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Indian Journal of Public Health Research & Development
Year : 2015, Volume : 6, Issue : 4
First page : ( 210) Last page : ( 214)
Print ISSN : 0976-0245. Online ISSN : 0976-5506.
Article DOI : 10.5958/0976-5506.2015.00229.6

A Clinico-Hematological Study of Congenital Bleeding Disorders

Nikethan B1,*, Chaitanya V2, Hanagavadi Suresh3, Hiremath S S4

1Assistant Professor, Department of Pathology, JJM Medical College, Davangere, Karnataka, India

2Assistant Professor, Department of Otorhinolaryngology, JJM Medical College, Davangere, Karnataka, India

3Professor, Department of Pathology, JJM Medical College, Davangere, Karnataka, India

4Professor and Head, Department of Pathology, JJM Medical College, Davangere, Karnataka, India

*Corresponding author: Dr. Nikethan B Assistant Professor, Department of Pathology, JJM Medical College, Davangere-577004 Karnataka, India. Mobile: + 919141654056, E-mail: nikethanbathi@gmail.com

Online published on 1 October, 2015.

Abstract

Context

Congenital bleeding disorders are abnormalities of hemostasis that may present with significant difficulties in diagnosis and management.

Aims

This study was done to know various clinical presentations of congenital bleeding disorders and also to note the hematological changes associated with them.

Materials and methods

This was a hospital based study conducted at haematology unit, Department of Pathology, JJM Medical College, Davangere. The present study includes 110 cases referred to our department with history of bleeding disease were evaluated clinically and investigated appropriately to detect the underlying cause for the abnormal hemostatic function.

Results

This clinico-hematological study of congenital bleeding disorders showed Glanzmann's thrombasthenia in 4 cases (3.64%). The remaining 106 cases are had diseases affecting clotting factor which included Hemophilia A-70 cases; Hemophilia B-19 cases; Factor VII deficiency-1 case; afibrinogenemia-1 case; Factor XIII deficiency-3 cases; vWD-11 cases and dysfibrinogenemia-1 case. Vascular disorders were not encountered. Majority of these cases presented with hemarthrosis and muscle hematoma. Plasma clotting tests (APTT) were prolonged in majority of cases, corrected with aged serum, adsorbed plasma, F VIII deficient plasma, F IX deficient plasma. Factor assay showed mild, moderate and severe forms.

Conclusion

In spite of various advanced diagnostic investigations, the basic hematological investigation remains first panel or step towards the approach to the diagnosis of congenital bleeding disorders.

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Keywords

Blood coagulation disorders, Inherited, factor VIII, hemophilia A.

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