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Application of the ISTH-BAT hemorrhagic score in constitutional bleeding

disorders

Conference Paper · January 2022

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Moueden Amine Mohamed El Horri

University of Oran Algiers University 1
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ABSTRACTS
PO169 ACQUIRED HAEMOPHILIA A ASSOCIATED TO
POSTPARTUM PERIOD: A CASE REPORT OF A DELAYED
DIAGNOSIS
R. Jurado Tapiador1,* ; M. F. Martínez García2 ; O. Benitez Hidalgo2
1 Hematology Department, Institut Català d’Oncologia-Hospital Universi-
tari Germans Trias i Pujol, Badalona; 2 Hemophilia Unit, Hematology Depart-
ment, Vall dt’Hebron University Hospital, Barcelona, Spain
Introduction: Pregnancy associated acquired haemophilia A (AHA) is
a rare entity. Most cases appear between the second to fourth month
after the first pregnancy with subcutaneous, mucosal and muscu-
loskeletal hematomas.
Methods: We report a case of a woman diagnosed with AHA in the
postpartum period, who presented bleeding symptoms short after her
second delivery but had a delayed diagnosis.
Results: The patient was a 27-year-old woman with an obstetric history
of two vaginal deliveries. The first went with no complications. After
the second one, in April 2020, she had heavier postpartum bleeding
that lasted up to three weeks.
Two months after the delivery she presented with heavy menstrual
bleeding, spontaneous hematomas, and an episode of haemorrhage
after a dental extraction which lasted two weeks. The patient was
referred to our centre 10 months after the delivery because of per-
sistent bleeding symptoms. The physical exam revealed a left painful
palmar muscular hematoma. The blood work showed activated partial
thromboplastin time (APTT) ratio of 2.47 and FVIII of 2.4%. Given the
suspicion of AHA, the study was completed by dosing the FVIII inhibitor
titer which was higher than 16BU. As extension study, an autoimmunity
and tumor panel was ordered, coming back negative.
Due to the presence of a muscular hematoma which could compro-
mise the upper limb, haemostatic treatment was started with 3 doses
of activated recombinant FVII (rFVIIa) 100mg/Kg every 4h followed by
every 8h. Immunosuppressive treatment was started with 3 bolus of
methylprednisolone 5mg/Kg/day followed by prednisone 1mg/Kg/day
and tacrolimus 5mg every 12h.
Complete remission was achieved 5 months after the diagnosis and
both immunosuppressors were discontinued after 7 months of treat-
ment.
Discussion/Conclusion: The rarity of pregnancy associated AHA leads
to delays in diagnosis. Our patient consulted several times during
7 months due bleeding symptoms until the diagnosis was made.
Because of the type of patient (young mothers who are usually
breastfeeding and could have the desire of other future pregnan-
cies) the type of immunosuppressive treatment must be chosen care-
fully. Prednisone in monotherapy or with Rituximab are the most
used treatments in this type of cases. Because our patient was
not breastfeeding at the time of the diagnosis, the treatment cho-
sen was corticosteroids and anticalcineurinics following our local
protocol.
Disclosure of Interest: None declared
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113
PO170 APPLICATION OF THE ISTH-BAT HEMORRHAGIC
SCORE IN CONSTITUTIONAL BLEEDING DISORDERS
R. Messaoudi1,* ; A. Moueden1 ; M. Elhorri2
1 Faculty of medecine of Oran, CHU Oran; 2 Faculty of medecine of Oran,
HMRO, Oran, Algeria
Introduction: Clinical assessment of the bleeding phenotype and the
severity of bleeding symptoms is a fundamental step in the evaluation
of patients presenting with possible hemostasis disorders. In order to
improve the collection and reproducibility of bleeding history, several
bleeding assessment tools (BAT) have been proposed and used. Does
the application of ISTH BAT score for primary hemostasis and coagula-
tion disorders allow a prognostic classification between these different
bleeding diseases?
Methods: A total of 170 patients were included, the population of
our study consists of patients from different wilayas of Western Alge-
ria who were already followed at the level of the hematology depart-
ment of the University Hospital of Oran (CHU ORAN); it is a retro-
spective and prognostic study of the constitutional bleeding diseases:
Hemophilia A and B whatever the degree of severity (severe, moderate,
minor), rare coagulation deficiencies (RBD) (FII, VII, V+VIII deficien-
cies), Von Willebrand disease whatever its type (type 1, type 2b, type
2N), constitutional thrombopathies of the Glanzmann thrombasthe-
nia (TG) type; Jean Bernad Soulier disease (JBS); May-Hagglin throm-
bopathy (MH) regardless of age and sex. We established and calculated
the ISTH-BAT (International Society of Thrombosis and Haemostasis -
Bleeding Assesment Tools) score for each patient based on the history
of bleeding events of these patients.
Results: The application of the ISTH-BAT score to our series classi-
fies severe hemophilia A, moderate hemophilia A, severe hemophilia
B, Glanzmann thrombasthenia and Jean Bernard Soulier disease in the
forms with a severe prognosis according to the ISTH-BAT score with
a score equal to or greater than 10. Von willebrand disease, minor
hemophilia A and May Hegglin thrombopathy are classified as moder-
ate prognostic forms according to the ISTH-BAT score.
Discussion/Conclusion: Despite the heterogeneity of clinical bleeding
manifestations in hemophilia, von willebrand disease, constitutional
thrombopathies and rare coagulation deficiencies, the ISTH-BAT has
allowed the quantification and the unification of the semiology of the
bleeding syndrome, thus allowing a prognostic classification. The ISTH-
BAT score is also used in the screening of these rare hemorrhagic dis-
eases
Disclosure of Interest: None declared
PO171 CLINICAL PHENOTYPE IN PATIENTS WITH
HEREDITARY FACTOR XII DEFICIENCY
E. Yakovleva1,* ; E. Demidova2 ; V. Surin2 ; V. Salomashkina2 ; D.
Selivanova2 ; L. Gorgidze3 ; N. Zozulya1