RARE INHERITED CLOTTING FACTOR DEFECTS OF COMMON PATHWAY
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Keywords
Rare Bleeding Disorders, Common Bleeding Pathway, Factor V, Factor VIII
Abstract
Objective: To access the coagulation factor activity of common pathway in rare inherited bleeding disorder.
Study Design: Cross-Sectional
Duration: The study was carried out in 6 months from 01-09-2022 to 31-03-2023.
Methods: The Cross-Sectional study was carried out at the Dept. of Pathology (Hematology) and the Diagnostic & Research Laboratory – Liaquat University of Medical & Health Sciences, Jamshoro. 60 suspected cases of inherited clotting factor deficiencies involved in common pathway were taken, after taking informed written consent. All the diagnosed cases of acquired bleeding disorders, inherited clotting factors defect involved in extrinsic and intrinsic pathway and patient with Platelets functional disorders (Bernard soulier syndrome and Glanzman’s thrombasthenia) were excluded from the study. This study was conducted via nonprobability, convenient sampling and the data was analyzed via SPSS 21.0.
Results: Out of the total population of 60 patients with rare bleeding disorders, 55.2% (33) patients were male and 44.9% (27) were female. The median age at presentation to the tertiary care hospital was 9 years and 3 months, ranging from 4 months to 42 years. Among the rare blood disorders observed in a group of 60 patients, the distribution of cases varied across different conditions. Factor I (FI) accounted for 5 patients, making up approximately 8.33% of the total. Factor II (FII) exhibited a slightly higher prevalence, with 7 patients constituting around 11.67% of the sample. Factor V (FV) presented in 9 patients, representing approximately 15.00% of the total cases. Combined factor V and factor VIII (FVIII) showed the highest occurrence, with 12 patients making up around 20.00% of the sample. Factor VII (FVII) had a lower incidence, affecting 4 patients, or roughly 6.67% of the group. Factor X (FX) was observed in 6 patients, accounting for approximately 10.00% of the cases. Similarly, factor XI (FXI) presented in 8 patients, making up around 13.33% of the sample. Factor XIII (FXIII) had a relatively low prevalence, affecting 3 patients, or approximately 5.00% of the group. Lastly, the vitamin Kdependent clotting factors were identified in 6 patients, representing roughly 10.00% of the cases.
Conclusion: Among the rare blood disorders, combined factor V and factor VIII deficiencies were found to be most prevalent followed by deficiency of Factor V which is more prominent in male population.
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Irfan Ahmed
Resident M.phil Haematology, Pathology Department, Liaquat university of Medical & Health Sciences Jamshoro.
Kiran Aamir
Consultant Haematologist, Associate Professor, Pathology Department, Liaquat university of Medical & health sciences Jamshoro,
Farhan Ahmed Shaikh
Consultant Haematologist, Lecturer in Pathology Department, Bilawal medical college for boys, Liaquat university of Medical & health sciences Jamshoro.
Aasma Naz
Assistant Professor, Gynaecology and Obstetric Department,People university of Medical & health sciences for women Nawabshah
Kiran Memon
Assistant Professor, Pathology Department, Indus Medical College, Tando Muhammad Khan.
Sidrah
Lecturer,Pathology Department Bilawal medical college for boys Liaquat university of Medical & health Sciences Jamshoro
Aamir Ramzan
Lecturer,Pathology Department Liaquat university of Medical & health sciences Jamshoro.
Rameez Iqbal Memon
Lecturer,pathology Department, Liaquat university of Medical & health sciences Jamshoro