A comparison between MRI, sonography and Functional Independence Score in Haemophilia methods in diagnosis, evaluation and classification of arthropathy in severe haemophilia A and B.

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. In this cross-sectional study on 25 severe haemophilia patients, FISH, MRI and sonography procedures were performed in the elbow or knee joint. 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 and analysed. Among the 25 patients (age range of 11-70 years), 22 patients were haemophilia A and three patients were haemophilia B. Affected joints were right knee in 12 patients, left knee in nine and right elbow in four. There was only a statistically significant negative correlation between FISH and MRI Additive (A) scale (rs = -0.537, P = 0.006). 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. On the basis of our results, FISH scores in severe haemophilia patients were negatively correlated with MRI A scale. Also, it seems that a FISH score less than 22 could be considered as loss of cartilage; however, due to the small number of our patients, it needs further assessment in different populations.

Modern management of haemophilic arthropathy.

Currently available factor concentrates for treatment of patients with haemophilia are virally inactivated or are made by recombinant technology and their broad use in developed nations has resulted in the dramatic elimination of the treatment-related viral illnesses that decimated the haemophilia community in the late 20th century. The major morbidity experienced by patients with haemophilia today is joint disease, a result of repeated bleeding episodes into joint spaces. Although administration of factor concentrates to prevent bleeding has been demonstrated to prevent haemophilic joint disease when applied assiduously, repeated bleeding episodes induce synovitis that is irreversible and may progress despite subsequent prophylaxis. Surgical and nuclear medicine interventions are available to reduce the pain of haemophilic arthropathy and to reduce further bleeding episodes. Patients with high titre inhibitors are at great risk for the development of joint disease and present the greatest therapeutic challenges when joint surgery is needed.

Intraarticular dexamethasone in advanced chronic synovitis in hemophilia.

From 1988 to 1966, 34 patients with advanced chronic hemophilic synovitis (25 Grade III and nine Grade IV) were treated with intraarticular injections of long acting dexamethasone (sodium phosphate of dexamethasone plus acetate of dexamethasone) in cycles of three injections with 3-week intervals between each injection with 6-month rest intervals between cycles for as many as three cycles, depending on the evolution of each case. All patients had chronic severe synovitis, axial deformity, muscular atrophy, and diminution of range of movement. There were 31 knees, two ankles, and two shoulders. Subjective and objective evaluations were done grouping the results in good, fair, and poor according to grade of patient satisfaction, presence of synovitis and pain, range of movement, and limitation of activities of daily living. In the subjective results there were 19 good results, 12 fair results, and four poor results, and in the objective evaluation there were 22 good results, nine fair results, and four poor results at an average followup of 1.5 years. The use of intraarticular dexamethasone as an alternative in the short to medium term for treatment of advanced chronic hemophilic synovitis with pain and limitation of function before doing an invasive surgical treatment is proposed.

Hemophilic arthropathy of the upper limb.

Little has been written about the effects of hemophilic arthropathy on the function and radiography of the upper limb. Therefore, 14 moderate and 19 severe hemophiliacs between the ages of 10 and 57 years were examined clinically and radiographically. The extent of the arthropathic changes in the shoulders, elbows, wrists, and hands were classified according to the system proposed by the World Federation of Hemophilia. The severity of the arthropathy increased with the age of the patient and with the number of hemarthroses recorded, although the correlation between these parameters was variable. The elbow joints were the site of recognizable arthropathy in 87% of the cases, with a slight preponderance for the nondominant side. The glenohumeral and wrist joints were affected in a small proportion of patients, although symptoms were rarely experienced. Hemorrhages affecting the hands were uncommon and produced arthropathy infrequently.

[On the classification of haemophilic arthropathy (author's transl)]. / Zur Klassifizierung der hämophilen Arthropathie.

In order to differentiate between the manifold phenomena of haemophilic arthropathy, and to treat them suitably, the author suggests a classification of haemophilic arthropathies. Qualitatively speaking, it is possible to differentiate between 6 forms of bleeders-joint: the virginal, restituted, acute, florid, latent and activated form. Quantitative subdivision is effected according to two criteria: the extent of actual destructive activity of the joint being one criterion of classification, the other one being the extent of permanent damage to the joint.