iron chelation therapy; complications including gallstone disease, hypothyroidism or hypogonadism, diabetes mellitus (DM), extramedullary hematopoiesis (EMH), leg ulcer, venous thrombosis and cerebral ischemia. During the study period, steady state hemoglobin (Hb) and serum ferritin levels in the recent year were obtained; organ iron deposition assessed using liver and cardiac T2* at 1.5T MRI; liver stiffness measured by Fibroscan; and presence of pulmonary arterial hypertension (PAH) evaluated by echocardiography. The disease profile and prevalence of complications were described with descriptive statistics. Factors impacting clinical parameters and development of complication were studied with univariate regressions. Age and sex adjusted β-coefficients or odd ratios were then determined with multivariable regression analysis. Results: A total of 96 Chinese patients were recruited (mean age 50±15 years; 31% patients 〉60 years; females 66%). There were 63 (65%) patients having deletional HbH disease, 20 (21%) with non-deletional HbH disease (ND-HbHD), and 13 (14%) with β-TI. The mean Hb was 8.9±1.1 g/dL. Transfusion requirement was never in 39 (41%), occasional in 50 (52%), and regular in 7 (7%) patients. Ten (10%) patients were splenectomized. Iron chelation was given to 21 (21%) patients and the median duration of therapy was 3 (range 1-21) years. Respectively 4 (4%) and 2 (2%) patients were hepatitis B and C carriers. The median serum ferritin was 473 [IQR 217-1029] ng/mL. The median liver iron concentration (LIC) estimated by MRI T2* was 3.6 [IQR 1.7-7.1] mg Fe/g with 26% of patients having moderate to severe liver iron overload (≥7.1 Fe mg/g). In the study population, the prevalence of liver fibrosis (liver stiffness ≥7.1kPa) was 26%, gallstones 50%, hypo-thyroidism/-gonadism 8%, DM 16%, EMH 5%, leg ulcer 1%, thrombotic events 2%, cardiac iron overload 1%. No patients had PAH (Table 1). In multivariable regression, advanced age (〉60 years) was associated with lower Hb (p=0.03), higher risk of liver fibrosis (p=0.04) and DM (p

Description: Introduction Red blood cell (RBC) transfusion is a frequently performed medical procedure. But it is not without risk. Evidence suggests that restrictive transfusion is safe and effectively lessens the demand for blood components. Nevertheless, there exist considerable variations in transfusion practice among different centers. The hematology unit of Princess Margaret Hospital, a secondary referral center providing inpatient services for patients with various haematological malignancies and autologous stem cell transplantation, adopted a two-phase patient blood management program to reduce the demand for blood transfusions. Firstly, single-unit policy was implemented in Feb 2015 to replace the original protocol of transfusing two units of RBC in every transfusion episode. Secondly, the hemoglobin (Hb) trigger for transfusion decreased from 8g/dL to 7g/dL in asymptomatic and hemodynamically stable patients without cardiovascular disease in Aug 2016. The impact of these strategies to blood use and patient outcomes is evaluated in the present study. Method The study periods included 01/01/2014 - 12/31/2014 (Period A), 04/01/2015 - 03/31/2016 (Period B) and 10/01/2016 - 09/30/2017 (Period C). All patients admitted to the center during the study periods were recruited. Demographic data, RBC consumption, length of hospital stay and all-cause inpatient mortality were retrieved from the electronic medical record system. The proportions of single-unit transfusion and the mean Hb level transfusion triggers in different periods were compared with analysis of variance for continuous variables and logistic regressions for categorical variables respectively. Blood use in different periods was expressed in terms of RBC unit transfused per patient-day of hospitalization, and compared by age and sex adjusted incidence rate ratios (IRRs) obtained from zero-inflation negative binomial regression. To study the length of stay, negative binomial regression was performed to determine the age and sex adjusted IRRs for days of hospitalization in different periods. Clustering of data was further adjusted by applying robust standard errors. Generalized estimating equation for logistic regression, controlling for age, sex, days of hospitalization and number of previous admissions, was applied to compare the inpatient mortality in different periods. Results A total of 815 patients were recruited in the study, and the total number of admissions was 1,836. The number of patients in Period A was 232 (median age: 61 [IQR 49-71] years, male 54.7%) with 580 admissions, in Period B was 258 (median age: 63 [IQR 49-71] years, male 53.1%) with 566 admissions, and in Period C was 325 (median age: 62 [IQR 52-70] years, male 57.8%) with 690 admissions. The number of transfusion episodes in Periods A to C was 721, 923 and 803 respectively. The proportion of single-unit transfusion increased from 21.8% in Period A to 81.8% in Periods B (p

Description: Introduction Myelofibrosis (MF) is a myeloproliferative neoplasm (MPN) comprising primary myelofibrosis (PMF) and myelofibrosis developing from either polycythemia vera (post-PV MF) or essential thrombocythemia (post-ET MF). Methods Two hundred and sixty-seven consecutive patients with MF (PMF, N=206); post-PV MF, N=26; post-ET MF, N=35) diagnosed between January 1988 and December 2013 at seven regional hospitals in Hong Kong were prospectively followed up. Data on the clinicopathologic features and treatment outcome were collected. Results The median duration of follow-up was 45 (1 – 247) months. The median overall survival (OS) was 65 months (95% confidence interval, CI: 56.3–73.7). The 5-year and 10-year OS were 52.2% and 26.7% respectively. On univariate analysis, factors associated with an inferior OS included age 〉 65 years (hazard ratio ,HR, =1.81; 95% CI:1.33–2.48; P