phosphorus-radioisotopes and Hemophilia-B

Radionuclide synovectomy has been identified as the procedure of choice in treating chronic haemophilic synovitis among Caucasian populations. Its effectiveness among East Asians has not been studied. A retrospective study was carried out on 12 Asian haemophiliacs who underwent 12 radionuclide synovectomies. The average follow-up was 30.7 months (range 6-55) for primary procedures. 32P chromic phosphate and 188Re-tin colloid were injected into target joints according to protocol. There was a significant 80% decrease in the median frequency of haemarthrosis from 1.4 per month (range 0.2-7.0) to 0.25 per month (range 0.0-1.8) (P<0.05). Half of the patients had excellent results by 1 year of synovectomy. The median factor usage for target joint haemarthrosis postsynovectomy was 792 units per month (range 0-3209) reduced significantly from a presynovectomy level of 1452 units per month (range 306-7125) (P<0.05). Patients also reported a reduction in joint pain scores, and an improvement in joint mobility and quality of life. The majority of patients were satisfied with the overall outcome of radionuclide synovectomy. Radionuclide synovectomy appears to be effective in reducing the incidence of target joint haemarthrosis and quantity of factor usage for such bleeds among Asians with haemophilic synovitis.

Topics: Adolescent; Adult; Chronic Disease; Follow-Up Studies; Hemarthrosis; Hemophilia A; Hemophilia B; Humans; Male; Middle Aged; Pain Measurement; Patient Satisfaction; Phosphorus Radioisotopes; Quality of Life; Radioisotopes; Retrospective Studies; Rhenium; Severity of Illness Index; Singapore; Synovial Membrane; Synovitis; Treatment Outcome

We hypothesized that magnetic resonance imaging (MRI) scans taken prior to radiosynoviorthesis may be predictive of response to the procedure in persons with haemophilia. Specifically, response would be inversely related to the severity of synovial hyperplasia. Radiosynoviorthesis was administered to 21 joints with recurrent haemorrhage (target joints). A detailed self-report of haemorrhage history, joint evaluation with scoring according to the World Federation of Haemophilia orthopaedic joint and pain scales, plain radiographs, and MRI studies of the joints were performed pre- and post-radiosynoviorthesis. To augment comparison of the MRI findings to those assessed using the Arnold-Hilgartner and Pettersson scales, a provisional MRI scale for evaluation of haemophilic arthropathy was designed. We found the MRI findings prior to the procedure were not predictive of clinical response; independent of the severity of synovial hyperplasia, most joints bled less and showed improvement by the WFH orthopaedic score. There was generally no change in the severity of synovial hyperplasia after the procedure. We conclude that MRI evaluation is not routinely indicated prior to radiosynoviorthesis.

Topics: Adolescent; Adult; Ankle; Child; Elbow; Follow-Up Studies; Hemarthrosis; Hemophilia A; Hemophilia B; Humans; Hyperplasia; Knee; Magnetic Resonance Imaging; Phosphorus Radioisotopes; Radiography; Severity of Illness Index; Synovial Membrane

Isotopic synovectomy is being proposed as an option in the treatment of patients with haemophilic arthropathy. We present our experience with 11 paediatric patients who underwent 17, P-32 isotopic synovectomies for chronic haemophilic arthropathy. P-32 was injected into the joint per protocol, approved by the institutional review board. All our patients were male. Nine were factor VIII and two were factor IX deficient. The following joints were treated: ankle (n=10 procedures), elbow (n=5) and knee (n=2). The first procedure was performed on December 1993. None were human immunodeficiency virus positive. Mean age at the first procedure was 10.8 years (range, 5.2-15.2 years). Mean pretreatment joint clinical scores using the World Federation of Hemophilia guidelines for the ankle was 5.5 (SD +/- 2.3), the elbow 4.2 (+/-2.5), and knee 5.5 (+/-3.5); the corresponding post-treatment scores were 2.6 (+/-2.0), 1.4 (+/-0.5) and 2.5 (+/-3.5) respectively. Presynovectomy mean radiological scores using the Pettersson method were: ankle 1.8, elbow 1.8, and knee 1.5. A scoring system used in our centre for evaluating joints using magnetic resonance imaging (MRI) gave the following mean pretreatment scores: ankle 9.5, elbow 8.4, and knee 5.0. A marked decrease (an 80-100% decrease) in bleeding was seen in 13 of 17 procedures, and a moderate decrease (51-79% decrease) in two procedures, accounting for 85% reduction in bleeding into the target joints. The procedure was well tolerated and no untoward side-effects were noted as of May 1999, with a median follow-up of 40 months (range 19-65 months). None had any clinical evidence of cancer. Three patients had their joints retreated [elbow (one), ankle (two)]. These procedures were also well tolerated. In conclusion, in our study, isotopic synovectomy using P-32 appears to be feasible, safe and efficacious in the treatment of haemophilic arthropathy in paediatric patients who have been followed for a median of 40 months. As previously shown, MRI appears to give more detailed information about joint arthropathy than plain radiographs.

Topics: Adolescent; Child; Child, Preschool; Evaluation Studies as Topic; Hemarthrosis; Hemophilia A; Hemophilia B; Humans; Injections, Intra-Articular; Magnetic Resonance Imaging; Male; Phosphorus Radioisotopes; Radiography; Synovectomy; Synovial Membrane; Treatment Outcome

Topics: Amino Acid Sequence; Antisense Elements (Genetics); Base Sequence; Blood Coagulation; Blotting, Southern; Codon; DNA; DNA Primers; Electrophoresis, Agar Gel; Exons; Factor IX; Hemophilia B; Humans; Indicators and Reagents; Introns; Molecular Sequence Data; Phosphorus Radioisotopes; Point Mutation; Polymerase Chain Reaction; Radioimmunoassay; Restriction Mapping; Sulfur Radioisotopes