J Korean Med Sci.  2010 Dec;25(12):1742-1747. 10.3346/jkms.2010.25.12.1742.

A Long Term Results of External Beam Radiation Therapy in Hemophilic Arthropathy of the Ankle in Children

• Kong M
• Kang JO
• Choi J
• Park SH

• KMID: 1792897
• DOI: http://doi.org/10.3346/jkms.2010.25.12.1742

Abstract

Bleeding into joint space is critical to develop hemophilic arthropathy. To reduce the frequency of bleeding in the ankle joint of children with hemophilic arthropathy, low dose external beam irradiation was performed for 37 patients. Among them, 35 patients followed-up for longer than 1 yr (median 87 months) were enrolled for analysis. The average number of bleedings per month was 3.6 during one year prior to radiation therapy. After radiation therapy, it was decreased to 2.1 during the first year, after then it was maintained in the range of 1.0 to 1.5 until the tenth year. The bleeding frequency was reduced to 42% at the first year and it was maintained in the range of 58% to 73% from the second to the tenth year. Especially the patients who had 3 or more bleedings per month, and who had MRI score more than 3 showed significant decreases. During the follow-up period, growth disturbances and secondary malignancies were not found. External beam radiotherapy can be considered for the hemophilic patients with surgical or isotope therapies are not amenable.

Keyword

Hemophilia; Arthropathy; Radiotherapy

MeSH Terms

Adolescent
*Ankle Joint/radiography
Child
Child, Preschool
Hemarthrosis/etiology/*radiotherapy
Hemophilia A/*complications
Humans
Male
Prognosis

Figure

• 

  Fig. 1 Image of treated ankle joint. (A) The simulation film for the radiation therapy. (B) Coronal T1-weighted magnetic resonance image showing prominent areas of hemosiderin, hyperplastic synovium adjacent to the ankle joint, some erosion, and small amount of effusion to give the score 6.

• 

  Fig. 2 The average number of bleedings for each year. Before radiation therapy, the number of bleeding was 3.6 per month. After radiation therapy, the average number of bleedings was decreased to 2.1 for the first year, and then the number was maintained in the range of 1.0 to 1.5 until the tenth year.

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  Fig. 3 The average number of bleeding for each year in patient group with pre-EBRT bleeding frequency 3 or more and pre-EBRT bleeding frequency less than 3. After radiation therapy, the average number of bleeding was maintained in the range of 1.3 to 2.7 in patient group with pre-EBRT bleeding frequency 3 or more, 0.6 to 1.5 in patient group with pre-EBRT bleeding frequency less than 3, respectively.

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  Fig. 4 The joints of group with pre-EBRT bleeding frequency 3 or more per month showed statistically significant decreased bleeding frequency (P=0.018). The patient was defined to have sustained bleeding when the bleeding frequency did not show statistically significant improvement in relation with pretreatment bleeding frequency by T-test.

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  Fig. 5 The percent of patients having critical target joint was in the range of 0% to 11.7% from the first to the tenth year in patient group with pre-EBRT bleeding frequency less than 3 per month. In patient group with pre-EBRT bleeding frequency 3 or more, the range was 0% to 33.3%. Patients with bleeding frequency less than 3 per month were well maintained without critical target joints from 5 yr after radiation therapy.

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  Fig. 6 Joints of the group with Arnold-Hilgatner's stage IV show statistically significant decreased bleeding frequency (P=0.004).

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  Fig. 7 Joints of the group with MRI score more than 3 show statistically significant decreased bleeding frequency (P=0.012).

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