Abstract

INTRODUCTION:

The majority of bleeds experienced by people with Haemophilia occur into the joints or muscles.The joints most frequently involved are the knees, ankles, and elbows. The consequences of recurrent bleeding into a joint leads to chronic hemophilic arthropathy. Knee arthropathy is crippling situation affecting Quality of Life (QOL) for Patient with Hemophilia (PWH).With the advent of the availability of Clotting Factor Concentrates (CFC), Total Knee Replacement (TKR) can now be performed safely. TKR is performed using Total 2000IU/kg of Plasma Derived (PD) or Recombinant CFC. Literature recommends 1000-1200IU/kg of Total Eloctate for TKR.

METHODS:

Aim for feasible, safe & cost effective CFC replacement policy for TKR

1) Use lower dose Eloctate during TKR

2) Perform Simultaneous Bilateral TKR

Patients and Methods:

Total 24 Severe Hemophilia A patients underwent 48 bilateral, simultaneous TKR. Age range was 28-51 years. Pre-operative 50IU/Kg Eloctate was infused. Pre-operative FVIIIc Level was median 108IU/kg (86-126 IU/dl). Post-operative Eloctate replacement was 40-50IU/kg once daily till day 5, then 30IU/kg once daily till Day15. FVIIIc assay was monitored at least once daily till day 10. Dose was tailored according to FVIIIc levels. Trough FVIIIc level was maintained above 60iu/dl for first 5 days. Average duration of surgery was 4 hours. Drains were removed after 48 hours. LMWH Thromboprophylaxis & physiotherapy was started from post-operative Day 2. Clips were removed on Day 14. Eloctate was received through Humanitarian Aid Program of WFH.

RESULTS:

Total of 48 bilateral, simultaneous TKR were performed on 24 patients. 23 patients had no significant intra or post-operative bleeding & no transfusion support was needed . One patient underwent pseudo tumour excision and TKR in the same sitting & required extra 50IU/kg Eloctate+ 8 packed red cells + 8 FFP + 10 Cryoprecipitate intra operative during excision of Pseudo Tumour for total blood loss of 2200 ml as expected . He also experienced no post-operative bleeding till discharge. No wound or prosthesis infection was noted. Prosthesis survival was 100%. Total Eloctate used was 510- 640 IU/kg over 14 days.

DISCUSSION:

There was no excess bleeding in our patients. The results from our assessment are quite similar to the overall prosthetic survival rates reported for TKAs performed on patients without hemophilia.In our study there were no periprosthetic infections reported. Other studies have showed rates of periprosthetic infection after primary TKA in patients with hemophilia are much higher (up to 11%). Late infection is a major concern after TKA in patients with hemophilia, an important factor is the high rate of HIV-positive patients (3).Another important complication of TKA surgery is aseptic loosening of the prosthesis. Aseptic loosening was not found in any of our patients. Rates of this complication increase over follow-up (4). In our data the ROM improved and flexion contracture reduced significantly after arthroplasty following the scheduled physiotherapy.As mentioned in an article by Schild FJA et al the Median amount of CFC (Plasma derived & recombinant) used for multi-joint procedure per patient was 57,680 IU; in another retrospective study by A Thes et al where the median CFC (Plasma derived & recombinant) usage per patient was 1,500 IU/kg; whereas we used Total Eloctate of 510- 640 IU/kg and hence reduced CFC usage compared to western protocol.

CONCLUSIONS :

  • Low dose Eloctate replacement is safe and cost effective for TKA in PWH.

  • Bilateral TKA in same sitting is feasible and increases cost effectiveness in PWH.

  • Thrombo-prophylaxis with LMWH can be done safely in PWH.

  • Bleeding events did not increase with low dose Eloctate & LMWH.

  • At these doses, total Eloctate replacement was 40-50% lower than previously reported for TKR surgery for PWH. Our protocol offers a cost effective and safe option in resource constraint countries.

Disclosures

No relevant conflicts of interest to declare.

Author notes

*

Asterisk with author names denotes non-ASH members.