A Comparison Between MRI, Sonography and FISH Methods in Diagnosis, Evaluation and Classification of Arthropathy in Severe Haemophilia a and B

Abstract

Background: Haemophilia A and haemophilia B are the most common severe inherited bleeding disorders. Chronic arthropathy is a major long-term disabling complication of haemophilia. Imaging and evaluation of joints in children with haemophilia is important in detecting abnormalities, staging their severity and following the effects of treatment. The aim of this study is to evaluate the correlation of FISH score (Functional Independence Score in Haemophilia) with the scores obtained by MRI and sonography for the diagnosis, evaluation and classification of arthropathy in severe Haemophilia A and B.Methods: This is a cross-sectional study which was conducted in a Hospital affiliated to Shiraz University of Medical Sciences in Shiraz from April 2013 to April 2014. Twenty five male patients with severe hemophilia above 10 years old were participated. FISH, MRI and sonography procedures were performed in the elbow or knee joint of the patients. All patients’ information including age, type of Haemophilia, affected joint, scores of MRI, sonography and FISH, dose of factor consumed, weight and prophylaxis protocol were collected. Correlation between scores of FISH, MRI and sonography was calculated by Spearman Correlation test. P-value of less than 0.05 was considered significant.Results: The patient’s age ranged between 11 to 70 years-old. Twenty-two patients were Haemophilia A(88%) and 3 patients were Haemophilia B (12%). Among the 25 patients 12(48%) affected joints were right knee, 9(36%) left knee and 4(16%) right elbow.The FISH score ranged between 16 and 28, with mean score of 20.8 ± 3.2. Only two of the 25 patients were rated as fully independent in all seven activities (maximum possible score of 28). Although 24 patients could walk, six required aids in the form of a cane or a brace. Seventeen patients could not squat (score 1) and three patients could not climb steps. It was determined that 18 patients (72%) did not have any problem in self-care management (eating, bathing and dressing), and the remaining were able to perform them with slight discomfort.In this study MRI P scale ranged between 9 and 10, MRI A scale ranged between 5 and 20 and the sonography scores (maximum 11 means cartilage loss) ranged between 4 and 11.Correlation between FISH scores, MRI (A and P scales) and sonography was assessed. There was only a statistically significant negative correlation between FISH and MRI Additive (A) scale (rs=-0.537, P value=0.006).Also we categorized the FISH scores of patients in different stages of MRI. Considering cartilage loss domain (related MRI A scale: 13-20), 20 patients (80%) were classified in this group with FISH scores ranged from 17 to 22.Conclusion: FISH appears to be a promising disease-specific instrument for assessing overall musculoskeletal function in haemophilia patients. Based on our results, FISH scores in severe hemophilia patients were negatively correlated with MRI A scale. Also, it seems that FISH score less than 22 could be considered as loss of cartilage; however, due to small number of our patients it needs further assessment in different populations. DisclosuresNo relevant conflicts of interest to declare.