bluuebunny/crossref_metadata_2025_split huggingface dataset:
Outcome of patients younger than 50 years old diagnosed with myelodysplastic syndromes (MDS): Single institution experience.
Authors: Al-Kali Aref , He Rong , Patnaik Mrinal , Hanna Karen , Viswanatha David , Greipp Patricia T. , Zblewski Darci , Elliott Michelle A. , Litzow Mark Robert , Nguyen Phuong L. 
Published: September 2018

e18560 Background: Myelodysplastic syndromes (MDS) are rare hematological neoplasms typically seen in the elderly. Young MDS (yMDS) patients ( < 50 years old) have been reported to comprise between 3-6% in the Surveillance, Epidemiology and End Results (Ma X et al, Cancer 2007; Rollison R et al, Blood 2008) with a better overall survival (OS). Methods: 1012 MDS patients from 1993 to 2015 were found after IRB approval was obtained. All cases had their bone marrow slides reviewed at our institution. yMDS Patients were included as cohort 1, while the rest as cohort 2. Survival estimates were calculated using Kaplan-Meier curves and univariate and multivariate analyses was based on log-rank testing using JMP software version 10. Results: We found 68 (7%) yMDS patients with a median age of 42 years (range, 18-49). Female gender was more common (43% vs 31%, p = 0.05) while platelets were lower in yMDS (61 vs 102, p < 0.0001). Therapy related MDS (t-MDS) was more frequent (33% vs 17%, p = 0.005), as was transformation to acute myeloid leukemia (AML) in yMDS (28 % vs 11%, p = 0.0004). Allogenic hematopoietic cell transplantation (HCT) was more frequent in yMDS (42% vs 4%, p < 0.0001). Survival outcome: Median OS was longer for cohort 1 vs cohort 2 but did not reach statistical significance (43 vs 21 months, p = 0.1). Median progression free survival (PFS) was shorter for cohort 1 vs cohort 2 but also did not reach statistical significance (8 vs 12 months, p = 0.3). Median OS for cohort 1 based on R-IPSS was 44, 105, 40, 18, and 12 months for very low, low, intermediate, high and very high risk groups, respectively (p = 0.09). Median OS was shorter in t-MDS vs de novo MDS in yMDS (13 vs 47 months, p = 0.04). yMDS patients who transformed to AML had worse median OS (18 vs 93 months, p = 0.001). On multivariate analysis neither t-MDS nor R-IPSS had a statistically significant impact on OS. Conclusions: MDS is rarely diagnosed under the age of 50 with IPSS-R being less powerful in detecting mOS. Among yMDS patients, there was a higher proportion of t-MDS compared to older patients with subsequent higher rates of AML transformation and higher rates of allogeneic HCT. In our study, we did not find an improved OS for yMDS patients compared to older patients.
Correlation between blast percentage in myelofibrosis and outcomes: A single-center experience.
Authors: Masarova Lucia , Bose Prithviraj , Newberry Kate , Abou Zahr Abdallah , Cortes Jorge E. , Kantarjian Hagop M. , Verstovsek Srdan 
Published: September 2018

e18562 Background: Patients with accelerated phase (AP) myelofibrosis (MF; >10% blasts [BL] in bone marrow [BM] or peripheral blood [PB]) have shorter overall survival (OS) than those in the chronic phase (<10% BM/PB BL). However, outcomes of patients with “lower” BM/PB BL (1-9%) are not well described. Methods: Clinical characteristics and OS of 1099 patients with MF who presented to MD Anderson Cancer Center between years 1984 – 2013 were retrospectively evaluated. Kaplan-Meier analysis and Cox regression models were used for calculations and comparisons. Results: PB and BM BL were available for 1038 (94%) patients at presentation. Five percent had AP, 10% had 5-9% BM or PB BL; 45% had < 5% BM BL and 1% (n=282), 2% (n=110), 3% (n=47) or 4% (n=27) PB BL; and 40% had < 5% BM BL and 0% PB BL (normal range). OS was similar among patients with 5-9% BM or 4-9% PB BL and those in AP (P>0.05). OS was also similar among patients with BL in the normal range and those with <5% BM and 1-3% PB BL (p>0.05). By grouping patients with similar OS, we have identified 2 groups with distinctive OS of 54 and 27 months, respectively (Table; p<0.001; HR = 1.81 [95%CI 1.5-2.2]). Five-year leukemia free survival was 65% for those in AP, 76% for ≥ 5-9% BM or ≥ 4-9% PB blasts, and 91% for < 4% BM or ≤ 3% PB BL (P<0.001). Clinical characteristics were similar for patients in the AP and with ≥ 5-9% BM or ≥ 4-9% PB blasts. Both groups had significantly higher leukocytes, lower hemoglobin and platelet levels, and were more likely to be transfusion dependent, have systemic symptoms, and unfavorable karyotype than patients with ≤ 4% BM or ≤ 3% PB blasts. There was no significant difference in age, splenomegaly, BM fibrosis grade, or distribution of JAK2, MPL and CALR mutations among these groups. Conclusions: Patients with ≥ 4% PB or ≥ 5% BM BL have OS that appears to be the same as those with >10% BL, suggesting that these patients may require a more aggressive treatment approach. [Table: see text]
Comparing adherence levels of CLL treatment from both the patient and physician perspective in the U.S.
Authors: Schenkel Brad , Hallworth Pam , Rider Alex , Macomson Brian , McRae Jacquelyn 
Published: September 2018

e19009 Background: Prior research has shown that there is a lack of congruency between physicians and patients regarding choice of therapy in CLL care. The aim of this study was to look at whether there are any similar differences in regard to perceptions of drug adherence between a group of CLL patients and their corresponding physicians. Methods: From February to May 2016, the Adelphi CLL Disease Specific Programme (DSP) recruited US physicians who are responsible for treatment decision-making in CLL. Patient Record Forms (PRF) and Patient Self-Completion questionnaires (PSC) were completed by physicians and patients, respectively. Means and standard deviations (SD) were calculated for continuous variables, and proportions were calculated for categorical variables. Results: A total of 81 physicians and 326 patients participated in the CLL DSP. The majority of physicians were heme-oncologists (68%), male (79%), and practiced in both a hospital and office-based setting (86%). Physicians reported that 87% of their CLL patients were “always” or “almost always” adherent to their CLL treatment. However, only 42% of patients reported that they were “never” or “hardly ever” non-adherent to CLL treatment. Patients reported that their physicians addressed adherence in the following methods: physicians asked patients about adherence (56%), nurses/other staff assisted patients (23%), physician did not check (15%), pill checking (3%), materials/resources were given (1%), and other (2%). Three-quarters (75%) of physicians reported that they discussed adherence with their CLL patients consistently. Conclusions: In this study, patients’ and physicians’ perceptions of adherence with regard to CLL treatment were not aligned. Although most physicians reported consistent discussions about adherence with their patients, the majority of patients reported subpar adherence to CLL treatment. As more oncology medications, including those approved in CLL, are available in a pill or capsule formulation and thus self-administered, it will be increasingly important to make patients aware of the importance of adhering to their medication and including physicians and caregivers in this dialogue.
A trial-based EuroQol EQ-5D health utility analysis in patients with classical Hodgkin lymphoma.
Authors: Wu Elise , Liao Jane , Balakumaran Arun 
Published: September 2018

e19011 Background: Pembrolizumab has shown a high response in patients classic Hodgkin lymphoma (cHL) patients who have experienced disease progression after brentuximab vedotin in KEYNOTE (KN)-087 and the results have been presented. This study aimed to evaluate the health-related quality of life (HRQoL) of the trial patients in KN087. Methods: KN-087 is an ongoing single-arm multi-center, non-randomized Phase II trial evaluating pembrolizumab 200mg Q3W IV in patients with relapsed or refractory cHL. In KN-087, HRQoL data were collected at baseline and every drug administration over the 18 months of follow-up. HRQoL was assessed using both the EQ-5D and EORTC QLQ-C30 instruments. The generic health statuses assessed from both instrument were converted to population-based utility values using published algorithms. More specifically, US-based scoring was applied to US patients, UK-based scoring for UK patients and EU-based scoring for all other patients. HRQoL was reported by status of respond and disease progression. Response was defined based upon IWG criteria. Furthermore, stratified analyses were conducted to examine the health disabilities of the patients who experienced grade 3+ adverse events (AEs), and by ECOG performance and the number of prior therapies. Results: Among 210 trial patients, HRQoL data were collected for 205 patients at baseline and the mean health utility score was 0.759 (95% CI 0.730-0.788). Mean health utility score among responders and non-responders was 0.826 (95% CI 0.811-0.842) and 0.760 (95% CI 0.718-0.801), respectively. The difference is considered clinically significant. Mean utility decreased from 0.820 (95% CI 0.807-0.833) for time spent prior to progression to 0.806 (95% CI 0.780-0.832) post disease progression. Progression-free patients who experienced grade 3+ AEs (N=17) had a mean health utility of 0.736 (95% CI 0.662-0.811), compared with 0.825 (95% CI 0.811-0.838) among those did not. Conclusions: The results showed a substantial HRQoL impact of R/R cHL. Treatment response was associated with significant clinically meaningful improvement in HRQoL. The utility estimates from the study are important for economic evaluations of treatments in R/R cHL patients. Clinical trial information: NCT02453594.
Ibrutinib associated infections: A retrospective study.
Authors: Chaul Barbosa Caroline , DeAngelis Lisa Marie , Grommes Christian 
Published: September 2018

e19020 Background: Ibrutinib (IBRU) is a Bruton tyrosine kinase (BTK) inhibitor that has been FDA approved for chronic lymphocytic leukemia (CLL), mantle cell lymphoma (MCL) and Waldenstrom's Macroglobulinemia (WM). BTK inhibition may contribute to immunosuppression through B- and T-cell inhibition, resulting in an increase in infections. We therefore characterized IBRU-related infections retrospectively. Methods: The study was an IRB approved retrospective review. Patients (pts) treated with IBRU between 4/2014-11/2016 who developed any infection were identified using ICD10 codes in a clinical database. Study population was defined using descriptive statistics. Results: 200 pts were identified: 78 pts had CLL (39%), 30 diffuse large B-cell lymphoma (15%), 28 MCL (14%), 19 WM (9.5%), 15 (7.5%) marginal zone lymphoma, 14 (7%) follicular lymphoma, 7 (3.5%) multiple myeloma, 7 (3.5%) T-cell lymphoma and 2 (1%) primary central nervous system lymphoma (PCNSL). Median age was 68 (range 28-96), 34% were men and median ECOG was 1. The majority of pts received IBRU as second line treatment (172, 86%) with a median of 2 prior lines of treatment; 23 pts (11.5%) were post transplant. Median IBRU dose was 420mg daily (range 140-840mg), administered a median of 316 days (range 3-1780). Single agent IBRU was used in 162 pts (81%). 105 pts (52%) developed an infection, with pneumonia (30%), upper airway infection (26%); skin infection (18%); and sinusitis (13%) being the most common. 10 (9.5%) had neutropenic fever and 1 (1%) cryptococcal pneumonia. Seven (7%) developed fungal infections, with invasive aspergillosis in 5 (5%). 34 (32%) developed ≥3 infections. 46 pts (44%) were hospitalized, 10 (9.5%) interrupted IBRU and 3 pts (2.9%) died due to infections (2 pneumonia; 1 neutropenic fever). The median time to infection after starting IBRU was 70 days (range 2-1261). The highest infection rate was seen in pts with PCNSL (100%, 2/2), MZL (67%, 10/14), followed by CLL (64%, 50/78). Conclusions: We identified infections in half of the pts treated with IBRU. Pts treated with IBRU should be monitored closely for the development of infections, particularly airway infections.
Clinicopathological characteristics compared between primary effusion lymphoma and human herpesvirus 8 (HHV8)-unrelated primary effusion lymphoma-like lymphoma.
Authors: Shin Junghoon , Koh Youngil 
Published: September 2018

e19024 Background: Primary effusion lymphoma (PEL) and human herpesvirus 8 (HHV8)-unrelated primary effusion lymphoma-like lymphoma (PEL-LL) are among the most rare subtypes of non-Hodgkin B-cell lymphoma. Although a few number of case reports and small case series have suggested a better outcome of PEL-LL compared to PEL, systematic comparison of prognosis between PEL and PEL-LL has never been conducted owing to their rare incidence. Methods: We consecutively collected PEL and PEL-LL patients diagnosed between 2000 and 2016 in 10 institutions in South Korea. All patients were cytogically confirmed and received systemic evaluation to exclude detectable tumor formation. Clinicopathological characteristics and prognosis were compared. Results: A total of 12 PEL and 4 PEL-LL cases were analyzed. Median age at diagnosis was 68.5 (range, 40-88) and 68.5 (range, 59-77) for PEL and PEL-LL, respectively. Male-to-female ratio was 7:5 and 3:1 in each group. Pleural effusion was the most common presentation in both groups. One patient in PEL-LL group presented with vitreous fluid containing lymphoma cells. Seven and two PEL patients were treated with CHOP and CVP-based chemotherapy, respectively and 3 did not receive chemotherapy due to poor performance. Three PEL-LL patients were treated with R-CHOP and the remaining 1 patient with malignant vitreous fluid received high dose methotrexate. One PEL patient received autologous hematopoietic stem cell transplantation after second remission and lived over 14 years thereafter. With estimated median follow-up duration of 99.6 and 20.7 months for PEL and PEL-LL respectively, 3 and 1 death occurred during the entire period in each group. Median overall survival was 119.6 and 66.5 months in PEL and PEL-LL group respectively without statistically significant difference. Conclusions: In our study, long-term prognosis of PEL and PEL-LL showed no difference. This result might be attributed to 2 remarkably long-lived patients in PEL group (longer than 174 and 123 months, respectively) and low statistical power owing to the small sample size.
Association of FDG PET-CT (PET) avid skeletal lesions (SL) with progression-free survival (PFS) in patients (pts) with previously untreated follicular lymphoma (FL).
Authors: Maly Joseph J. , Wei Lai , Hemminger Jessica , Christian Beth , Maddocks Kami J. , Jones Jeffrey Alan , Wright Chad , Blum Kristie A. 
Published: September 2018

e19026 Background: PET scan is frequently utilized in FL. Reduced EFS has been observed in DLBCL pts with SL treated with RCHOP (Held, JCO 31:4115, 2013). Methods: We performed a retrospective single center study to assess outcomes of FL pts with PET avid SL between January 2005 and November 2015. 131 pts with newly diagnosed FL and PET performed within 1 month of diagnosis were included. Results: 32 of these pts had SL (median 4, range 1-11) on initial PET. Median age was 57 (range 43-79), 15 (47%) were female, 30 (94%) had stage IV disease, LDH was elevated in 6 (19%), 6 (19%) had bulky disease > 6 cm, and FLIPI-1 score was low in 5, intermediate in 11, and high 16 pts. 27 pts had grade (gr) 1-2 FL, 2 had gr 3a, and 3 had gr 3 (not classified). All but 1 patient received rituximab (R)-containing therapy (9 received BR, 7 received RCHOP, 5 RCVP, 9 other). 8 pts received maintenance R, and none received radiation. There were no statistically significant differences in median age, tumor gr, LDH, or use of anthracycline containing therapy (28% in SL group vs 16% in non-SL group, p = 0.13) in pts with SL compared to those without SL (n = 99). Pts with SL had higher incidence of bone marrow involvement (27% vs 9%, p = 0.013). With a median follow-up of 35 months, SL pts had 44% rate of transformation to DLBCL compared 12% in non-SL pts (p = 0.004). Median PFS was 45.8 months in SL pts not-reached in non-SL pts (p = 0.003). Median OS was 105.9 months in SL pts and not reached in non-SL pts (p = 0.08). In the multi-variate analysis, SL (p = 0.037), male gender (p = 0.048), higher FLIPI-1 score (p = 0.009), and absence of anthracycline containing therapy (p = 0.005) were significantly associated with decreased PFS using backward selection. Conclusions: The presence of PET identified SL in previously untreated FL is associated with an increased risk of transformation and reduced PFS in this single center retrospective analysis. Larger studies of uniformly treated pts are needed to validate these data. The identification of high-risk PET avid SL in FL pts in future prospective therapeutic trials could be used to select pts for specific induction regimens, maintenance rituximab, or consolidative radiation.
Primary bone lymphoma (PBL) real world experience of a single cancer hospital. results, prognostic factors, treatment and long term follow up of 12 patients.
Authors: Stoumbos Dionisios , Gkekas Christos , Asmanis Evangelos , Christaki Evangelia Eleni , Dadakaridou Magdalini , Lampropoulou Polykseni , Megalakaki Aikaterini , Repousis Panagiotis , Kotsopoulou Maria 
Published: September 2018

e19033 Background: PBL is a very rare condition which constitutes less than 1% of all lymphomas. Due to the uncommon entity of this disease there is a lack of concordance in the characteristics, optimal management, treatment strategies and the associated outcome. Methods: Herein, we retrospectively review 12 consecutive patients initially diagnosed with PBL and also treated at our institution from 2001 to present. Results: The median age of the patients was 49 years old. Eight patients were female and 4 male. The histological type of all 12 patients presented was DLBCL. Ten patients (83%) were presented with Ann Arbor Stage I or II disease. While 2 patients (17%) had stage IV disease, none of them showed bone marrow involvement at their initial diagnosis. The median follow-up after achieving CR was 80 months. Our analysis demonstrated that age less than 47 years, lack of B symptoms, normal LDH level, Stage I or II and female gender were found to be favorable prognostic factors for achieving CR and OS. All 12 patients underwent chemotherapy with most of them (10; 83%) receiving 6 to 8 cycles of the RCHOP regimen. The majority (11; 92%) of them received chemotherapy followed by radiotherapy. After front line chemotherapy followed by radiotherapy most patients (9; 75%) achieved CR. Among the remaining three patients, the one patient died before completing first line chemotherapy due to an infection during chemotherapy-induced neutropenia, the other patient is undergoing autologous stem-cell transplantation due to refractory PBL and the third one achieved CR after he underwent high-dose chemotherapy and autologous stem-cell transplantation due to refractory PBL. Both patients that had refractory disease were male and initially diagnosed with stage IV PBL. The median OS was 100 months. Conclusions: The data from our study supports that most PBL patients have had early stage disease (IE-IIE) on diagnosis which also was the most important favorable prognostic factor. Overall, patients with primary lymphoma involving the bone with DLBCL have an excellent prognosis and only a limited number of patients underwent autologous stem-cell transplantation in order to achieve CR.
Age as a potential new prognostic indicator in primary cutaneous B-cell lymphoma (PCBCL).
Authors: Desai Poorvi Kirit , Van den Bergh Magali , Zhang Xiaohui , Zhang Hailing , Schaible Braydon , Ma Zhenjun , Sokol Lubomir 
Published: September 2018

e19034 Background: PCBCL is a group of rare lymphoproliferative disorders, with an estimated annual incidence of 2.5 per 1,000,000 persons. Indolent subtypes include Primary Cutaneous Marginal Zone Lymphoma (PCMZL) and Primary Cutaneous Follicular Center Lymphoma (PCFCL). Primary Cutaneous Diffuse Large B-cell Lymphoma (PCDLBCL) is an aggressive subtype with a fatality rate of 50%. The Cutaneous Lymphoma International Prognostic Index (CLIPI) can risk-stratify indolent subtypes, but age is not considered. Here we present our single-institutional analysis of clinicopathologic features and outcomes of patients with PCBCL. Methods: This is a retrospective study of patients evaluated at Moffitt Cancer Center between 01/1990 and 12/2016. Patients were identified using our PCBCL database and diagnosis was verified by independent hematopathologists and dermatopathologists. Staging was determined by ISCL/EORTC criteria. Demographics, subtype, stage, disease course, and CLIPI scores were collected. Continuous and categorical values were tested using Kruskal-Wallis ANOVA method and Fisher’s Exact Test, respectively. Kaplan-Meier curves were produced to determine PFS. Results: We identified 37 patients who met diagnostic criteria for PCBCL (35% PCFCL, 40.5% PCMZL, 13.5% PCDLBCL, and 11% indolent, unspecified). Male:female ratio was 2.4:1. 51% of patients were ≥ 60 years old (yo), and 49% were < 60 yo. 54% had stage T1 disease, 27% T2, and 19% T3. Median PFS for patients <60 was 1.1 years, but was not reached for those ≥60. Mean follow-up time was 2.6 years for all patients. Log-rank test showed a statistically significant difference in PFS between the two age groups (p=0.01). PFS for stage of indolent subtypes showed marginal significance (p <0.06). CLIPI for indolent subtypes did not show a significant difference in PFS. Conclusions: We found that age is a highly statistically significant prognostic parameter in PCBCL, as patients ≥ 60 yo had a longer PFS compared to younger patients, even after adjusting for stage and CLIPI. These results are promising for age as a possible prognostic indicator for PCBCL, but validation is needed with a larger sample size.