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