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Meandros medical and dental journal, 2021-04, Vol.22 (1), p.1-6
Ort / Verlag
Aydın: Adnan Menderes Üniversitesi
Erscheinungsjahr
2021
Quelle
Alma/SFX Local Collection
Beschreibungen/Notizen
Objective: Bleeding is one of the most important problems in humans. Absence,
deficiency or dysfunctions of protein factors in the coagulation system can
cause prolonged bleeding, morbidity or mortality. Although factor VIII, factor IX
and von Willebrand factor deficiencies are the most common, deficiencies in all
other factors exist, called rare factor deficiencies. This study aimed to present the
clinical presentations, laboratory findings, treatments, and surgical interventions in
patients with rare factor deficiencies other than factor VII followed up in our clinic.
Materials and Methods: Patients who were diagnosed with rare factor deficiency
other than factor VII in the department of pediatric hematology and oncology
between July 1997 and June 2020 were included in this study. Patients’ demographic
characteristics, clinical presentations, family history, prothrombin time, activated
partial thromboplastin time and factor levels, treatments and surgical interventions
were recorded retrospectively from patients’ files.
Results: Nineteen patients were included in the study, of which 7 (37%) had
factor X deficiency, 5 (25%) had factor XI, 3 (16%) had factor V+VIII, 2 (10%)
had factor V and 1 (5%) had factor I and XIII deficiencies. Parents of 12 patients
had consanguinity marriages. All patients with factor X deficiency had bleeding
episodes, and three of them were under prophylaxis with prothrombin complex
concentrate. Other patients were under on-demand treatment. In total, 19 surgical
interventions (11 minor; 8 major) were performed.
Conclusion: Rare bleeding disorders are very uncommon and heterogeneous, with
variable associations between coagulation factor activity and bleeding phenotype.
A multidisciplinary and expertise team (haematologists, nurses, gynaecologists,
obstetricians, orthopaedist, etc.) is necessary for the treatment and regular followup
of patients with rare bleeding disorders.