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Journal of Thrombosis and Haemostasis, 5: 604606

OFFICIAL COMMUNICATION OF THE SSC

The scoring system of the Scientific and Standardisation


Committee on Disseminated Intravascular Coagulation of the
International Society on Thrombosis and Haemostasis: a 5-year
overview1
C . H . T O H * and W . K . H O O T S O N B E H A L F O F T H E S S C O N D I S S E M I N A T E D I N T R A V A S C U L A R
COAGULATION OF THE ISTH
*The Roald Dahl Haemostasis & Thrombosis Centre, Royal Liverpool University Hospital, Liverpool, UK; and Gulf States Haemophilia Centre,
University of Texas Medical School, Houston, TX, USA

To cite this article: Toh CH, Hoots WK, on behalf of the SSC on Disseminated Intravascular Coagulation of the ISTH. The scoring system of the
Scientific and Standardisation Committee on Disseminated Intravascular Coagulation of the International Society on Thrombosis and Haemostasis:
a 5-year overview. J Thromb Haemost 2007; 5: 6046.

Introduction
In 2001, the International Society on Thrombosis and
Haemostasis (ISTH) Sub-Committee of the Scientic and
Standardisation Committee (SSC) on Disseminated Intravascular Coagulation (DIC) proposed that the working denition
of DIC be delineated into two phases. Non-overt DIC would
represent subtle hemostatic dysfunction while overt DIC
recognized its decompensated phase [1]. Central to the diagnosis is a scoring system rooted in rapid and readily available
tests. This would then enable the diagnosis to be utilized widely
and serve as the reference standard for diagnostic and
therapeutic purposes.
For overt DIC, a cumulative score of 5 or more from
prolonged prothrombin time (PT), reduced platelets and
brinogen, and elevated brin-related markers was proposed
(Table 1). This required prospective conrmation. For nonovert DIC, scoring would incorporate abnormal trends and
results in both simple global tests and molecular markers of
coagulation (Table 2). Studies were required to establish
feasibility and to derive a prognostic score.
Methods
We examined all articles citing the 2001 communication.
Searching on MEDLINE and EMBASE (December 2001 to
Correspondence: Cheng Hock Toh, Roald Dahl Haemostasis &
Thrombosis Centre, Royal Liverpool University Hospital, Prescot
Street, Liverpool L7 8XP, UK
Tel.:
+44-151-706-4322;
fax:
+44-151-706-5810;
e-mail:
toh@liv.ac.uk
1

Full paper available at http://www.med.unc.edu

June 2006) also included the terms DIC and diagnosis,


ISTH, overt, non-overt plus related links. Selection was
based on use of the scoring systems in DIC diagnosis and
prognosis.
Results and discussion
In the absence of a reference gold standard for the ISTH
overt DIC score, comparisons with the Japanese Ministry of
Health and Welfare (JMHW) score were important as the
best-evidenced working diagnosis to date. Studies in the
1990s closely correlated increasing JMHW scores with
increasing mortality [2]. In 1284 patients, Wada et al. found
the rate of agreement between both ISTH and JMHW
scores at 67.4% [3]. Discordance was primarily in the
JMHW sensitivity to DIC in hematologic malignancies with
high brinolytic activity. Without such patients, a further
comparative study showed 93% concordance [4]. Although
these were mainly in patients with infection, Gando et al.
found mortality to be also high in the DIC of non-infected
patients diagnosed using ISTH criteria [5]. Using a different
approach with blinded expert assessments, Bakhtiari et al.
found sensitivity of 91% and specicity of 97% with the
ISTH DIC score [6]. Increasing scores strongly correlated
with mortality. Others have similarly conrmed this independent prediction of mortality by the ISTH DIC score and
its added prognostic value to the Acute Physiology and
Chronic Health Evaluation (APACHE) II system [79].
In relation to therapeutic relevance, retrospective analyses
from two large treatment trials in sepsis [10,11] utilizing data
that did not include brinogen levels also highlighted the
prognostic power of the ISTH DIC score. In the PROWESS
study, the DIC score separated those with favorable responses
to recombinant activated protein C from those with less
2006 International Society on Thrombosis and Haemostasis

ISTH DIC score 605


Table 1 Scoring system for overt Disseminated Intravascular Coagulation
(DIC)
1. Risk assessment: does the patient have an underlying disorder known to
be associated with overt DIC?
If yes: Proceed.
If no: Do not use this algorithm.
2. Order global coagulation tests (platelet count, prothrombin time,
fibrinogen, fibrin-related marker).
3. Score global coagulation test results.
Platelet count
(>100 = 0; <100 = 1; < 50 = 2)
Elevated fibrin related marker (e.g. D-dimers; fibrin degradation products)
(no increase = 0; moderate increase = 2; strong increase = 3)
Prolonged prothrombin time
(< 3 s = 0; > 3 but < 6 s = 1; > 6 s = 2)
Fibrinogen level
(>1.0g L1 = 0; < 1.0g L1 = 1)
5. Calculate score
If 5: compatible with overt DIC: repeat score daily
If < 5: suggestive (not affirmative) for non-overt DIC: repeat next 12 days.

Table 2 Scoring system for non-overt Disseminated Intravascular Coagulation (DIC)


1.

2.

Risk assessment: does the patient have an underlying disorder known to


be associated with DIC?
yes = 2, no = 0
Major criteria
9 1

>100x10 l = 0
Platelet
Count
<3 s = 0
PT
Prolongation
Normal
=0
Fibrin
related-markers

3.

4.

<100x109 l1 = 1

Rising = 1 Stable = 0 Falling = 1

>3 s = 1

Falling = 1 Stable = 0 Rising = 1

Raised = 1

Falling = 1 Stable = 0 Rising = 1

estimations with ongoing work to standardize reagents, precise


cutoff recommendations remain to be dened.
For non-overt DIC, Toh and Downey demonstrated that
the proposed template was workable [13]. In addition, a score
of 5 or greater could diagnostically dene patients with a poor
prognosis from haemostatic dysfunction, independent of
developing overt DIC. Although the scoring system allowed
for PC and AT measurements, the combination of scoring
abnormal trends and results in the PT, platelet count, and
D-dimer provided sufcient robustness without recourse to the
molecular markers. Indeed, studies of simplied scoring
variations that include longitudinal observations show promise. An evolving score based on PT and platelet count in the
rst 48 h of intensive care reected clinical severity [14].
Worsening coagulopathy during the rst day of severe sepsis
was also associated with increased development of new organ
failure [15]. Notably, it was PT and D-dimer changes but not
PC or AT that were signicantly related to mortality when
analyzed as continuous variables.
Recommendation
A score of 5 or greater can identify overt DIC by the system
proposed in the 2001 communication of the ISTH SSC on
DIC. Using a different template whereby abnormal trends are
scored alongside abnormal results, non-overt DIC can be
diagnosed from a score of 5 or greater. There appears to be no
added value in including PC or AT estimations. Further
prospective validation and similar approaches of capturing
evolving coagulopathy through readily available assays are
recommended.

Specific criteria
Antithrombin

Normal = 1

Protein C

Normal = 1

Low = 1

------------

Normal = 1

Abnormal = 1

Low = 1

Calculate score:

impressive effects [10]. Comparable results were shared by the


KyberSept cohort of patients treated with antithrombin
without concomitant heparin [11].
Studies have also addressed specic differences in reporting
laboratory results. In laboratories where the PT in seconds is
not reported, the prothrombin index has been used with 0 for
>70%, 1 for 40%70% and 2 for <40% [9]. Studies have also
attempted to dene D-dimer cutoff levels. One approach
assigned scores for moderate and strong increases on 25% and
75% quartiles derived from over 1000 samples in intensive care
[12]. Dempe et al. [12] found this to be approximately
1 lg mL)1 and approximately 3 lg mL)1, respectively, with
good concordance between two D-dimer assays. In a Dutch
intensive care cohort, 0.44 lg mL)1 and > 4lg mL)1 Ddimer was used for moderate and strong increases, respectively
[6]. As issues remain on the accuracy of high D-dimer
2006 International Society on Thrombosis and Haemostasis

Disclosure of Conflict of Interests


The authors state that they have no conict of interest.
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2006 International Society on Thrombosis and Haemostasis

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