Clinical Investigation of Orthopedic Outcome in Patients with Severe Hemophilia Under Prophylactic Treatment. Disadvantage of a late Prophylactic Treatment?

Clinical Investigation of Orthopedic Outcome in Patients with Severe Hemophilia Under Prophylactic Treatment.

Disadvantage of a late Prophylactic Treatment?

S. Meister, T. Spranger, K. Jersch and G. Auerswald

Introduction

One essential aim in the hemophilia treatment is to prevent hemorrhaging into joints by prophylactic treatment with factor concentrates. Hemophilic arthropathy is a possible consequence of recurrent joint bleeds. Efficacy of early prophylactic treatment has been shown within the last years.

A physical examination and a joint status was done of all regularly treated pati- ents in our treatment centre born 1985–1996 (40 patients) with a FVIII/IX residual activity of <2%. Excluded was one patient with former cranial hemorrhage and spastic hemiplegia. All patients were on prophylactic FVIII or IX treatment at the time of investigation.

Patients

Two groups were formed: the first group with patients born 1985-1990 (17 patients, average age 15.1 years), and the second group with patients born 1991–1996 (23 patients, average age 9.3 years). Prophylaxis was started at the average age of 6.1 years (median 4.9 years) in the first group. Prophylactic treatment was started earlier at the average age of 4.1 years (median 3.6 years) in the second group.

Anamnestic data

Most bleeds were reported in the ankle joints (33 patients, 82.5%). Other locations were the knees (25 patients, 62.5%) and elbows (20 patients, 50%). Bleeds were less

Group 1 Group 2

Year of birth 1985–1990 1991–1996

Number of patients 17 23

Hemophilia A/B patients 14/3 21/2

Average age 15.1 9.3

Median age 15 9.3

Start of prophylactic treatment (average) 6.1 4.1

Start of prophylactic treatment (median) 4.9 3.6

I. Scharrer/W. Schramm (Ed.)

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Hemophilia Symposium Hamburg 2003

” Springer Medizin Verlag Heidelberg 2005

often reported in hip, hand and feet. This distribution was found in both groups;

patients of the second group were affected less often (Fig. 1).

Physical Examination

3 patients had synovitis in the ankle joint, in two other patients synovitis was found in the left knee. Crepitations were found in 13 patients, mostly in the knees (10 pati- ents, 25%), in 6 patients even both knees were affected. Also crepitations were found in elbow and ankle.

10 patients of the first group (59%) and 8 patients of the second group (35%) had limited range of motion. The decreased range of movement was in most pa- tients mild. The ankle joint was affected most often (Fig. 2) (47% group 1, 35%

group 2) followed by the elbow (29% group 1, 9% group 2). Discreet ankle joint dis- orders occurred especially more often in group 2 (Fig. 3). Surprisingly we found no decreased range of motion in the knees. A total of 5 patients (29%) in the first group and in contrast only one patient (4%) in the second group had more than one joint with limited range of motion (Fig. 4).

Clinical Investigation of Orthopedic Outcome in Patients with Severe Hemophilia 197

0 20 40 60 80 100

ankle knee elbow hip other

N u mb e r o f p a ti e n ts in % Group 1 Group 2

0 10 20 30 40 50 60 70

ankle knee elbow hip total

N u m b e r of pa ti e n ts i n %

Group 1 Group 2 Fig. 1.

Locations of bleeds

Fig. 2. Joints

with limited

ranges of

motion

198 S. Meister et al.

Summary

This clinical investigation supports former data regarding the efficacy of early pro- phylactic treatment (Kreuz et al., Aledort et al.). It becomes most clear looking at the number of joint disorders in each group. Decreased ranges of movements in several joints were also found clearly more often in the first group. Possible causes are either the prophylactic treatment (which was started later in the first group) or the patient’s age at the time of investigation. Patients who were older than 8 years when prophy- lactic treatment was started often had limitations in the range of movement in sever- al joints. Further investigation in patients born 1997-2002 is planned. Also a follow up of the two groups will be done with a functional motion study (a three dimensional ultrasound study was done in some patients already). The prophylactic treatment with factor concentrates was in most cases started after occurrence of the first joint hemorrhage; in the rest of the cases it was started after the second joint bleeding.

References

1. Kreuz et al.: When should prophylactic treatment in patients with haemophilia A and B start? The German experience. Haemophilia. 1998 Jul;4(4):413-7.

2. Aledort et al.: A longitudinal study of orthopedic outcomes for severe factor VIII-deficient hemophiliacs. The orthopedic outcome study group. J Intern Med 1994; 236(4): 391-9

0 5 10 15 20 25 30 35 40 45

Group 1 Group 2

N u mb e r o f p a ti e n ts in %

discreet manifest

0%

20%

40%

60%

80%

100%

Group 1 Group 2

N u mb e r o f p a ti e n ts in %

none 1 joint 2 joints >2 joints

Fig. 3. Limited ranges of motion of the ankle joints

Fig. 4. Joints with

limited ranges of

motion in each

patient