Haemophilia; Pattern of Clinical Presentation and Disease Severity

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Haemophilia; Pattern of Clinical Presentation and Disease Severity

Zahida Qasim,* Lubna Naseem,** Naghmi Asif, **** Khalid Hassan***
*Department of Pathology, Divisional Headquarter Teaching Hospital Mirpur, Azad Kashmir, Pakistan
** Department of Pathology, Pakistan Institute of Medical Sciences, Islamabad
*** Department of Pathology, Islamabad Medical and Dental College Islamabad

Abstract

Objective: To observe pattern of clinical presentation in patients of Hemophilia A and B.
Materials and Methods: Fifty one patients of Haemophilia A and B were evaluated on the basis of Patient`s demographic data, history (family, medication, past) and parameters like age, clinical features (pallor, number of joint involved, ankylosis and pain). Blood complete picture, Coagulation screening tests, bleeding time, PT, APTT, mixing studies and factor assays were documented.
Results: In total of fifty one patients, twenty two (43.13%) had severe degree of disease (<1% factor concentrate), while 19(37.2%) patients were diagnosed to have moderate degree of disease (factor concentrate 1-5%) and 10 (19.6%) had mild disease (factor concentrate 5-50%). Patients having severe disease were diagnosed earlier than those having moderate and mild disease, and had frequent episodes of bleeding in soft tissues, joints, muscles etc. Conclusion: Pattern of clinical presentation helps to categories the haemophilia patients as having mild, moderate or severe degree of disease Key words: Haemarthrosis, haemophilia A, haemophilia B, spontaneous bleeding, ankylosis.

         

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