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Challenges in the Diagnosis and Management of Non-Severe Hemophilia.

Authors :
Boeriu E
Arghirescu TS
Serban M
Patrascu JM
Boia E
Jinca C
Schramm W
Traila A
Ursu CE
Source :
Journal of clinical medicine [J Clin Med] 2022 Jun 09; Vol. 11 (12). Date of Electronic Publication: 2022 Jun 09.
Publication Year :
2022

Abstract

(1) Background: Mild and moderate hemophilia, synonymous with non-severe hemophilia (NSH), are of constant interest for the clinicians. Bleeding occurs usually after trauma, injury, surgery, or inhibitor development, sometimes leading to a shift of the clinical phenotype from mild to severe, even with life-threatening and unexpected outcomes. (2) Methods: We performed a retrospective observational study conducted on 112 persons with congenital coagulopathies, 26 of them with NSH, admitted to our clinic in the period 2000 to 2022. For the diagnosis, we used laboratory studies (complete blood cell count, coagulation assays, biochemistry, thromboelastography, genetic tests) and imaging investigations (X-ray, ultrasound, CT, MRI). We selected four cases confronted with pitfalls of diagnosis and evolution in order to illustrate the sometimes provocative field of NSH. (3) Results: Confronted with challenging cases with under-, missed or delayed diagnosis and severe consequences, we aimed at presenting four such selected cases with mild or moderate hemophilia, real pitfalls in our clinical activity. (4) Conclusions: In the field of NSH, if not timely recognized, tending sometimes to remain ignored by caregivers and patients themselves, we can be confronted with challenging diagnostic situations and life-threatening bleeds.

Details

Language :
English
ISSN :
2077-0383
Volume :
11
Issue :
12
Database :
MEDLINE
Journal :
Journal of clinical medicine
Publication Type :
Academic Journal
Accession number :
35743393
Full Text :
https://doi.org/10.3390/jcm11123322