Catherine Lambert

The role of physiotherapy after total knee arthroplasty in patients with haemophilia.

Summary.  With the availability of clotting factor concentrates, advances in surgical techniques, better implant design, and improvements in postoperative management, total knee arthroplasty has become the treatment of choice for haemophilia patients suffering from end‐stage haemophilic knee arthropathy. The success of this surgery is also dependent on close collaborations among the orthopaedic surgeon, the haematologist and the physiotherapist. Although haemophilic patients undergoing this surgery would likely benefit from a targeted rehabilitation programme, its specificities, modalities and limitations have thus far not been extensively studied. Employing the published data of rehabilitation after knee prosthesis in patients with osteoarthritis and haemophilic arthropathy along with clinical experience, the authors present a comprehensive and original review of the role of physiotherapy for patients with haemophilia undergoing knee arthroplasty.

Subclinical deep venous thrombosis observed in 10% of hemophilic patients undergoing major orthopedic surgery

To cite this article: Hermans C, Hammer F, Lobet S, Lambert C. Subclinical deep venous thrombosis observed in 10% of hemophilic patients undergoing major orthopedic surgery. J Thromb Haemost 2010; 8: 1138–40. Deep venous thrombosis (DVT) is a common postoperative complication in patients undergoing major orthopedic surgery of the lower limbs, such as total hip replacement (THR), total knee replacement (TKR) or hip fracture surgery (HFS). In the absence of thromboprophylaxis, subclinical venous thrombosis rates as high as 60% have been reported when using systematic bilateral phlebography after orthopedic surgery. As a result, routine pharmacological thromboprophylaxis with low-molecular-weight heparin (LMWH) or an alternative antithrombotic agent is strongly recommended in patients undergoing these procedures [1]. With the availability of efficient and safe clotting factor concentrates, THR, TKR as well as ankle arthrodesis are frequently performed in subjects with hemophilia suffering from chronic hemophilic arthropathy [2]. Yet, pharmacological prophylaxis of venous thromboembolism (VTE) in this patient group remains controversial. With the exception of retrospective case reports and small series, the incidence of VTE disease in hemophilic patients after major orthopedic surgery is still unclear. Despite the concern that pharmacological thromboprophylaxis might increase bleeding complications in these patients, no properly sized study has objectively evaluated the need, appropriate timing, dosage and duration of low-molecular weight heparin (LMWH) prophylaxis in this

Optimal management of hemophilic arthropathy and hematomas.

Hemophilia is a hematological disorder characterized by a partial or complete deficiency of clotting factor VIII or IX. Its bleeding complications primarily affect the musculoskeletal system. Hemarthrosis is a major hemophilia-related complication, responsible for a particularly debilitating chronic arthropathy, in the long term. In addition to clotting factor concentrates, usually prescribed by the hematologist, managing acute hemarthrosis and chronic arthropathy requires a close collaboration between the orthopedic surgeon and physiotherapist. This collaboration, comprising a coagulation and musculoskeletal specialist, is key to effectively preventing hemarthrosis, managing acute joint bleeding episodes, assessing joint function, and actively treating chronic arthropathy. This paper reviews, from a practical point of view, the pathophysiology, clinical manifestations, and treatment of hemarthrosis and chronic hemophilia-induced arthropathy for hematologists, orthopedic surgeons, and physiotherapists.

Percutaneous transcatheter closure of interatrial septal defect in adults: Procedural outcome and long-term results†

Background: Percutaneous transcatheter closure of patent foramen ovale (PFO) and atrial septal defect (ASD) has been shown to be feasible. Aim: The aim of this study was to evaluate the safety and efficacy of transcatheter interatrial septal shunt closure with prosthesis implantation in adults patients during long‐term follow‐up. In addition, the impact of thrombophilia and pulmonary hypertension on the outcome were investigated. Methods: Between June 1999 and November 2009, 287 patients (112 males, 43 ± 14 years) were treated in our institution by transcatheter closure of PFO (N = 175) or ASD (N = 112). Clinical and echocardiographic follow‐up were prospectively performed at 1, 6 and 12 months followed by a 1 once a year evaluation. Results: All procedures were successful with eight procedural complications (2.7%): one stroke, two femoral pseudoaneurysms, three transient atrial fibrillation, two minors pericardial effusions. Among patients with presumed paradoxical embolism, thrombophilia was observed in 29 patients (17%); only one of them experienced a recurrent stroke. Among patients with ASD, pulmonary hypertension was observed in 32 cases (28%) and significantly reduced 6 months after shunt closure (from 47 ± 7 to 31 ± 11 mm Hg, P < 0.0001). 99% of patients achieved a complete follow‐up. Clinical improvement was observed in 93%. Freedom from death, cardiac surgery or recurrent embolism was 98 ± 1% at 5 years. Conclusion: Percutaneous transcatheter interatrial septal defect closure is a safe and effective treatment in adults patients, even in case of thrombophilia or pulmonary hypertension, during a long‐term follow‐up, up to 11 years.

Rivaroxaban for arterial thrombosis related to heparin-induced thrombocytopenia

Heparin-induced thrombocytopenia (HIT) may be a critical condition in intensive care patients. Diagnosis of HIT is often difficult, and management too, as physicians have usually a limited experience with alternative anticoagulants. A 36-year-old man was admitted for orthopaedic surgery after a trauma causing a fracture of the sacrum and right ankle. Anticoagulant prophylaxis was made by nadroparin (3800  IU/day). But the patient developed less than 10 days after nadroparin exposure a significant drop in platelet count. The diagnosis of HIT was based on the pretest clinical score and demonstration of platelet factor 4 and heparin antibodies. Fondaparinux was transiently administered but was replaced 3 days later by rivaroxaban (15  mg twice a day during 21 days then 20  mg/day), after the demonstration of an acute thrombosis of the left radial artery. Platelet count returned to normal range and a partial recanalization of arterial thrombosis was noted. The use of rivaroxaban in this indication is of theoretical interest but requires further experience.

Plasma extraction rate and collection efficiency during therapeutic plasma exchange with Spectra Optia in comparison with Haemonetics MCS

For therapeutic plasma exchange (TPE), continuous and intermittent flow separators are known to be efficient. This study was undertaken to compare the performances of the Spectra Optia, a continuous flow centrifugal apheresis system recently developed by CaridianBCT, with the Haemonetics Multicomponents System (MCS)+ apheresis system based on intermittent flow centrifugation. The primary objective of the study was to compare the time required to exchange one total plasma volume with both separators. The secondary objectives were to determine the plasma exchange efficiency, the plasma extraction rate, the percentage of target exchange volume achieved, and the loss of cellular components. The study involved prospectively paired comparison of 16 TPE on each device performed in patients with chronic diseases treated with TPE. The time required to exchange 1 total plasma volume was 182 ± 36 minutes for MCS+ procedures and 100 ± 20 minutes for the Spectra Optia procedures (P < 0.05, all results presented as mean ± standard deviation). A significantly higher plasma extraction rate was achieved (30.2 ± 4.3 vs 16.8 ± 3.4 mL/min, respectively, P < 0.05), and the plasma exchange efficiency was slightly better with the Spectra Optia compared with the MCS+ procedures (83.4 ± 7.0 vs 80.0 ± 8.5%, P < 0.05). The platelet loss was significantly lower with the Spectra Optia compared with the MCS+ procedures (1.6 ± 2.3 vs 7.5 ± 4.2%, respectively, P < 0.05), whereas the red blood cells loss was comparable. In conclusion, the Spectra Optia has significantly higher extraction rate and exchange efficiency than the MCS+ allowing to remove the same amount of plasma in less time, by processing less blood. It also removes significantly less platelets than the MCS+ separator. J. Clin. Apheresis, 2011.

Deep vein thrombosis induced by thalidomide to control epistaxis secondary to hereditary haemorrhagic telangiectasia.

Thalidomide was recently reported to reduce the severity and frequency of epistaxes in patients with hereditary haemorrhagic telangiectasia (HHT). We here describe the case of a patient with HHT and severe epistaxes refractory to medical and local surgical treatments who developed an extensive deep vein thrombosis shortly after initiation of treatment with thalidomide. This is the first report of venous thromboembolic complication induced by thalidomide prescribed in this setting. Although thalidomide was recently found to provide an alternative therapeutic strategy in patients with HHT and refractory epistaxes, this agent should be used with great caution in this indication, given its thrombogenicity and difficulties to manage systemic anticoagulation in patients with HHT.

Intron 22 homologous regions are implicated in exons 1–22 duplications of the F8 gene

The intron 22 inversion found in up to 50% of severe hemophilia A patients results from a recombination between three intron 22 homologous copies (int22h). This study evaluated the implication of these copies in the formation of extended duplications comprising exons 1–22 of the factor 8 (F8) gene and their association with hemophilia and mental retardation. Two hemophilic patients with moderate and severe phenotypes and a third nonhemophilic patient with developmental delay were studied. All exhibited a duplication of F8 gene exons 1–22 identified by multiplex ligation-dependent probe amplification along with abnormal patterns on Southern blotting and unexpected long-range PCR amplification. Breakpoint analysis using array comparative genomic hybridization was performed to delimit the extent of these rearrangements. These duplications were bounded on one side by the F8 intragenic int22h-1 repeat and on the other side by extragenic int22h-2 or int22h-3 copies. However, the simultaneous identification of a second duplication containing F8 gene exons 2–14 for the moderate patient and the classical intron 22 inversion for the severe patient are considered in this study as the genetic causal defects of hemophilia. This study shows that the well-known int22h copies are involved in extended duplications comprising F8 gene exons 1–22. These specific duplications are probably not responsible for hemophilia and intellectual disability, but should be carefully considered in genetic counseling, while continuing to investigate the causal mutation of hemophilia.

Dabigatran etexilate (Pradaxa®) for preventing warfarin-induced skin necrosis in a patient with severe protein C deficiency

Dabigatran etexilate (Pradaxa®) for preventing warfarin-induced skin necrosis in a patient with severe protein C deficiency -

Does the site of platelet sequestration predict the response to splenectomy in adult patients with immune thrombocytopenic purpura

Abstract Splenectomy is the only potentially curative treatment for chronic immune thrombocytopenic purpura (ITP) in adults. However, one-third of the patients relapse without predictive factors identified. We evaluate the predictive value of the site of platelet sequestration on the response to splenectomy in patients with ITP. Eighty-two consecutive patients with ITP treated by splenectomy between 1992 and 2013 were retrospectively reviewed. Platelet sequestration site was studied by 111Indium-oxinate-labeled platelets in 93% of patients. Response to splenectomy was defined at last follow-up as: complete response (CR) for platelet count (PC) ≥100 × 109/L, response (R) for PC≥30 × 109/L and <100 × 109/L with absence of bleeding, no response (NR) for PC<30 × 103/L or significant bleeding. Laparoscopic splenectomy was performed in 81 patients (conversion rate of 16%), and open approach in one patient. Median follow-up was 57 months (range, 1–235). Platelet sequestration study was performed in 93% of patients: 50 patients (61%) exhibited splenic sequestration, 9 (11%) hepatic sequestration and 14 patients (17%) mixed sequestration. CR was obtained in 72% of patients, R in 25% and NR in 4% (two with splenic sequestration, one with hepatic sequestration). Preoperative PC, age at diagnosis, hepatic sequestration and male gender were significant for predicting CR in univariate analysis, but only age (HR = 1.025 by one-year increase, 95% CI [1.004–1.047], p = 0.020) and pre-operative PC (HR = 0.112 for > 100 versus <=100, 95% CI [0.025–0.493], p = 0.004) were significant predictors of recurrence-free survival in multivariate analysis. Response to splenectomy was independent of the site of platelet sequestration in patients with ITP. Pre-operative platelet sequestration study in these patients cannot be recommended.