ASH Clinical News ACN_5.3_web | Page 34

On Location 2018 ASH Annual Meeting Next is my favorite #ASH18 Twitter theme: #SHEmatology and #WOMANcology. In addition to celebrating accomplishments, these pointed out the often-subtle forms of discrimination against women in medicine, such as the tendency at some conferences to refer to male presenters as “Dr. So-and-so” and female presenters by their first names (e.g., “Jennifer,” or worse, “Jenny” – especially if not even your mom calls you “Jenny”). In this cul- ture and in these public meetings, respect is conveyed with titles. Until and unless we all get on a first- name basis (which I admit I would love), this particular intimation of inequality is unacceptable. I also heard attendees judging female researchers more harshly for the style of their presentations, with comments like, “The research was great, but way she talked ...” or “Really nice work, but did you see what she was wearing?” While I wouldn’t argue for a moratorium on discussing style, whether of speech or clothing or PowerPoint slides, for persons of any gender, I was frustrated by the vitriol that was directed, as far as I heard, solely toward women. Every human, and every human organization, deals with some ver- sion of issues of bias, discrimina- tion, nepotism, conflicts of interest, inequity, and iniquity. In all of this, we can do better. And, judging by the very existence of Dr. Tshilolo’s report on hydroxyurea and the Twit- ter hashtags, I think we are. But we have a long way to go. (Do you see how quickly I have been assimilat- ed, and now speak in the inclusive “we”?) The End? I called this reflection on my first ASH annual meeting a post-vivum instead of a post-mortem because this meeting is a living organism, with homeostasis and dynamism. It is evolving and adapting, tissues and cells, groups and individuals. Many are in S phase, some are se- nescent, others apoptotic. (I did not appreciate any necrosis, but I do not deny that it may have been pres- ent.) As with anything that is real, it is gorgeous and disturbing, profane and celestial. I’m grateful to be a part of it. See you in Florida. C. Beau Hilton is a fourth-year medical student at Cleveland Clinic Lerner College of Medicine. 32 ASH Clinical News Attendees in between sessions at the 2018 ASH Annual Meeting. Predicting Thrombosis and Mortality in Patients With Sickle Cell Disease Two studies presented at the 2018 ASH Annual Meeting character- ized the risk for complications, and offered models for predicting these risks, in patients with sickle cell disease (SCD). SCD and Thrombotic Risk First, Andrew Srisuwananukorn, MD, from the University of Illinois (UI) at Chicago, presented findings from an analysis of nearly 1,200 patient records, characterizing the high thrombotic risk among patients with SCD and identifying several laboratory, clinical, and genetic factors that increase this risk. In the study, data were collected from the electronic medi- cal records of 1,193 patients diagnosed with SCD at UI Health between January 2008 and December 2017. During a median follow up of 5.6 years (interquartile range = 2.3-9.3 years), 210 patients (17.6%) experienced arterial (n=75) or venous thromboembolic events (VTEs; n=268). The majority of events were deep vein thromboses (DVTs; n=170), and the authors determined that most of the events were catheter-related. After adjusting for age, sex, and hydroxyurea use, the only factors that were significantly associated with any thrombotic event were: • HbSS/Sβ0 thalassemia (odds ratio [OR] = 2.07; p=0.002) • higher hospitalization rate (OR=1.07; p<0.001) • central venous catheter placement (OR=1.22; p<0.001) • baseline creatinine at the initial outpatient visit (OR=1.94; p=0.004) The authors also focused on VTE, finding similar risk factors, with the addition of: • number of pregnancies (OR=1.39; p<0.001) • increased systolic blood pressure at the initial outpatient visit (OR=4.91; p=0.04) Using data from 329 patients for whom genotyping was available, the investigators identified only two genetic risk variants associ- ated with thrombotic events, both in the thrombomodulin protein (OR=1.5; p<0.05 for both). In the cohort of 174 patients who experienced VTE, 31 (18%) experienced a recurrent VTE in the 10-year time span of this study. “Not surprisingly, most of these patients were started on warfarin (for a duration of 3-6 months), but we were not able to find an association with either type or duration of anticoagulation and risk of recurrent VTE,” Dr. Srisuwananukorn reported. Although the study involved a large cohort of patients with SCD with a broad age range, the authors noted that the find- ings are limited by the study’s single-center, retrospective design. Compliance to anticoagulation and thrombotic events occurring outside of the hospital also were difficult to capture, which may have confounded the results. Dr. Srisuwananukorn noted that researchers are now looking into the risk factors associated with recurrent hospitalizations and other thrombotic complications in patients with SCD. SCD and Pulmonary Hypertension Next, in an analysis of long-term data from a cohort study of adults with SCD, researchers confirmed that pulmonary hypertension (PH) is associated with a poor prognosis, identifying a “hyper- hemolytic phenotype” of SCD associated with a higher risk of death. The findings were presented by Pablo Bartolucci, MD, PhD, from Henri Mondor Hospital in Creteil, France. The prospective ETENDARD (Evaluation of the Prevalence of Pulmonary Hypertension in Adult Patients With Sickle Cell Disease) study evaluated the prevalence of PH in 398 outpatients with SCD enrolled between February 2007 and March 2009 at referral centers in France. All patients were homozygous for hemoglobin S or had Sβ0 thalassemia. In an earlier analysis, researchers estimated the prevalence of PH related to SCD to be 6 percent, and the risk for develop- ing PH was greater in patients who had a tricuspid regurgitation velocity (TRV) ≥2.5 m/s and a mean pulmonary arterial pressure (mPAP) ≥25 mmHg (as measured by right-sided heart catheter- ization; RHC). To determine the long-term prognostic value of TRV ≥2.5 m/s and the level of mPAP, the authors analyzed 10-year follow- up data from the ETENDARD cohort. Per study protocol, participants underwent Doppler echocardiography to determine TRV; those with suspected PH (defined as TRV ≥2.5 m/s) then underwent RHC to determine mPAP and confirm PH diagnosis (defined as mPAP ≥25 mmHg). A total of 289 patients had no suspected PH (group A); among the remaining 109 patients, 98 underwent RHC. Of these, 74 had suspected PH (TRV ≥2.5 m/s and mPAP <25 mmHg; group B) and 24 had confirmed PH (TRV ≥2.5 m/s and mPAP ≥25 mmHg; group C). Dr. Bartolucci noted that the rate of con- firmed PH was 6 percent, which was consistent with the earlier analysis. During a median follow-up of 8.8 years (range not reported), 25 patients were lost to follow-up. Thirty-nine of the remaining February 2019