The value of HEAD-US system in detecting subclinical abnormalities in joints of patients with hemophilia

ABSTRACT Background: Prevention of hemarthrosis is the key factor in the adequate management of people with hemophilia (PWH). If hemarthrosis occurs, early diagnosis of joint damage is essential to make personalized treatments. This study is aimed at gaining an understanding of the ability of point-of-care ultrasound (US) using the `Hemophilia Early Arthropathy Detection with Ultrasound´ (HEAD-US) protocol to detect abnormalities in joints without history of hemarthrosis and clinically asymptomatic joints of PWH. Methods: The sample included 976 joints from 167 PWH (mean age 24.86 years). Data were collected from routine practice over a 3-year period and analyzed based on history of hemarthrosis and results of clinical (HJHS 2.1) and HEAD-US examinations. Results: In our series, 14% of patients exhibited HEAD-US signs of incipient arthropathy in joints with no history of bleeding and with a HJHS 2.1 score of 0. The most severely involved joint was the right ankle. Synovitis, articular cartilage and subchondral bone damage scores in joints with subclinical findings were slower than in joints with previous hemarthroses or HJHS 2.1 > 1 Conclusions: Our study demonstrates that HEAD-US is better than hemarthrosis records and the HJHS 2.1 scale in detecting the early signs of joint damage in PWH.

[1]  V. Blanchette,et al.  A systematic review of ultrasound imaging as a tool for evaluating haemophilic arthropathy in children and adults , 2017, Haemophilia : the official journal of the World Federation of Hemophilia.

[2]  M. Pisters,et al.  Comparing findings of routine Haemophilia Joint Health Score and Haemophlia Early Arthropathy Detection with UltraSound assessments in adults with haemophilia , 2017, Haemophilia : the official journal of the World Federation of Hemophilia.

[3]  T. Humphries,et al.  Point-of-care ultrasonography (POCUS) in hemophilia A: a commentary on current status and its potential role for improving prophylaxis management in severe hemophilia A , 2017, Therapeutic advances in hematology.

[4]  P. Babyn,et al.  Choosing outcome assessment tools in haemophilia care and research: a multidisciplinary perspective , 2017, Haemophilia : the official journal of the World Federation of Hemophilia.

[5]  V. Jiménez‐Yuste,et al.  New findings on inhibitor development: from registries to clinical studies , 2017, Haemophilia : the official journal of the World Federation of Hemophilia.

[6]  J. Oldenburg,et al.  European retrospective study of real‐life haemophilia treatment , 2017, Haemophilia : the official journal of the World Federation of Hemophilia.

[7]  V. Blanchette,et al.  Correlating clinical and radiological assessment of joints in haemophilia: results of a cross sectional study , 2016, Haemophilia : the official journal of the World Federation of Hemophilia.

[8]  J. Oldenburg,et al.  Potential biomarkers of haemophilic arthropathy: correlations with compatible additive magnetic resonance imaging scores , 2016, Haemophilia : the official journal of the World Federation of Hemophilia.

[9]  M. Makris,et al.  Safety surveillance in haemophilia and allied disorders , 2016, Journal of internal medicine.

[10]  D. DiMichele Hemophilia Therapy--Navigating Speed Bumps on the Innovation Highway. , 2016, The New England journal of medicine.

[11]  P. Ambrosino,et al.  Assessment of Hemophilic Arthropathy by Ultrasound: Where Do We Stand? , 2016, Seminars in Thrombosis & Hemostasis.

[12]  C. Tinelli,et al.  Interobserver reliability of ultrasound assessment of haemophilic arthropathy: radiologist vs. non‐radiologist , 2016, Haemophilia : the official journal of the World Federation of Hemophilia.

[13]  C. Altisent,et al.  Early prophylaxis in children with severe haemophilia A: clinical and ultrasound imaging outcomes , 2016, Haemophilia : the official journal of the World Federation of Hemophilia.

[14]  A. Tagliafico,et al.  Point‐of‐care ultrasound in haemophilic arthropathy: will the HEAD‐US system supplement or replace physical examination? , 2016, Haemophilia : the official journal of the World Federation of Hemophilia.

[15]  I. C. Schaaf,et al.  Value of routine ultrasound in detecting early joint changes in children with haemophilia using the ‘Haemophilia Early Arthropathy Detection with UltraSound’ protocol , 2016, Haemophilia : the official journal of the World Federation of Hemophilia.

[16]  B. Bartels,et al.  Using routine Haemophilia Joint Health Score for international comparisons of haemophilia outcome: standardization is needed , 2016, Haemophilia : the official journal of the World Federation of Hemophilia.

[17]  A. Doria,et al.  Imaging Evaluation of Hemophilia: Musculoskeletal Approach , 2015, Seminars in Thrombosis & Hemostasis.

[18]  P. Moerloose,et al.  Improving haemophilia patient care through sharing best practice , 2015, European journal of haematology.

[19]  A. Čeponis,et al.  Point‐of‐care musculoskeletal ultrasound is critical for the diagnosis of hemarthroses, inflammation and soft tissue abnormalities in adult patients with painful haemophilic arthropathy , 2015, Haemophilia : the official journal of the World Federation of Hemophilia.

[20]  R. Moineddin,et al.  Diagnostic accuracy of ultrasound for assessment of hemophilic arthropathy: MRI correlation. , 2015, AJR. American journal of roentgenology.

[21]  R. Ljung,et al.  Definitions in hemophilia: communication from the SSC of the ISTH , 2014, Journal of thrombosis and haemostasis : JTH.

[22]  K. Fischer,et al.  Haemophilia Joint Health Score in healthy adults playing sports , 2014, Haemophilia : the official journal of the World Federation of Hemophilia.

[23]  A. Moret,et al.  Comparison of ultrasound and magnetic resonance imaging for diagnosis and follow‐up of joint lesions in patients with haemophilia , 2014, Haemophilia : the official journal of the World Federation of Hemophilia.

[24]  A. Muñoz,et al.  Comparison of ultrasound and magnetic resonance imaging for diagnosis and follow-up of joint lesions in patients with haemophilia. , 2014 .

[25]  K. Fischer,et al.  Using the Haemophilia Joint Health Score for assessment of teenagers and young adults: exploring reliability and validity , 2013, Haemophilia : the official journal of the World Federation of Hemophilia.

[26]  A. Čeponis,et al.  Rapid musculoskeletal ultrasound for painful episodes in adult haemophilia patients , 2013, Haemophilia.

[27]  K. Khair,et al.  Factors affecting the Haemophilia Joint Health Score in children with severe haemophilia , 2013, Haemophilia : the official journal of the World Federation of Hemophilia.

[28]  D. Grobbee,et al.  Turning severe into moderate haemophilia by prophylaxis: are we reaching our goal? , 2013, Blood transfusion = Trasfusione del sangue.

[29]  M. Salvatore,et al.  Magnetic resonance imaging and ultrasound evaluation of “healthy” joints in young subjects with severe haemophilia A , 2013, Haemophilia : the official journal of the World Federation of Hemophilia.

[30]  M. Morfini,et al.  Development and definition of a simplified scanning procedure and scoring method for Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) , 2013, Thrombosis and Haemostasis.

[31]  E. Rodríguez‐Merchán,et al.  The role of ultrasonography in the diagnosis of the musculo‐skeletal problems of haemophilia , 2012, Haemophilia : the official journal of the World Federation of Hemophilia.

[32]  K. Kurnik,et al.  Identification and long‐term observation of early joint damage by magnetic resonance imaging in clinically asymptomatic joints in patients with haemophilia A or B despite prophylaxis , 2012, Haemophilia : the official journal of the World Federation of Hemophilia.

[33]  S. Riva,et al.  Ultrasonography of haemophilic arthropathy , 2012, Haemophilia : the official journal of the World Federation of Hemophilia.

[34]  Christine A. Lee,et al.  Joint protection in haemophilia , 2011, Haemophilia : the official journal of the World Federation of Hemophilia.

[35]  A. Dunn Pathophysiology, diagnosis and prevention of arthropathy in patients with haemophilia , 2011, Haemophilia : the official journal of the World Federation of Hemophilia.

[36]  R. Engelbert,et al.  Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: Validity of the hemophilia joint health score , 2011, Arthritis care & research.

[37]  J. Jacobson Musculoskeletal ultrasound: focused impact on MRI. , 2009, AJR. American journal of roentgenology.

[38]  F. Lafeber,et al.  Understanding haemophilic arthropathy: an exploration of current open issues , 2008, British journal of haematology.

[39]  Alan R. Cohen,et al.  Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. , 2007, The New England journal of medicine.

[40]  P. Babyn,et al.  Sonography for assessment of haemophilic arthropathy in children: a systematic protocol , 2007, Haemophilia : the official journal of the World Federation of Hemophilia.

[41]  S. Donadel-Claeyssens Current co‐ordinated activities of the PEDNET (European Paediatric Network for Haemophilia Management) , 2006, Haemophilia : the official journal of the World Federation of Hemophilia.

[42]  R. Ljung,et al.  MRI scores of ankle joints in children with haemophilia – comparison with clinical data , 2005, Haemophilia : the official journal of the World Federation of Hemophilia.

[43]  J. Ahnström,et al.  A 6‐year follow‐up of dosing, coagulation factor levels and bleedings in relation to joint status in the prophylactic treatment of haemophilia , 2004, Haemophilia : the official journal of the World Federation of Hemophilia.

[44]  D. Grobbee,et al.  Association between joint bleeds and Pettersson scores in severe haemophilia. , 2002, Acta radiologica.

[45]  J. Gago,et al.  Ultrasound in the diagnosis of the early stages of hemophilic arthropathy of the knee. , 1992, Acta orthopaedica Belgica.