After the diagnosis of an internus obturator muscle haematoma in

After the diagnosis of an internus obturator muscle haematoma in Patient 2 (P2) in November 2011, we performed an exhaustive research of such a case in our patient database including severe and moderate inherited haemophilia A (n = 260; about 20% with actual or past medical history of inhibitor) and B (n = 63; about 2% with actual or past medical history of inhibitor). A second patient (P1), displaying the same diagnosis than P2, was identified in November 1987; both patients exhibited an inhibitor to FVIII at the time of the bleeding event. Ultrasonography (US),

Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) were performed, respectively, at first or secondly according to their availability and their results. The radiologic BTK inhibitor ic50 criteria of haematoma of the obturator internus muscle corresponded to the definition proposed by Ali et al. as an ‘asymmetry in size or focal blood attenuation in the substance of the muscle’ [4], notably in the CT pictures. The two patients (P1 and P2) had no other medical history than an inherited haemophilia A. P1 has

been lately diagnosed with a sporadic moderate haemophilia (FVIII, 2–3%) at 7 years of age because of a traumatic right thigh haematoma that had required local treatment only. At 11-year old, he was firstly infused with plasmatic FVIII concentrate (16 days exposure) to cover a surgery for appendicular peritonitis. click here Within the following

2 months spontaneous muscular and joint bleedings (left iliopsosas, right knee, left FK506 calf, left forearm) occurred concomitantly to a low level inhibitor of FVIII (FVIII < 1%; inhibitor, 1–2 Bethesda Unit (BU)). P2 has been diagnosed with a sporadic severe haemophilia (FVIII < 1%; non-sens Ser568X mutation) at 8 months of age because of multiple ecchymoses and muscle haematomas. A persisting high-titre inhibitor occurred rapidly at 12 months old under on-demand treatment with recombinant FVIII therapy (6 days exposure; history peak titre, 53 BU). Successive immune tolerance and recombinant activated FVII (rFVIIa) or activated prothrombin complex concentrate treatment procedures exhibited transient and partial success only. The patient exhibited thereafter several muscle and joint bleedings with two targets joints (left ankle and right knee), but he had never life-threatening haemorrhage. Both patients complained increasing right iliopelvic pain for 24–36 h with slight lameness, after a long step. None of them exhibited fever. At diagnosis P1 was 11-year old and inhibitor appeared 3 months ago was very low or undetectable (<0.6 or 1 BU with FVIII ≤ 1%). P2 was 13-year old and received daily infusions of plasmatic FVIII (100 UI kg−1) associated with on-demand rFVIIa treatment. The inhibitor rate was 15.1 BU, whereas it was only 2.

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