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Magnitude of arthropathy in patients with hemophilia: A single‑center experience

Rawand Polus Shamoon

Iraq Joural of Hematology المجلة العراقية لامراض الدم
ISSN: 20728069/25432702 Year: 2017 Volume: 6 Issue: 2 Pages: 78-83
Publisher: Al-Mustansyriah University الجامعة المستنصرية

Abstract

BACKGROUND: Hemophilia is a sex‑linked bleeding disorder. Affected patients suffer spontaneousor post-traumatic bleeding into various sites of the body, mainly into joints, depending on the levelof coagulation factor deficiency.AIMS: This descriptive study is designed to assess the prevalence and extent of arthropathy andevaluate the functional status of hemophiliacs in Erbil, Northern Iraq.SETTINGS AND DESIGN: A descriptive study of all registered hemophiliacs at NanakaliHemato‑Oncology Teaching Centre, Erbil, Iraq.PATIENTS AND METHODS: Over 15 months (October 2015 to February 2017), a total of 133hemophilia patients were studied. Their hospital records were used to retrieve clinical and laboratorydata, mainly their coagulation profile. All patients were clinically examined at the daycare center;plain radiography was used to evaluate the degree of joint damage based on Petterson score. Themagnitude of joint disease was assessed, and patients’ functional status was evaluated dependingon Functional Independence Score in Hemophilia (FISH).STATISTICAL ANALYSIS USED: Statistical analysis used MS Excel 2010.RESULTS: Hemophiliacs mean age was 12.9 years. Patients with severe hemophilia presented earlierand had more bleeding episodes. At least one bout of hemarthrosis was recorded in 103/133 patientsduring the course of their disease with knee joint most frequently involved (in eighty patients) followedby elbow, ankle, wrist, and shoulder. The Petterson score related significantly to age of the patient,number of bleeds, and severity of hemophilia. Majority, 67%, of hemophiliacs found to have thelimitation of movement. FISH score significantly related with factor activity level. Functional disabilitywas encountered in 9.7% of cases; majority had severe hemophilia.CONCLUSIONS: The incidence and severity of joint bleeding and functional disability were high.The FISH and Pettersson scoring systems are very useful tools in assessing patients with hemophilicarthropathy.

Keywords

Arthropathy --- Erbil --- hemarthrosis --- hemophilia --- Iraq --- limitation of movement